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236 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-0815 WCN19-0841

Poster shift 03 - Autonomic nervous system disorders/history of Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications orders and nutritional disorders or intoxications

Multiple sclerosis and fabry disease, overlooked in the differential Chorea hyperglycemia basal ganglia (C-H-BG) syndrome as the diagnosis of multiple sclerosis? In Turkish population first presentation of type 2 diabetes mellitus in a 65-year-old male S. Guler ̇ a a b b Trakya University, Department of Neurology, Edirne, Turkey^ D. Weerathunga , S. Ambawatte , A. Dissanayake , S. Somarathne aPostgraduate Institute of Medicine- University of Colombo, Neurology, Background Colombo, Sri Lanka bTeaching Hospital- Karapitiya, Neurology, Galle, Sri Lanka ^Central nervous system involvement and chronic white matter ^ ^ lesions are observed in both Fabry disease (FD) and multiple sclerosis (MS), which can confound the differential diagnosis. Background Chorea Hyperglycemia Basal Ganglia (C-H-BG) Syndrome is a Objective known complication of nonketotic hyperglycemia among patients We present an overview of cases from MS, initially misdiagnosed with long term uncontrolled diabetes mellitus. The condition is with multiple sclerosis and report the clinical, MR-tomographical, mainly reported in the elderly Asian population with a female and laboratory findings. preponderance. C-H-BG syndrome in previously undiagnosed diabetic patients also rarely been reported. We report a case of C-H-BG Methods In patients with low α-Gal A activity, screening of the GLA mutation was performed using DBS cards based on Sanger sequence analysis and GLA gene sequence analysis was performed by using MiSeq next generation sequencing (NGS) platform, a FDA approved diagnostic system. All coding exons of the gene and their flanking splice site junctions were amplified using PCR primers, designed with Primer Designer v.2.0^software.^

Results We analyzed the GLA gene, which encodes α-gal A, in 110 patients with clinical and neuroradiological findings consistent with MS to determine whether they had FD. Proband 1 he has ^patient N ^427G A (rs 104894845) ^(p.A143T) hemizigot ^mutation and his N family 2 patient ^427G A (rs 104894845) ^(p.A143T) hemizigot N ^mutation ând 6 patient ^427G A (rs 104894845) ^(p.A143T) fi ^heterozygous ^mutation. ^We identi ed ^1 proband and ^his family eight^patient diagnosed had GLA mutations associated with FD.

Conclusion Asymmetric conﬂuent MRI lesions as well as typical radiological features for MS can be observed. FD should be considered in young patients with unexplained stroke-like episodes, especially in those who have infarction in the vertebrobasilar arterial system, angiokeratomas, and proteinuria. Our results indicate that family history besides neurological ﬁndings should be evaluated in patients with an uncertain diagnosis of MS.^

doi:10.1016/j.jns.2019.10.1240 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 237 syndrome as the first presentation of Diabetes mellitus in a male Subjects & methods patient. To our knowledge this is the first such case reported in Sri Both latency and amplitude of cVEMP (p13-n23) and oVEMP Lanka. (n10-p15) were recorded in10 healthy ^matched controls^,10^MS patients with BS lesion(s)and 10 MS patients without BS lesion(s). Case All patients underwent a complete audiological examination, clinical A 65-year-old previously healthy male who presented with acute neurological evaluation and brain MRI scanning. onset left sided choriform movements for 6 ^days, had high blood sugar values on admission. He had no previous manifestations of Results hyperglycemia and no other micro or macro vascular complications The latency of P13-N23 and N10-P15 in MS participants with and of diabetes were noted. without BS lesions were significantly prolonged compared to normal fi ≤ His non-contrast CT of the brain ( g.1) revealed characteristic controls (p^ ^0/05). Additionally latency of P13-N23 and N10-P15 in fi asymmetrical high intensity signals in bilateral basal ganglia. MRI ^patients of MS with BS lesion(s) were signi cantly prolonged ≤ brain revealed non-contrast enhancing T1 high intensity signals in compared to patients without BS lesion(s) (p^ ^0.05).^No relationship bilateral basal ganglia mainly involving right lentiform nucleus was found between the clinical state and VEMP responses.No (fig.2) and low intensity in T2 with normal FLAIR images. relationship was found between the radiological findings of the He found asymptomatic after 2 ^weeks, with good glycemic patient and VEMP responses except for the group of MS with BS control. affection. A good correlation was found between VEMP latencies and EDSS. Conclusion C-H-BG Syndrome should be considered in patients with acute Conclusion onset hemichorea, irrespective of their previous history of Diabetes Abnormality of cervical VEMP and ocular VEMP in patients of mellitus. Good glycemic control alone can cure C-H-BG syndrome in ^Multiple Sclerosis with brainstem lesion(s) are more frequent than majority of patients. în patients of Multiple Sclerosis without brainstem lesion(s) .

Keywords: Chorea, Basal Ganglia, Hyperglycemia, C-H-BG Syndrome doi:10.1016/j.jns.2019.10.1242

WCN19-0845 doi:10.1016/j.jns.2019.10.1241

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-0844 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Poster shift 03 - Autonomic nervous system disorders/history of Dexterity, attention and working memory in patients with neurology/motor neuron disease/movement disorders/MS & Parkinson's disease demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications N. Çetişli Korkmaza, M. Duraya, D. Topçua, T. Can Akmana, H.F. Gündüza, L.S. Birb Assessment of cervical and ocular vestibular evoked myogenic aPamukkale University, School of Physical Therapy and Rehabilitation, potentials in multiple sclerosis(MS) patients Denizli, Turkey b Faculty of Medicine, Neurology, Denizli, Turkey^ J. Mekkya, D. Elmoazenb, H. Kozouc, M. Gawishd a Alexandria University- Faculty of Medicine - AAN - AASM - ESRS Objective Member, Neuropsychiatry, Alexandria, Egypt Disturbed manual dexterity is common among patients with b Alexandria University, Ototlaryngology, Alexandria, Egypt Parkinson's Disease(PD), even in early stages of the disease and may c Alexandria University, Otology, Alexandria, Egypt be less responsive to pharmacological treatment. Cognitive impair- d Alexandria University - Faculty of Medicine, Otolaryngology, Alexndria, ments are associated with old age and severe motor symptoms and Egypt^ occur mostly in the later stages. The aim of this study is to investigate hand dexterity, attention and working memory and their Multiple sclerosis (MS) is one of the most important chronic association in patients with PD.^ neurological disease affecting the central nervous system and is the leading cause of disability due to brainstem affiction. Vestibular Methods evoked myogenic potentials (VEMPs) are short-latency muscle PD patients were grouped according to Hoehn and Yahr staging responses. as early-stage (1–2, Group1; n = 16) and mid-stage (3–4, Group2; n Cervical VEMP (Cvemp) is a ^demonstration of vestibule-collic = 16) Hand dexterity, attention and working memory were assessed fl re ex^, while ôcular VEMP (oVEMP) which is a manifestation of with Nine Hole Peg Test(NHPT), Stroop Color-Word Test (SCWT) and vestibulo–ocular reflexes. subtests of Wechsler Memory Scale-Revised(WMS-R), respectively.^

Aim Results ^The aim of this study was to assess cVEMP and oVEMP in MS The mid-stagers' NHPT, Logical Memory-II subtest of WMS-R and patients with and without brainstem lesion(s) and comparing the b ^ ^ SCWT scores were worse than early-stagers'(p ^.05). However, it ﬁndings with normal controls. was seen that the groups had similar properties in terms of WMS- ARTICLE IN PRESS

238 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Digit Span (p N 0.05). The relation between left hand dexterity Conclusions fi b (NHPT), attention and memory were signi cant (p ^.05). While the ICF is a useful tool for multidisciplinary approach, which can stage of PD was correlated with NHPT, SCWT and WMS-Logical promote the communication and collaboration in the healthcare b fi fi Memory I (p ^.05), we could not nd a signi cant relationship with team by establishing a common language across different disciplines. N fi the rest of WMS-R subtests(p ^.05). ICF could be an essential tool in identifying and measuring ef cacy and effectiveness of assessment and treatment approaches, both Conclusion through functional profiling and intervention targeting on self-care Dexterous impairment leads to difficulties in activities of daily and independence in MS. Research about motor behaviour of the living (ADL) that require fine motor skills, especially with the upper limb in MS patients undergoing neurorehabilitation could progression of the PD's stage. These could be in association with the focus on comprehensive assessment with biopsychosocial model, deficient cognitive performance, which could be appear in early- which is an unexplored field. stage PD patients. Cognitive assessment and dexterity could be in the context of assessment and treatment of PD patients from the early doi:10.1016/j.jns.2019.10.1244 terms with multidisciplinary approach. doi:10.1016/j.jns.2019.10.1243 WCN19-0854

Poster shift 03 - Autonomic nervous system disorders/history of WCN19-0849 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Poster shift 03 - Autonomic nervous system disorders/history of orders and nutritional disorders or intoxications neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- A 3-year observation of excessive daytime sleepiness after orders and nutritional disorders or intoxications subthalamic deep brain stimulation in patients with Parkinson's disease ICF biopsychosocial model for self-care perspective to understand the dexterity and independence in patients with multiple Y.J. Junga, H.J. Kimb, W.W. Leec, G. Ehmd, T.J. Leea, S.Y. Ryua, S.B. Leea, sclerosis B. Jeonb aDaejeon St. Mary's Hospital, College of Medicine, The Catholic T. Can Akmana, L.S. Birb, N. Çetişli Korkmaza, F. Yarara University of Korea, Neurology, Daejeon, Republic of Korea aPamukkale University, School of Physiotherapy and Rehabilitation, De- bParkinson Study Group and Neuroscience Research Institute, College of nizli, Turkey Medicine, Seoul National University, Neurology, Seoul, Republic of Korea b c Neurology, Denizli, Turkey^ Nowon Eulji Medical Center, Eulji University, Neurology, Seoul, Republic of Korea d Objectives National Medical Center, Neurology, Seoul, Republic of Korea^ Multiple Sclerosis(MS) leads to impaired function and limited participation in activities and self-care, which impact life-quality and Background and purpose independence, directly. The International Classification of Function- We aimed to assess the longitudinal changes of the quality of ing, Disability and Health(ICF), provides standard language and sleep and excessive daytime sleepiness (EDS) in PD patients framework for description of health and health-related state with undergoing STN DBS and identify which factors are associated with biopsychosocial model. Appropriate measures compatible with the presence of EDS before and after STN DBS. content of ICF is a parallel need in documenting the impact and nature of limitations of function, activities and participation. The aim Methods of this study was to determine the relation of hand function and A total of 33 PD patients who underwent bilateral STN DBS independence with ICF's self-care subcomponent in patients with between July 2011 and October 2015 were recruited. We evaluated Multiple Sclerosis(MS).^ subjective sleep quality assessed by Parkinson's Disease Sleep Scale (PDSS) and EDS using Epworth Sleepiness Scale (ESS) preoperatively Methods and^6 months, 1^year, and 3^years postoperatively. A cut-off ESS score Seventy-nine MS patients with 3.41 ± 0.92 mean EDSS score and of ≥11 was applied. 8.42 ± 5.32 years' disease duration, included. ICF's subcomponent of self-care, Jamar Hydraulic Hand Dynamometer(JHHD), Nine-Hole Results Peg Test(NHPT) and Functional Independence Measure(FIM) used There is a significant improvement in PDSS, and a noticeable for assessment protocol.^ change occurs immediately after the surgery. After DBS, the number of patients with persistent EDS gradually decreased, but patients with Results newly developed EDS were added. Baseline ESS score was highly Right-handed 72 patients' mean NHPT and JHHD scores were correlated with EDS at 6^months and 1^year postoperatively, and older 27.35 ± 10.16 and 52.56 ± 18.65. Relationships of JHHD, NHPT and age of PD onset was highly associated with EDS at 1^year after DBS. At 3 FIM(except toileting) with ICF's self-care subcomponents(eating, ^years after DBS, the total PDSS score is a main contributing factor for caring for body parts, washing oneself, toileting, putting on clothes, EDS. There was no significant difference in agonist LED and LEDD fi putting on footwear) were signi cant(p = .001).^ between groups with and without EDS at any time points.^ ARTICLE IN PRESS

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Conclusions Methods Bilateral STN DBS improves the subjective sleep quality, but EDS We studied 34 people with self-reported dizziness. Dizziness was ﬁ may improve or worsen. The risk factors for EDS change over time de ned as a giddiness^, faint feeling and light-headedness. Dizziness after STN DBS. Interestingly, dopaminergic medication did not affect severity was assessed using^dizziness handicap inventory ( DHI)^and EDS in DBS-treated PD patients. short physical performance battery (SPPB). Brain perfusion data were analyzed in patients using semi quantetive software and statistical doi:10.1016/j.jns.2019.10.1245 parametric mapping (SPM).

Results There were 34 patients (mean age 72.01^years, SD^=^8.28) with WCN19-0857 dizziness precipitated in the study.^ Seventeen (50%) cases had 1 or 2 regions involvement in SPECT and 13(38.8) cases had 3 or more regions involvement.^ Poster shift 03 - Autonomic nervous system disorders/history of In addition, data analysis by SPM in 11 patients was done and neurology/motor neuron disease/movement disorders/MS & showed that all patients had hypoperfusion regions in SPECT.^ fi demyelinating diseases/neuromuscular disorders/vestibular dis- In qualitative assessment of brain SPECT^, no signi cant associa- orders and nutritional disorders or intoxications tion was seen between perfusion defect severity with DHI score(p = fi ^0.12). SPM analysis of imaging ^showed a signi cantly decreased Cerebral perfusion abnormalities in patients with dizziness: A brain perfusion in right precuneus, cuneus, superior and inferior spect study Occipital lobe, inferior frontal, mid frontal, temporal^, inferior parietal and left superior parietal, precuneus, cerebellar, insula, putamen and a significant hypoperfusion in the bilateral cerebellum. In qualitative R. Nematia, M.A. Nayerib, M. Assadib ^ assessment, significant association was seen between perfusion aNuclear Medicine Research Center, Department of Molecular Imaging defect severity with SPPB test(p = 0.019). and Radionuclide Therapy MIRT, Bushehr Medical University Hospital, ^ Faculty of Medicine, Bushehr University of Medical Sciences, Bushehr, ^ Conclusion Iran The study may demonstrate that multiple regions of brain bDepartment of Neurology, Bushehr Medical University Hospital, Faculty impaired in patients with dizziness that supported dizziness may of Medicine, Bushehr University of Medical Sciences, Bushehr, Iran ^ be manifestation of an abnormal integration of multisensory modality. Background^ The presence of perfusion and structural abnormalities on brain imaging in people with dizziness has not been fully addressed. Thus, doi:10.1016/j.jns.2019.10.1246 the purpose of this study was to investigate the brain perfusion in patients with dizziness using SPECT.^ ARTICLE IN PRESS

240 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-0861 Tumefactive multiple sclerosis (TMS) is a rare variant of multiple sclerosis (MS) comprising of large demyelinating lesions that frequently mimic intracranial neoplasm or abscess. MR Poster shift 03 - Autonomic nervous system disorders/history of Spectroscopy (MRS) is an exciting new tool to differentiate neurology/motor neuron disease/movement disorders/MS & radiologically. demyelinating diseases/neuromuscular disorders/vestibular dis- We present three cases with TMS who were diagnosed with MRS orders and nutritional disorders or intoxications brain.

Etiopathogenetic factors and clinical picture of restless legs Case 1 syndrome in persons of Uzbek nationality A 24-years-old male presented with sensory symptoms that resolved after steroid pulse therapy. MRI spine and brain showed U. Yodgarovaa, M. Raimovab, K. Boboyevc lesions consistent with MS and CSF showed Oligoclonal bands. aTashkent State Stomatology Iistitute, Neurology, Tashkent, Uzbekistan Despite disease modifying therapy, he developed new symptoms. A bTashkent State Stomatogy Institute, Neurology, Tashkent, Uzbekistan repeat MRI brain was static and MRS showed evidence of elevated cTashkent State Stomatology Institute, Neurology, Tashkent, Uzbekistan peaks of Choline and lactate with mild reduction of NAA consistent with demyelination.^ Restless legs syndrome (RLS) is a sensomotor disorder character- Case 2 ized by unpleasant sensations in the lower limbs at rest, and often A 32-years-old male came for lower limb weakness and leads to sleep disturbance. numbness with progressive gait impairment. He had history of urinary symptoms, imbalance, dizziness, ataxia and excessive fatigue. Aim CSF analysis was normal. MRI brain revealed multiple lesions To study the etiopathogenetic factors and the clinical character- consistent with MS along with a left frontal lesion with perilesional istics of restless legs syndrome in persons of Uzbek nationality. edema and faint rim enhancement. MRS showed elevation of lactate and choline consistent with TMS. NAA was reduced. He was started Material and methods on Cladribine with good response.^ We examined 44 patients with RLS from 21 to 70^years old (mean age 41.8 ± 8.1 years). The diagnosis was made according to special ^ Case 3 criteria that can be differentiated. A 37-years-old female known to have MS, on Fingolimod, developed a new relapse. MRI brain showed a left parietal cystic Results lesion with no contrast enhancement along with other lesions The ratio of women and men was 1.75: 1. Primary RLS was consistent with MS. MRS of the cystic lesion shows low NAA and detected in 14 patients (35%) and secondary RLS in 30 patients high Choline. She was switched to Natalizumab. (65%). The causes of secondary RLS were: 33.3% — chronic renal insufficiency, 16.6% pregnancy, 16.7% iron deficiency anemia, 20% Conclusion diabetes mellitus, in 4 patients (13,3%) Parkinson's disease and MR spectroscopy may serve as a helpful diagnostic tool to multiple sclerosis. A sleep score on a five-point scale revealed differentiate TMS from other counterparts. abnormalities in 2 (4.5%) patients — as very pronounced sleep disorders, 7 (16%) patients — as severe disorders, in 25 (56.8%) doi:10.1016/j.jns.2019.10.1248 patients, moderate, and 10 (^22.7%) patients - as light sleep disorders. When determining the severity of RLS on the D. Hopkins scale in 30 patients (68.1%), RLS is rated as mild, in 10 patients (22.7%) - moderate, in 4 (9%) patients as very severe. WCN19-0867 Conclusion The etiopathogenetic structure of RLS in our region is similar to Poster shift 03 - Autonomic nervous system disorders/history of data from studies in other regions. RLS is accompanied by severe and neurology/motor neuron disease/movement disorders/MS & moderate sleep disturbances and is characterized by a predominance demyelinating diseases/neuromuscular disorders/vestibular dis- of mild to moderate severity. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1247 Switching from double-blind entacapone or placebo to open- label opicapone: UPDRS-II and III results from patients who ended 1-year BIPARK-I extension on opicapone 50 mg WCN19-0866 F. Rochaa, J. Ferreirab, A. Leesc, E. Tolosad, F. Stocchie, A. Santosf,D. Magalhaesa, P. Soares-da-Silvaf, N. Savica Poster shift 03 - Autonomic nervous system disorders/history of aBIAL - Portela & Co SA, Global Parkinson's Disease, S. Mamede neurology/motor neuron disease/movement disorders/MS & Coronado, Portugal demyelinating diseases/neuromuscular disorders/vestibular dis- bFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal c orders and nutritional disorders or intoxications National Hospital for Neurology and Neurosurgery, Neurology, London, United Kingdom d Tumefactive multiple sclerosis: Emerging role of MR spectroscopy Hospital Clinic Universitari de Barcelona, Neurology, Barcelona, Spain eIRCCS San Raffaele, Neurology, Rome, Italy fBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, S. Siddiqi, J.S. Inshasi Portugal Rashid Hospital, Department of Neurology, Dubai, United Arab Emirates^ ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 241

Ôbjective Introduction To evaluate the impact on UPDRS-II and III in levodopa-treated Multiple sclerosis (MS) is a neurological disorder which predom- Parkinson's Disease (PD) patients who switched from placebo (PLC) inantly affects females. Significant number of males too suffer from or entacapone (ENT) to opicapone (OPC) and ended the 1-year MS.Ît's an emerging neurological problem in Sri Lanka. We looked at extension taking OPC-50 mg. OPC, a once-daily COMT inhibitor, gender difference in a cohort of 100 Sri Lankan patients with fl fi proved effective in the treatment of motor uctuations in PD patients con rmed MS attending a specialized MS clinic.^ in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. Objective^ Methods To determine whether any gender differences exist between men After completing the BIPARK-I double-blind part, PLC- and ENT- and women suffering from MS in Sri Lanka in terms of clinical patients switched to a 1-year open-label extension on OPC-treatment. characteristics and treatment responses.^ This post-hoc analysis investigated the impact on UPDRS-II and III scores in PLC and ENT ‘switchers’ who ended the 1-year extension Methods taking OPC-50 mg using a linear mixed-effect model repeated 100 MS patients attending the MS clinic at the National Hospital measurement (MMRM) with region as factor and baseline as covariate. of Sri Lanka were randomly assessed using direct interviews and patient registries. Diagnosis was based on McDonald's (2010) and Results MAGNIMS (2016) criteria. NMOSD and MOGADs were excluded by In total, 199 patients switched from PLC (n =99)orENT(n =100) AQP4-IgG and MOG-IgG testing.^ to 1-year OPC open-label extension. LS mean changes of UPDRS-II from − − − double-blind baseline were ^2.4, 3.1 and ^3.0 for PLC, ENT and Results^ − b OPC-50 mg, and further decreased for PLC ( 2.7; p ^.0004) and ENT (n = 100) 63% females and 37% males evaluated. Âge at onset − ( 0.6; p =^.4179) switched-subjects by the end of the 1-year open- was similar, average 29Y. More males presented with spinal cord label OPC extension. LS mean changes of UPDRS-III from double-blind syndromes (p =^.004), visual (n = 30, 47%) cortical syndrome (n = − − − baseline were ^3.7, 4.4 and ^4.5 for PLC, ENT and OPC-50 mg, and 28, 44%) were common in females.^RRMS is the predominant type in − b − fi further decreased for PLC ( 5.1; p ^.0001) and ENT ( 1.4; p =^.2404) both.În Males, PPMS was signi cantly commoner (p =^.015). Brain fi switched-subjects by the end of the 1-year open-label OPC extension. lesions were similar in both. ^Spinal cord lesions were signi cantly more in females (p =^.001). Annualized relapse rate (ARR) was N Conclusion higher for females (0.51vs0.43) but a mean EDSS score^ 3.5 was fi There was either no worsening (ENT) or signi cantly less seen in 38% males and in only 18% females (p =^.02).^ disability (PLC) in UPDRS non-motor and motor domains for ENT and PLC switched-subjects to 1-year open-label OPC. Conclusions Male MS patients in Sri Lanka had more progressive disease with Uncited references greater disability compared to females. They presented with spinal [1,2] cord syndromes more often. No gender differences were noted in any other clinical or demographic parameters. References Keywords:^Sri Lanka, Multiple sclerosis, Men & Women [1] Ferreira, et al., Lancet Neulrology 15 (2) (2016) 154–165. [2] Lees et al., 2017; 74(2):197–206.

doi:10.1016/j.jns.2019.10.1249

WCN19-0876

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Multiple sclerosis in Sri Lanka across gender: A comparative analysis of clinical characteristics and treatment responses in men and women

B. Senanayake, M. Aravinthan, S. Thevasakayam, I. Rajapakshe National Hospital of Sri Lanka, Institute of Neurology, Colombo, Sri Lanka^ ARTICLE IN PRESS

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doi:10.1016/j.jns.2019.10.1250 medical assessments. Excluding them, there was a tendency for the rate of cardiopulmonary assessment to increase. In rare disease such as DMD, it is difficult even for specialists to have sufficient experience. In this survey, we could confirm that DMD-GL became WCN19–0877.

The effects of a practical guideline on medical management of patients with Duchenne muscular dystrophy

T. Matsumuraa, H. Komakib aNational Hospital Organization Osaka Toneyama Medical Center, Neurology, Toyonaka, Japan bNational Center of Neurology and Psychiatry, Translational Medical Center, Kodaira, Japan

We conducted questionnaire surveys of Japanese certified Neurologists and Child Neurologists (CN) before and four years after the publication of the Japanese practical guideline for Duchenne muscular dystrophy (DMD-GL), published in 2014. After the publication, the number of responders who did not require expert support to treat new DMD patients increased from 33% to 37%. In addition, DMD-GL was listed as a preferred information source by 86% of specialists practicing DMD. Although there was no significant difference, CN has become more active in steroid therapy (Fig. 1), rehabilitation, and management of scoliosis. The ratio of introduction of non-invasive ventilation and mechanical assisted cough was significantly increased for respiratory care (Fig. 2). Nevertheless, the implementation rate for disaster countermeasure was rather declined. As for the myocardial treatment, specialists using ACEI preferentially increased while diuretics and digitalis decreased. There was no change in beta-blockers. Regarding nutritional management, the response to wait for the explanation of gastrostomy until the advanced stage decreased, and the response to recommend it early increased (Fig. 3). About medical care of carriers, around 40% of specialists responded that they did not have carriers requiring ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 243

inadequate symptom control and possible eligibility of device-aided therapies was made based on frequency and severity of motor symptoms, non-motor symptoms and functional limitations. A high concordance between clinical judgement and MANAGE-PD recommendation was observed (Intra-class co-efﬁcient: 0.82; weighted kappa statistic:0.71; unweighted kappa statistic:0.78; concordance for the categories ranged from 82.35 to 88.24%).

Conclusions MANAGE-PD demonstrated high reliability and validity. Future steps include validation with a large global survey of general neurologists and optimizing scoring algorithm based on patient-level data.

doi:10.1016/j.jns.2019.10.1252 an essential tool in best practicable care. Based on the results, we wish to use it for DMD-GL revision and future activities. doi:10.1016/j.jns.2019.10.1251 WCN19-0900

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-0886 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Early MRI changes of neuromyelitis optica spectrum disorder in demyelinating diseases/neuromuscular disorders/vestibular dis- patients of Uzbekistan orders and nutritional disorders or intoxications S. Shokhimardonova, S. Khudjanovb aTashkent Medical Academy, Neurology, Tashkent, Uzbekistan MANAGE-PD: A clinician-reported tool to identify patients with bTashkent Medical Academy, Surgery, Tashkent, Uzbekistan Parkinson's disease inadequately controlled on oral medications – results from vignette-based validation Background Early diagnosis of Neuromyelitis optica spectrum disorders H. Yehia (NMOSD) is critical. Unlike multiple sclerosis, NMOSD episodes are Medical Science Liaison, Medical Department -AbbVie, Dubai, United normally quite severe. These episodes can lead to irreversible Arab Emirates consequences. The diagnosis of NMOSD is based on the presence of evidence of optic neuritis and transverse myelitis. Study additional Objectives ﬁndings in early MRI scans could play important role in early To evaluate the reliability and validity of MANAGE-PD tool. diagnosing and treatment course.

Background Objective Timely identification of advancing symptoms of people living with To establish MRI changes pattern among Uzbekistan patients with Parkinson’s Disease for clinical practice improvement. MANAGE-PD is NMOSD. a simple clinician-reported screening tool developed to address this practice gap. Through timely evaluation of PD symptoms. Methods We retrospectively analyzed the results of MRI scanning among Methods 45 patients in the Tashkent Medical Academy Neurology Department Hypothetical vignettes (n = 10) were developed to represent a with a diagnosis NMOSD. wide spectrum of disease severity. this tool was conducted via a web-based survey of selected panelists. Steering Committee classi- Results fied each vignette into 3 categories: adequately controlled on oral The results of MRI study demonstrated a high incidence of therapy; inadequately controlled on oral therapy and recommend involvement of the brain stem (44%) and hemispheres per- oral optimization only; inadequately controlled on oral therapy and iventricular white matter (21%) at the time of disease clinical debut. recommend evaluation for device-aided therapies along with oral Lesions involving corticospinal tracts and diencephalic region was optimization. Each panelist evaluated one anchor vignette and four find only in one patient (2,2%). randomly assigned vignettes using the MANAGE-PD tool. Concor- dance between clinical judgment for management of patient versus Conclusion MANAGE-PD recommendation was assessed. This study confirms that early MRI diagnosing results among patients with NMOSD in Uzbekistan usually demonstrate involve- Results ment of the brainstem and hemispheres periventricular white The panel included MDS (n = 19) from 15 countries, with matter. extensive experience in treating PD [Mean: 24.4 ± 7.6 years; Mean patients treated/month: 73.2 ± 45.4]. In open-ended feedback, panel- doi:10.1016/j.jns.2019.10.1253 ists reported no issues with usage of the tool. The determination of ARTICLE IN PRESS

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WCN19-0909 Background Neuroacanthocytosis is a group of rare movement disorders and often not diagnosed. Neurologic problems usually consist of either Poster shift 03 - Autonomic nervous system disorders/history of movement disorder of ataxia, personality changes, cognitive deteri- neurology/motor neuron disease/movement disorders/MS & oration, and seizures. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Purpose To describe the first case of neuroacanthocytosis in Dr. Sardjito Documentation of clinical and laboratory features of patients Hospital, Yogyakarta, Indonesia with spinal isolated syndrome who have no cranial lesion Method and result L.S. Bir, S. Tekin A 32 year-old male came to our clinic with a history of fi Pamukkale University Medical Faculty, Neurology, Denizli, Turkey progressive orofacial dyskinesia, chorea, dif culty of swallowing, and ataxia. Symptoms worsened over the course of four years and patient was hospitalized multiple times. Upon the latest hospitaliza- Introduction tion we ran several diagnostic tests and imaging. Blood smear Risk conversion to MS in spinal clinically isolated syndrome (CIS) revealed acanthocytes and MRI revealed atrophied cerebrum. Patient patients were reported as 41% - 61%, but most of these patients have was diagnosed with neuroacanthocytosis and given symptomatic also asymptomatic cranial lesions. In this series we reported clinical treatment. and laboratory characteristics of the spinal CIS patients who were evaluated in last two years whose cranial MRI were normal. Conclusion This rare disorder should be considered in the differential Cases diagnosis of cases with chronic orofacial dyskinesia, ataxia, chorea, Only 5 cases met this specification in last two years. 40-year-old and cognitive impairment. We found that extension of the head to female with hand and feet numbness who has lesion at C2-3 level; the back and bitten tongue was the symptoms that differentiate 56-year-old female with right hemihypoesthesia and hemiparesis neuroacanthocytosis from other diseases. Currently there is no who has lesions at levels of C4-5 and C7-T1; 24-year-old male with specific treatment for the primary diseases so symptomatic treat- hypoesthesia on left hand who has plaque at C2 level; 36-year-old ment is given to suppress the symptoms. male patient with left hemihypoesthesia and Lhermitte sign who has lesion at C2-3 posterolaterally; 25-year-old female patient with doi:10.1016/j.jns.2019.10.1255 numbness of legs and sensorial ataxia who has multiple cervical and thoracal cord lesions will be presented. Only one patient had second attack also related with cord after 2 monts in follow up.

Results WCN19-0927 Cervical cord was the most common involved area. VEP's of all patients were normal. IgG index were detected high in two patients Poster shift 03 - Autonomic nervous system disorders/history of whom also have OCB (Type 2) Anti MOG and anti NMO antibodies neurology/motor neuron disease/movement disorders/MS & were negative in all patients Vitamin D levels were low in 4 patients. demyelinating diseases/neuromuscular disorders/vestibular dis- Discussion orders and nutritional disorders or intoxications Isolated spinal cord syndrome can be seen as first demyelinated event in some patients without cranial MRI lesions. It is unclear how Profile of vitamin b12 deficiency in a urban neurology clinic in this process will go on. We wanted to take attention to this atypical Mumbai, Maharashtra group and emphasize the importance to define varied CIS groups’ characteristics and prognosis. N. Surya, V. Patil Bombay Hospital and Research Centre, Neurology, Mumbai, India doi:10.1016/j.jns.2019.10.1254 Background Vitamin B12 deficiency a systemic disease affecting the entire nervous system. In India, vitamin B12 deficiency is seen in about 3.8% WCN19-0923 of population, largely linked to the predominantly vegetarian diet. (See Table 1.)

Poster shift 03 - Autonomic nervous system disorders/history of Methods neurology/motor neuron disease/movement disorders/MS & An observational study was conducted at an urban tertiary demyelinating diseases/neuromuscular disorders/vestibular dis- neurology clinic. We studied demographics, clinical features, orders and nutritional disorders or intoxications biochemical, imaging and electrophysiological characteristics of all patients who were detected with low serum vitamin B12 The ﬁrst neuroacanthocytosis in Yogyakarta, Indonesia: A case levels. report Results S. Sekarlangit, S. Subagya, I. Setyaningsih, A. Astuti Out of approximately 5100 patients who presented to the clinic, Universitas Gadjah Mada, Neurology, Yogyakarta, Indonesia 290 patients (males-161/females-129) had low vitamin B12 levels. The mean age and serum vitamin B12 levels were 39.73 ± 14.23 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 245

years and 171.29 ± 98.42 pcg/ml respectively. Majority were vege- dementia, cognition, and neuropsychiatric disturbances were exam- tarians (87.6%). Table 1 shows Various neurological diagnoses seen in ined in the patients with PDD. patients with vitamin B12 deficiency Results Conclusion The PDD group showed hypoperfusion in most of the cortical, Vitamin B12 deficiency presents with varied neurological mani- subcortical, and cerebellar areas compared to healthy controls at festations. Whether low vitamin B12 levels in patients with primary baseline. At the follow-up, changes in rCBF, CDR (p = 0.32), sum of headache especially migraine is an incidental or a causal finding, box of CDR (p = 0.49), MMSE (p = 0.61), GDS (p = 0.79), and NPI remains yet to be explored. (p = 0.23) were not significant in the PDD patients. doi:10.1016/j.jns.2019.10.1256 Conclusions Our findings implicate that memantine may delay the progression of brain perfusion deficits and clinical symptoms of PDD in the long term. WCN19-0931 doi:10.1016/j.jns.2019.10.1257

Poster shift 03 - Autonomic nervous system disorders/history of Efficacy of NMDA receptor antagonist on regional cerebral blood neurology/motor neuron disease/movement disorders/MS & flow in Parkinson's disease with dementia demyelinating diseases/neuromuscular disorders/vestibular dis- K.S. Leea, I.U. Songa, Y.A. Chungb, S.W. Chunga orders and nutritional disorders or intoxications aCatholic University of Korea, Neurology, Seoul, Republic of Korea bCatholic University of Korea, Radiology, Seoul, Republic of Korea Peripheral facial palsy as a first symptom of multiple sclerosis – A warning cases report Background & objective a b c d Although the treatment efficacy of memantine in Parkinson’s A. Feitosa , T. Winckler , J. Khouri , C. Assunção a disease dementia (PDD) has been reported after several weeks of Vita Batel Hospital, Neurology, Curitiba, Brazil b administration, the long-term effects on brain perfusion and Universidade Positivo, Neurology, Curitiba, Brazil c clinical symptoms remain unclear. The current study aimed to UFRJ, Neurology, Rio de Janeiro, Brazil d follow-up PDD patients after 18 months of memantine treatment University of Louisville, Neurology, Louisville, USA using 99mTc hexamethylpropylene amine oxime single photon emission computed tomography (SPECT). Since the beginning of our medical and neurological study, we learn that there are two types of facial palsy, central and peripheral; Methods and only one way to a peripheral facial palsy be caused by a central A total of 15 patients with PDD and 11 healthy participants were lesion. recruited into this study and they were assessed with brain SPECT, Therefore, when we think about a young healthy person who Mini-Mental State Examination (MMSE), Clinical Dementia Rating presents themselves in an emergency room (ER) with a peripheral (CDR), Global Deterioration Scale (GDS), and Neuropsychiatric facial palsy as the only symptom, the most common cause is either Inventory (NPI). Differences in regional cerebral blood flow (rCBF) Bell's palsy or caused by Herpes Virus. We report a few cases of between the two groups were evaluated at baseline. After 18 months Multiple Sclerosis (MS) on which the first symptom was peripheral of memantine administration, changes in brain perfusion, severity of facial palsy, and describe the evolution unique of each one of the ARTICLE IN PRESS

246 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx cases. The aim is to alert that multiple sclerosis can mimic other more benign conditions and specialists should consider MS as the reason for peripheral facial palsy. doi:10.1016/j.jns.2019.10.1258

WCN19-0954

Assessment of cognitive difficulties and depression in a cohort of were seronegative for AQP4&MOG. MMSE, MoCA and Becks depres- Sri Lankan patients diagnosed with clinically and radiologically sion scale (validated versions) were used for data collection. definite multiple sclerosis: A cross sectional descriptive study Results I. Rajapakshe, B. Senanayake, M. Aravinthan, R. Sudath Mean age at diagnosis was 29 years with 61.4% females. At Institute of Neurology, National Hospital of Sri Lanka, Colombo, Sri evaluation average age was 35 with an average disease duration of 6 Lanka years. 63.9% had cognitive impairment and 49.4% had some degree of depression, 3.6% severe, 14.5% moderate and 30.1% mild. 50.9% of Background cognitively impaired patients were also depressed. Significant Multiple sclerosis (MS) is an immune mediated demyelinating association noted between cognitive impairment and TTFR (Time disease.Cognitive impairment in MS is known to occur in 40%–65%. It To First relapse) with p value of 0.045 (p b 0.05) and depression and mainly involves complex attention, information processing speed duration of diagnosis (p = 0.02). Cognition and depression were not and executive functions. This study analyzes the level of cognitive significantly associated (p = 0.88). deficiency, prevalence of depression and their association with disease severity in a cohort of Sri Lankan MS patients. Conclusions Cognitive impairment and depression significantly affect South Objectives Asian MS populations. To assess level of cognitive difficulties and depression in Sri Lankan MS patients and to describe the effects of socio-demographic doi:10.1016/j.jns.2019.10.1259 characteristics. Also to assess whether these parameters are affected by disease severity, duration, and number of relapses and level of motor disability. WCN19-0963 Method 82 confirmed MS patients (McDonald 2010, MAGNIMS 2016) attending a dedicated MS clinic at the National Hospital of Sri Lanka Poster shift 03 - Autonomic nervous system disorders/history of were randomly using direct interviews and patient registries. They neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Correlation of driving duration and low back pain among online taxi driver in Makassar City, South Sulawesi, Indonesia

W.S. Andia, K. Khairunnisab, A. Emyc aUniversity of Muhammadiyah Makassar, Neurology, Makassar, Indo- nesia bUniversity of Muhammadiyah Makassar, Physiology, Makassar, Indo- nesia cUniversity of Muhammadiyah Makassar, Medical Education, Makassar, Indonesia

Background Low back pain (LBP) is a non-speciﬁc condition that refers to complaints of acute or chronic pain and discomfort in the lumbosacral area. This condition is often found in the community as one of the musculoskeletal disorders, psychological disorders and as the consequences of wrong mobility. Nowadays, innovations due to rapid technology development increase online public ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 247 transportation businesses that have an impact on social and these relapses occurred within 2 years of pharmacological remission economic conditions in Indonesia especially in Makassar. Driver is a while patients who were in PR for 5 years did not show relapse. CSR job that has a risk that can cause LPB, due to poor body posture, was seen in 1% and PR in 18% of patients after 5 years. Patients inadequate seat support and fatique of back muscles. Online taxi with thymoma had poor remission rates compared to non drivers generally drive their vehicles for quite a long time, on thymomatous subgroup (p b 0.008). There was no correlation of average 8 hours / days. This condition increases the risk of LPB remission rates with age, gender, number of crisis, disease severity occurrence in the online taxi driver population. at onset, disease duration, antibody status or levels. The clinical profile and outcome were similar in Juvenile, early and late onset Objective myasthenia gravis. To investigate the prevalence of LBP among online taxi drivers and its correlation with duration of driving. Conclusion Though the response to therapy in most myasthenics is good, Methods sustained pharmacological and complete remission rates were low The design of this study is quantitative analytic with cross and significant proportion of patients relapse eventually. The most sectional approach. The sample consisted of 53 online taxi drivers important negative predictor for remission was presence of selected using total sampling method. The datas were collected by thymoma. Sustained pharmacological remission of least 5 years is questionnaire and processed using the Chi-Square Test. associated with low relapse risk and good long term outcome. Thus we propose that long term immunosuppression for atleast 5 Results years should be considered in myasthenia gravis due to low There were 83,3 % of respondents had more than 8 hours of remission rates, unpredictable course, especially in thymomatous driving and 16.7% of respondents had less than 8 hours of driving. MG The results of statistical tests with chi-square test found a significant correlation between LBP with duration of driving (p = 0.026) among doi:10.1016/j.jns.2019.10.1261 online taxi drivers in Makassar.

Keywords: Driving duration, Low Back Pain, Online Driver WCN19-0982 doi:10.1016/j.jns.2019.10.1260 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- WCN19-0965 orders and nutritional disorders or intoxications

Poster shift 03 - Autonomic nervous system disorders/history of Treatment responses, disability outcomes and prognosis of a neurology/motor neuron disease/movement disorders/MS & cohort of Sri Lankan patients with clinically definite multiple demyelinating diseases/neuromuscular disorders/vestibular dis- sclerosis: Preliminary data from a dedicated MS clinic orders and nutritional disorders or intoxications B. Senanayake, M. Aravinthan, I. Rajapakshe Cure for myasthenia gravis: A far fetched reality? National Hospital of Sri Lanka, Institute of Neurology, Colombo, Sri Lanka G. Panandikar, S. Ravat Seth GSMC & Kem Hospital, Neurology, Mumbai, India Introduction Multiple sclerosis (MS) is a demyelinating disorder of the CNS which is an emerging neurological problem in Sri Lanka. We looked Background at treatment responses and disability outcomes of a cohort of 100 Sri Myasthenia gravis (MG) has a fluctuating and unpredictable Lankan patients with confirmed MS in terms of their EDSS, course. Currently, there are no definite guidelines regarding duration annualized relapse rates (ARR) and time to first relapse (TTFR) of immunotherapy and previous studies show conflicting results regarding prognostic factors affecting remission.

Aim To study pharmacological remission (PR), complete stable remission (CSR) and prognostic factors in myasthenia gravis patients.

Methodology Retrospective observational study in a tertiary neurology center.100 patients fulﬁlling inclusion criteria were included. Remissions (PR, CSR) were analyzed with respect to age and severity at onset, gender, disease duration, antibody levels, thymectomy status and crisis episodes.

Results 32% had PR (n = 32) at some time during disease course.43.75% (n = 14) of these patients eventually relapsed, 56.25% (n = 18) continued to stay in remission on low dose immunotherapy. Most of ARTICLE IN PRESS

248 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

scale75%

Objective WCN19-0991 To determine the disability outcomes and overall prognosis of Sri Lankan MS patients in terms of their demographic, clinical, radiological and treatment status. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Methods demyelinating diseases/neuromuscular disorders/vestibular dis- 100 conﬁrmed MS patients (McDonald 2010 & MAGNIMS 2016) orders and nutritional disorders or intoxications attending the MS clinic at the National Hospital of Sri Lanka were randomly assessed using direct interviews and patient registries. Antibiotic-induced relapse of nonthymomatous myasthenia AQP4-IgG and MOG-IgG serology was negative in all. gravis with previously post-thymectomy complete remission

Results Y.M.T. Siahaana, A. Sutantoa, W. Raffaelob Majority were females (63%). Average age at onset was 29Y. aSiloam Hospitals Lippo Village, Neurology, Tangerang, Indonesia Predominantly RRMS (75%). Mean EDSS 2.8 (Mean duration of bFaculty of Medicine, Pelita Harapan University, Neurology, Tangerang, b follow-up 7.9y) with 81% having EDSS 3.5. EDSS was worse with Indonesia longer disease duration (p=0.072) and significantly better in those without cerebellar lesions (EDSS 2.5vs3.7; p=0.004), brain stem Introduction lesions (EDSS 2.6vs3.2;p=0.05) and 5 or less MRI lesions (EDSS Myasthenia Gravis (MG) is an autoimmune disorder of neuro- 2.3vs3.3;p= 0.0127). Those with a higher ARR were more likely to muscular transmission with a potentialy devastating outcome if not be on Disease-modifying-Treatment (DMT) but it didn’tinfluence treated carefully. Thymectomy has been long proposed as one EDSS. ARR reduced with increasing disease duration but TTFR didn’t. promising treatment option with better clinical outcome even in The number of brain lesions had no influence on ARR or TTFR. Out of nonthymomatous myasthenia gravis group. Despite the complete 58% patients initiated on DMTs, 83% had no relapse thereafter. remission condition after thymectomy, patients are still at risk of relapse events, in which several drugs, including types of antibiotics Conclusion have been associated with its induction or exacerbation. MS patients in Sri Lanka had reasonably good outcomes in terms of disability and progression perhaps due to early diagnosis and Objective treatment. To present a case of successfully treated antibiotics associated relapse in post-thymectomy nonthymomatous Myasthenia Gravis Keywords: Sri Lanka, Multiple sclerosis, Treatment, Disability patient who previously has achieved complete remission. outcomes Case A 15-year-old male presented with complaints of progressive double vision, bilateral ptosis, and fluctuating general weakness. No doi:10.1016/j.jns.2019.10.1262 thymoma or other mediastinal mass were found on chest CT scan. His symptoms showed only slight improvement and his condition ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 249 deteriorate despite treatment given. He then underwent plasma levels of all the vitamins as compared to paucibacillary cases exchange and total thymectomy in which he achieved complete (Pb0.05). Lepromatous leprosy cases showed lowest vitamin levels. remission for four years. He returned with his original complaints after taking cefixime and clindamycin. Conclusion Vitamin levels in leprosy is lower indicating nutritional deficien- Results cies. This deficient state may be an important risk factor for leprosy Our examinations yield no possible other causes that may become susceptibility. his relapse trigger. He was then stopped from his antibiotics and treated with pyridostigmine 120 mg daily for two consecutive Keywords: Leprosy, Vitamin, Neuropathy, Mycobacterium leprae months and once again achieved complete remission without further medication.

Conclusions doi:10.1016/j.jns.2019.10.1264 Medication in patient with Myasthenia Gravis should be carefully considered even in patients who have achieved complete remission as induction and exacerbation may still be possible. WCN19-0998 doi:10.1016/j.jns.2019.10.1263

Poster shift 03 - Autonomic nervous system disorders/history of Unihemispheric demyelination-A rare variant of acute dissemi- neurology/motor neuron disease/movement disorders/MS & nated encephalomyelitis demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications S.C.J. Chandran Kerala Institute of Medical Sciences, Neurology, Thiruvananthapuram, Vitamin A, C, D, E and B12 levels in leprosy: A case control study India

N. Kumara, H.S. Malhotraa, V. Garga, R.K. Garga, K.P. Malhotrab ADEM is a monophonic inflammatory demyelination of the aKing George's Medical University, Neurology, Lucknow, India central nervous system, characterized by scattered focal or multifocal bDr. Ram Manohar Lohia Institute Of Medical Sciences, Pathology, (disseminated) inflammation of the brain and/or spinal cord. We Lucknow, India describe three cases of unihemispheric involvement in ADEM. Case 1 A 30-year-old male presented with acute vomiting, right Abstract gaze palsy, right LMN facial palsy, and right cerebellar signs. MRI Background brain showed T2, FLAIR hyperintense lesions involving right half of Vitamins are essential part of nutrition and well-being. Many brainstem, right cerebellum, and posterior limb of the right reports indicate that vitamins may influence the etio-pathogenesis of internal capsule. CSF study was normal. He improved with leprosy. steroids. Case 2 An 8- year - old boy presented with left focal to secondary Aims generalized seizures and left hemiplegia. He was in coma. MRI Brain We aimed to compare blood vitamin (A, C, D, E and B12) levels in showed T2, FLAIR hyperintense lesions involving right half of recently diagnosed leprosy patients with healthy controls. brainstem, right cerebellum, thalamus, basal ganglia, right cortical gray matter and subcortical white matter. CSF showed elevated Methods protein without pleocytosis. He was intubated and was given pulse We enrolled 60 newly- diagnosed patients of leprosy. We also methylprednisolone anti edema measures and levitiracetam. He included age and sex matched control subjects. All patients were worsened and expired. categorized according to World Health Organization classification Case 3 A 30 year old man presented with right focal seizures and and the Ridley Jopling classification of leprosy. Vitamins A, C, D, E right hemiparesis. MRI Brain showed T2 FLAIR hyper intensities and B12 levels were measured by enzyme-linked immunosorbent involving left frontal temporal and parietal subcortical white matter assay. and left basal ganglia with micro hemorrhages. CSF study was normal. He improved with steroids. Results Unilateral involvement is rare in ADEM. Relevant literature and Out of 60, 55% patients had paucibacillary leprosy whereas 45% differentiation from other unilateral inflammatory diseases will be had multibacillary. Mononeuritis multiplex was the commonest discussed. electrophysiological finding. The levels of vitamin A, C, D, E and B12 were significantly lower in leprosy cases as compared to healthy doi:10.1016/j.jns.2019.10.1265 controls (Pb0.05). Multibacillary cases showed significantly lower ARTICLE IN PRESS

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WCN19-1005 R. Kulkarni, S. Pujari Deenanath Mangeshkar Hospital and Research Center, Neurology, Pune, India Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Introduction demyelinating diseases/neuromuscular disorders/vestibular dis- Myasthenia gravis (MG) is adisorder neuro-muscular junction where orders and nutritional disorders or intoxications 50-85% patients have acetylcholine receptor antibody (AChR-Ab). About 30-40% sero-negative patients have antibodies to muscle-speciﬁc Character changes as a predictor of Parkinson’s disease in persons tyrosine kinase (MuSK). This disorder represents a distinct subset of of Uzbek nationality MG where clinical features and treatment options are different.

R. Matmurodov, K. Khalimova, E. Abdukodirov Methods Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan We retrospectively analysed MuSK-MG patients seen at our center in last 4 years. Parkinson's disease (PD) is a very topical problem not only in Uzbekistan, but around the world. Non-motor disturbances may be Results predictors of (PD), including character changes. From 2015 to 2019, there were 6 MuSK-MG patients. Eighty-three percent were female, with disease onset in the fourth to ﬁfth decade. Objective Oculobulbar symptoms were present at onset in 83%. Eventually To study the character changes of patients with PD in persons of ocular involvement was seen in 83%, bulbar in 83% and limbs Uzbek nationality. weakness in 83%. Fifty percent patients had myasthenic crisis. No patient had antibodies to AchR and of those tested, CT scan of Material and methods thorax did not show any thymic tumour. We examined 24 patients with PD at the age of 33 to 65 years No patients responded to pyridostigmine. Sixty six percent (mean age 46.7±8.1 years), 12 men and 12 women. The control patients had moderate to good response to steroids. Sixty six percent group consists of 10 patients, without parkinsonism. All patients patients were treated with plasma exchange (PLEX) which produced underwent clinical, neurological, neuropsychological studies. improvement while none was treated with intravenous immunoglobulin. Immunosupression with azathioprine (50%), mycopheno- Results late (16%) and rituximab (50%) was used during the course of illness. In 24 patients with PD, changes in the character of patients were All patients achieved good remission over a median follow up of 2 analyzed before and after the manifestation of the main motor years. symptoms, such as tremor, hypokinesia and rigidity. In 16 patients (62.5%), changes in character, as the intrusiveness began a few years Conclusions (10-15 years) earlier than tremor, hypokinesia and rigidity. At the MuSK-MG has a marked female predominance with frequent same time, of these 10 patients, disturbances of touch and sleep oculobulbar weakness and crises. Pyridostigmine does not help to disorders were observed. Speech at these patients became very relieve symptoms and PLEx shows better results compared to monotonous and they began to speak in incomprehensible words. In steroids. Most patients need immunosuppression and remission can patients without disturbance of touch and sleep disturbance, which be achieved with it. only character changes had, the main motor symptoms of PD were doi:10.1016/j.jns.2019.10.1267 also observed, such as tremor, hypokinesia and rigidity. In these patients, akinetic-rigid form was observed more than a tremor form. All these patients were observed slow-progressive course of the disease. WCN19-1010 Conclusion Based on this, it can be predicted that changes in the character of Poster shift 03 - Autonomic nervous system disorders/history of a person, like intrusiveness, can be a predictor of PD in people of neurology/motor neuron disease/movement disorders/MS & Uzbek nationality. demyelinating diseases/neuromuscular disorders/vestibular dis- doi:10.1016/j.jns.2019.10.1266 orders and nutritional disorders or intoxications

Phosphorylated neuroﬁlament heavy chain (PNF-H) and motor function achievement in nusinersen-treated individuals with WCN19-1007 spinal muscular atrophy (SMA)

C.J. Sumnera, B.T. Darrasb, F. Muntonic, T.O. Crawfordd, R.S. Finkele,E. Poster shift 03 - Autonomic nervous system disorders/history of Mercurif, D.C. De Vivog, M. Oskouih, E.F. Tizzanoi, M.M. Ryanj, Y. Liuk, neurology/motor neuron disease/movement disorders/MS & M. Petrillol, C. Stebbinsl, E. Koenigm, S. Fradettem, W. Farwellm demyelinating diseases/neuromuscular disorders/vestibular dis- aJohns Hopkins University School of Medicine, Departments of Neurol- orders and nutritional disorders or intoxications ogy and Neuroscience, Baltimore, MD, USA bBoston Children’s Hospital, Department of Neurology, Boston, MA, USA c Anti-musk antibody myasthenia gravis: Clinical ﬁndings and long UCL Great Ormond Street Institute of Child Health & Great Ormond term follow up Street Hospital Foundation Trust, Dubowitz Neuromuscular Centre, London, United Kingdom ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 251 dJohns Hopkins University School of Medicine, Departments of Neurol- WCN19-1012 ogy and Pediatrics, Baltimore, MD, USA eNemours Children’s Hospital, Division of Neurology, Department of Pediatrics, Orlando, FL, USA Poster shift 03 - Autonomic nervous system disorders/history of fUniversità Cattolica del Sacro Cuore, Department of Paediatric neurology/motor neuron disease/movement disorders/MS & Neurology, Rome, Italy demyelinating diseases/neuromuscular disorders/vestibular dis- gColumbia University Irving Medical Center, Departments of Neurology orders and nutritional disorders or intoxications and Pediatrics, New York, NY, USA h McGill University, Department of Pediatrics and Department of Apigenin loaded phospholipid based nanoemulsion in therapeu- Neurology and Neurosurgery, Montreal, Canada tics of Parkinson's disease via attenuation of oxidative stress and i Hospital Vall d’Hebron and CIBERER, Department of Clinical and upregulation of dopamine Molecular Genetics, Barcelona, Spain j ’ Royal Children s Hospital University of Melbourne and Murdoch M. Rahmana, V. Kumarb ’ Children s Research Institute and University of Melbourne, Department aFHS- SHUATS, Pharmaceutical Sciences, Allahabad, India of Neurology, Parkville, Victoria, Australia bFhs-Shuats, Pharmaceutical Sciences, Allahabad, India kBiogen, Biostatistics, Cambridge, MA, USA lBiogen, Biomarker Development and Bioanalytical Sciences, Cambridge, Objective MA, USA Parkinson’s disease (PD) is a neurodegenerative disorder it is mBiogen, Clinical Development, Cambridge, MA, USA linked with the oxidative stress, neuroinflammation and apoptosis. The aim of the present study was to develop, optimizes and evaluate Background the antiparkinson effects of apigenin loaded phospholipid based fi Elevated neuro lament levels have been detected in neurode- nanoemulsion via attenuation of oxidative stress and upregulation of fi generative disorders. We assessed phosphorylated neuro lament dopamine. heavy chain (pNF-H) levels in individuals from nusinersen clinical trials with presymptomatic, infantile-onset, or later-onset SMA, and Backgrounds volunteers without SMA, and investigated the association with Apigenin (AP) is a flavone enriched in fruits and vegetables, motor function achievement in nusinersen-treated individuals. which possess numerous therapeutic actions including anti-apopto- tic, anti-inflammatory and free radical scavenging activities. Methods fi ™ pNF-H plasma levels were quanti ed using the ProteinSimple Methods SimplePlex ELLA immunoassay. Motor function assessments in The antioxidant action was evaluated by 1,1-diphenyl-2-picryl- individuals with presymptomatic and infantile-onset SMA included hydrazyl (DPPH) and reducing power assay, which founds high CHOP INTEND. Motor function assessments in individuals with later- scavenging efficiency for apigenin loaded phospholipid based onset SMA included HFMSE and RULM. Regression analyses were nanoemulsion as compared to pure apigenin. The pharmacokinetic performed to assess pNF-H levels (or declines) and motor function parameters and Dopamine concentration were also determined. achievement associations. Patient consent/IRB approval were obtained. Results The said formulation delivers higher concentration of apigenin to Results brain via nasal route. Whereas the Biochemical estimation evaluation Baseline pNF-H levels were higher in individuals with SMA (n = showed that higher levels of antioxidant enzymes including 302) than age-matched controls without SMA (n = 34). In treated glutathione, superoxide dismutase and lower level of thiobarbituric individuals with SMA, plasma pNF-H levels declined during the acid reactive substances in treated group of rats after receiving of nusinersen loading period then stabilized at lower levels through said formulation via intranasal route over haloperidol-induced latest observed visits (infantile-onset SMA: Day 302; later-onset Parkinson's disease group (control). The intranasal administration SMA: Day 456). Lower absolute pNF-H levels (presymptomatic and of said formulation founds brain: blood ratio of 3.01 N 0.097 in 1 h infantile-onset SMA participants) or greater decrease in pNF-H levels over apigenin solution respectively. Furthermore, it showed direct from baseline (later-onset SMA participants) during the loading nose to brain delivery of drug by passing blood brain barrier. Apart phase were associated with greater improvement in motor function from this, the pharmacokinetics of intranasally administered at Day 302 and at Day 456, respectively, after controlling for age and apigenin loaded phospholipid based nanoemulsion showed signifi- baseline motor function. cantly higher dopamine concentration (19.21 ± 2.06 ng/mL) over haloperidol treated rats (7.21 ± 1.06ng/mL). Conclusions pNF-H levels were elevated at baseline in individuals with SMA. Conclusion Nusinersen treatment reduced pNF-H to lower levels; lower levels or The apigenin loaded phospholipid nanoemulsion might play an greater decrease in pNF-H levels during the nusinersen loading important role in the effective management of parkinsons in the near phase appears to be associated with greater improvement in future. subsequent motor function response. Further evaluation is warranted. Originally presented at CureSMA 2019. doi:10.1016/j.jns.2019.10.1268 doi:10.1016/j.jns.2019.10.1269 ARTICLE IN PRESS

252 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-1013 WCN19-1018

A blind computerized analysis. Effect of subthalamic deep brain Fabrication of solid lipid nanoparticles containing vitexin pro- stimulation on posture in Parkinson's disease tects dopaminergic neurons against 6-hydroxydopamine induced Parkinson's disease model via altered the genetic backgrounds M. Rakhimova, B. Sultanov TASHIUV, Neurology, Tashkent, Uzbekistan M. Rahman, V. Kumarb aFHS- SHUATS, Pharmaceutical Sciences, Allahabad, India b Introduction Fhs-Shuats, Pharmaceutical Sciences, Allahabad, India^ We sought to assess the effect of subthalamic deep brain stimulation (STN DBS) on Parkinson´s disease (PD)-associated Objectives^ postural abnormalities. Vitexin is a flavonoid which obtained from Crataegus pinnatifida Bunge. Further, its neuroprotective effects are examined in PD model. Methods The motive of the current study was to fabricate vitexin loaded solid A computerized analysis of posture was used to quantify the lipid nanoparticles and evaluate the effect against 6- thoracolumbar, thoracic, and cervical-occipital ventral angles, as hidroxidopamine (6-OHDA) induced parkinson's disease animal well as the thoracolumbar and cervical-occipital lateral angles model.^ from the video-repository of three specialized movement disorder centers (n = 158 patients). Data was extracted from frames Materials and methods from video-recordings in the pre-surgical medication-ON (dopa- Intracerebroventricular injection of 6-OHDA was used for induc- minergic therapy) and post-surgical stimulation-ON/medication- tion the PD and mice were treated with the said drug loaded fi ON states (STN DBS plus dopaminergic therapy). The sum of the nanocarrier for 28 ^days. In our study, hot melt emulsi cation and five postural angles (global postural angle) was used to ultrasonication method was employed for the preparation of Vitexin compare pre-vs. post-surgical trunk posture alterations. A multi- loaded nanolipoidal carrier. Further, the enzymatic activities incudes variate analysis was used to examine the association between Catalase (CAT), superoxide dismutase (SOD), glutathione (GSH) changes in the postural angles and demographic or clinical glutathione peroxidase (GPx), glutathione S-transferase (GST), level variables. of reactive oxygen species, total reactive antioxidant dopamine and its metabolite homovanilic and 3, 4-dihydroxyphenylacetic acid, Results were determined in the striatum. Furthermore, the behavioural There was a 6.7% amelioration in the global postural angle parameters including locomotor, memory and depressive were between the pre- and post-surgical assessments (p = 0.031). estimated.^ Motor response to and pre-surgical dosage of levodopa, male gender, and shorter PD duration were identified as predictors for Results posture improvement after STN DBS. Cases meeting criteria for The Vitexin loaded solid lipid nanoparticles therapeutics in 6- lower (n = 2) or upper (n = 1) camptocormia respectively OHDA induced Parkinson's, results clearly showed improvement in improved by 48.1% in the ventral thoracolumbar angle (from total reactive antioxidant, CAT, GPx, Dopamine and its metabolite 36.4 ± 0.0° to 18.9 ± 4.2°) and 13.8% in the ventral thoracic angle level in striatum of mice. We also observed that reactive oxygen (from 49.1° to 42.3°). Cases meeting criteria for Pisa syndrome (n species in the striatum were diminished by solid lipid nanoparticles = 2) improved by 67.5% in the lateral thoracolumbar angle (from of Vitexin. Furthermore, the said formulation is well efficient in 16.9 ± 2.0° to 5.5 ± 4.7°). averting the memory destruction in Morris water maze test as well as depressive like behaviour in tail suspension test.^ Conclusions fi STN DBS has a relatively small but signi cant effect on PD- Conclusion^ associated postural abnormalities, potentially enhancing the effect of Vitexin loaded solid lipid nanoparticles founds protective effects dopaminergic medications alone. in neurodegeneration on 6-OHDA induced neurotoxicity in mice. In features, this could be alternative effective therapeutic approach for the management of PD.^ doi:10.1016/j.jns.2019.10.1270 doi:10.1016/j.jns.2019.10.1271 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 253

WCN19-1025 Conclusions ^ Continued analysis of data from children treated with nusinersen via the SHINE study will increase the information available on the Poster shift 03 - Autonomic nervous system disorders/history of long-term safety/tolerability and efﬁcacy of repeated nusinersen neurology/motor neuron disease/movement disorders/MS & doses. Originally presented at AAN 2019. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1272

Interim report on the safety and efﬁcacy of longer-term treatment with nusinersen in later-onset spinal muscular atrophy (SMA): Results from the shine study WCN19-1035

J. Kirschnera, B.T. Darrasb, M.A. Farrarc, E. Mercurid, C.A. Chiribogae,N. Poster shift 03 - Autonomic nervous system disorders/history of L. Kuntzf, P.B. Shiehg, M. Tuliniush, J. Montesi, S.P. Reynaj,G. neurology/motor neuron disease/movement disorders/MS & Gambinok, R. Fosterk, I. Bhanl, J. Wongj, W. Farwellj aUniversity Hospital Bonn, Department of Neuropediatrics, Bonn, demyelinating diseases/neuromuscular disorders/vestibular dis- Germany orders and nutritional disorders or intoxications bBoston Children's Hospital, Department of Neurology, Boston, MA, USA cSydney Children's Hospital Network and UNSW Medicine, Paediatric First report of neuromyelitis optica in a mother-daughter pair Neurology, Sydney, Australia from Iran dUniversità Cattolica del Sacro Cuore, Department of Paediatric Neurology, Rome, Italy S. Esmaeili, F. Haji Akhondi, B. Zamani, M. Mojtahed eColumbia University Irving Medical Center, Department of Neurology Iran University of Medical Sciences, Neurology, Tehran, Iran^ and Pediatrics, New York, NY, USA f Ann & Robert H. Lurie Children's Hospital of Chicago, Division of Background Neurology, Chicago, IL, USA Most cases of Neuromyelitis Optica (NMO) seem to be sporadic. g UCLA Clinical and Translational Research Center, Department of However, Rare reports of familial aggregation have suggested a Neurology, Los Angeles, CA, USA component of genetic susceptibility. Reported cases were mostly h Gothenburg University and The Queen Silvia Children's Hospital, siblings who developed the disease at similar ages. There has been Department of Pediatrics, Gothenburg, Sweden only few reported case in which family members developed the i Columbia University Irving Medical Center, Departments of Neurology disease at different stages of life.This is the ﬁrst report form Iran. and Rehabilitation and Regenerative Medicine, New York, NY, USA j Biogen, Clinical Development, Cambridge, MA, USA Methods k Biogen, Biostatistics, Maidenhead, United Kingdom We report a case report mother-daughter pair diagnosed with l Biogen, Drug Safety, Cambridge, MA, USA^ NMO and treated at the Iran University of Medical Sciences. We describe the demographic, clinical, neuroimaging, and NMO-IgG Background serologic status. ^Our objective is to report interim results from the SHINE study (NCT02594124) for participants with later-onset SMA (most likely to Results develop Type II or III SMA) who transitioned from CHERISH. Iranian Mother and Daughter were diagnosed with NMO with different presentations and different ages at onset. Mother developed Methods transverse myelitis along with optic neuritis at age of 54 and the SHINE is an open-label extension for previous nusinersen study daughter developed optic without pyramidal symptoms After participants. Safety/tolerability is the primary endpoint; secondary extensive at age of 30. endpoints include WHO motor milestone achievement, Hammersmith Functional Motor Scale-Expanded (HFMSE), and Conclusions Revised Upper Limb Module. These integrated analyses focus on Our familial case demonstrate that NMO may differ clinically children treated with nusinersen or sham control in CHERISH who within a family. This report suggests complex genetic susceptibility transitioned to SHINE. Patient consent/IRB approval were obtained. in NMO.

Results doi:10.1016/j.jns.2019.10.1273 In CHERISH, 84 participants received nusinersen and 83 transitioned to SHINE; all 42 participants in the sham control group transitioned. Baseline data are presented for 3 groups: previous sham control (CHERISH data; n = 42); previous sham control/SHINE data WCN19-1047 (n = 42); previous nusinersen in CHERISH/SHINE data (n = 84). Median (range) age at ﬁrst dose/sham procedure was 43.3 (25–90), 58.2 (40–107) and 49.7 (25–111) months, and median age at Poster shift 03 - Autonomic nervous system disorders/history of symptom onset was 11.0 (6–20), 11.0 (6–20), and 10.0 (6–20) neurology/motor neuron disease/movement disorders/MS & months; 50%, 50% and 55% were female, respectively. Mean baseline demyelinating diseases/neuromuscular disorders/vestibular dis- (SD) WHO motor milestones was 1.5 (1.02), 1.4 (1.11) and 1.4 orders and nutritional disorders or intoxications (0.96); mean (SD) HFMSE score was 19.9 (7.23), 19.8 (8.39), and 22.4 (8.33), in the 3 groups respectively. Results from an interim Apomorphine induced immune hemolytic anemia analysis with a 15 October 2018 data cutoff will be presented. ARTICLE IN PRESS

254 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

S. Esmaeili, G. Shahidi, F. Hajiakhondi differentiating inflammatory and non-inflammatory aetiologies If Iran University of Medical Sciences, Neurology, Tehran, Iran there is an underlying inflammatory cause they are at high risk of further attacks. So they may require long term immuno suppression Drug‐induced immune hemolytic anemia (DIIHA) is not a to prevent further attacks. frequent complication yet has threatening consequences. Managing patients with parkinsonism, L-Dopa was first described as a drug doi:10.1016/j.jns.2019.10.1275 which directly cause DIHA. Very few confirmed cases have been reported for the same adverse effect of other dopaminergic drugs such as apomorphine and cabergoline. Hereby, We Describe a patient with severe parkinsonism for whom Apomorphine was started as the WCN19-1058 last choice since he was resistant to high dose L-Dopa. He developed pallor and jaundice and further work up revealed DIHA at very early Poster shift 03 - Autonomic nervous system disorders/history of stages of administration of the drug at Intensive Care Unit. The drug was withdrawn for the prevention of serious adverse effects. Strong neurology/motor neuron disease/movement disorders/MS & clinical suspicion is required to detect this rare complication. proper demyelinating diseases/neuromuscular disorders/vestibular dis- immunohematological test and prompt management is warranted orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1274 Nusinersen in infants who initiate treatment in a presymptomatic stage of spinal muscular atrophy (SMA): Interim results from the phase 2 nurture study

WCN19-1052 M.M. Ryana, D.C. De Vivob, E. Bertinic, W.L. Hwud, T.O. Crawforde, K.J. Swobodaf, R.S. Finkelg, J. Kirschnerh, N.L. Kuntzi, J. Parsonsj, R.J. Butterfieldk, H. Topaloglul, T. Ben Omranmn, V.A. Sansoneop, Y.J. Jongq, Poster shift 03 - Autonomic nervous system disorders/history of F. Shur, R. Fosters, I. Bhant, S. Fradetteu, W. Farwellu neurology/motor neuron disease/movement disorders/MS & aRoyal Children's Hospital and University of Melbourne and Murdoch demyelinating diseases/neuromuscular disorders/vestibular dis- Children's Research Institute, Department of Neurology, Melbourne, orders and nutritional disorders or intoxications Australia bColumbia University Irving Medical Center, Departments of Neurology A case of longitudinally extensive viral myelitis. Clinical, radio- and Pediatrics, New York, NY, USA logical profile and outcome cPost-Graduate Bambino Gesù Children's Research Hospital, Unit of Neuromuscular and Neurodegenerative Disorders, Rome, Italy d D.R. Rajendrana, G. Prakashb, J. Leenac, C. GovindaRajud, M.P. Ranjithe National Taiwan University Hospital, Departments of Medical Genetics aAlbustan Speciality Hospital, Neurology, Abudhabi, United Arab and Pediatrics, Taipei, Taiwan, ROC e ^^ Emirates Johns Hopkins University School of Medicine, Department of Neurology, bAlbustan Speciality Hospital, Radiology, Abudhabi, United Arab Emir- Baltimore, MD, USA f ates Massachusetts General Hospital, Center for Genomic Medicine, Boston, cAlbustan Speciality Hospital, Pathology, Abudhabi, United Arab Emir- MA, USA g ates Nemours Children's Hospital, Department of Pediatrics, Orlando, FL, dAl Ahli Hospital, Neurology, Abudhabi, United Arab Emirates USA h eAl Ahli Hospital, Cardiology, Abudhabi, United Arab Emirates University Hospital Bonn, Department of Neuropediatrics, Bonn, Germany i This case report provides a comprehensive clinical and radiolog- Ann & Robert H. Lurie Children's Hospital of Chicago, Division of ical profile of a case of Longitudinally extensive transverse myelitis Neurology, Chicago, IL, USA j (LETM) in middle east Asia. This 37 year old Indian manual labourer University of Colorado School of Medicine, Department of Pediatrics, presented with acute onset paraplegia with early bladder involve- Aurora, CO, USA k ment, without any preceding febrile illness. His MRI showed a long University of Utah, Department of Pediatrics and Neurology, Salt Lake segment T2 heterogeneous hyperintensity in the spinal cord City, UT, USA l extending from C3 to D11 level involving predominantly the central Hacettepe University, Department of Pediatric Neurology, Ankara, grey matter. The adjacent white matter was also involved in the Turkey m upper dorsal level. Mild cord edema was present. On contrast Sidra Medicine Qatar Foundation, Department of Pediatrics, Doha, administration, there was no significant enhancement. Patient was Qatar n evaluated for the possible etiologies which were negative. But Hamad Medical Corporation, Division of Clinical and Metabolic neutrophilia in CSF with normal glucose and protein suggest a Genetics and Department of Pediatrics, Doha, Qatar o probable unknown viral etiology. If no cause is identified after a University of Milan, Neuromuscular Omniservice, Milan, Italy p thorough work-up, transverse myelitis can also be categorized as an Università degli Studi di Milano, Department of Biomedical Sciences for idiopathic monophasic event, which occurs in approximately 15–30% Health, Milan, Italy q of cases. We did not obtain a spinal angiogram to definitively rule out Kaohsiung Medical University, Graduate Institute of Clinical Medicine, a spinal arteriovenous shunt, since the clinical response was good Kaohsiung, Taiwan, ROC r ^^ and gradient did not show any blooming. So our patient probably is a David Geffen School of Medicine at UCLA, Department of Neurology, Los case of LETM of unknown viral etiology or idiopathic who has Angeles, CA, USA s recovered well with steroids. Patients presenting with LETM require Biogen, Biostatistics, Maidenhead, United Kingdom t a thorough work-up to exclude other treatable infectious and Biogen, Drug Safety, Cambridge, MA, USA u inflammatory causes. The management of LETM is dependent on Biogen, Clinical Development, Cambridge, MA, USA ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 255

Background Material-methods Nusinersen is the first approved treatment for SMA. We present We examined 94 patients with multiple sclerosis. Age varied from interim results from the ongoing NURTURE study (NCT02386553) 20 to 45 (30,2 ± 1,63). Of these, 72% (58) women, 28% (36) men. All examining efficacy/safety of intrathecal nusinersen, initiated prior to patients underwent MRI scans, the Kurzke scale and EDSS. symptom onset, in infants with 2 or 3 SMN2 copies. Results Methods It was divided into groups depending on the clinical course, Enrolled infants were age ≤ 6 weeks at first dose, clinically which were clinically radiologically associated (according to MRI presymptomatic, and genetically diagnosed with SMA. Primary data). They are primary progressive (PP)-6(6%), secondarily pro- endpoint is time to death or respiratory intervention (≥6 h/day gressive (SP)14(15%), remitting course (RC) 59(63%), clinically continuously for ≥7 days or tracheostomy). Patient consent/IRB isolated (CIS) 4(4%)and radiologically isolated syndromes (RIS)11 approval were obtained. (12%). RIS and ICC were not clinically radiologically associated. Symptoms of numbness in the face and extremities (sensory motor Results manifestations like goosebumps, numbness, tremor, trembling)) in As of 15 May 2018, 25 infants (2 copies SMN2 [n = 15]; 3 copies PPC-2(25%), SPC-7(57%), RC-37(63%), RIS-10(98%), CIS-3(75%). But [n = 10]) were enrolled. Median age at last visit was 26.0 on the Kurzke scales it was point PPC-10,2 ± 0,32, SPC-8 ± 0,5, RC- (range:14.0–34.3) months. All infants were alive and none required 7,3 ± 0,29, RIS-0,6 ± 0,17-CIS-2,8 ± 0,27. permanent ventilation. Median time to death or respiratory intervention could not be estimated because of too few events. Four Conclusion infants (all 2 SMN2 copies) required respiratory intervention for ≥6 1. sensory manifestations as chills, numbness, tremors, trembling h/day continuously for ≥7 days, with all cases initiated during acute, were observed in all forms of multiple sclerosis due to the clinical reversible illness. All infants achieved the WHO motor milestone debut, which was a precursor of the disease. sitting without support and 22/25 (88%) achieved walking with 2. The presence of a functional impairment on the Kurtzk scale assistance; 17/22 (77%) were walking alone. Phosphorylated neuro- affected disability in all forms, in contrast to RIS and CIS, which filament heavy chain levels rapidly declined during the nusinersen scores remained closer to the norm, which are considered loading phase and then stabilized. AEs occurred in all infants; 20/25 outpatient patients. had mild/moderate AEs; 9 had SAEs. No new safety concerns were identified. Results from a new, Spring 2019 interim analysis, doi:10.1016/j.jns.2019.10.1277 including additional assessments, will be presented.

Conclusions There was continued beneﬁt to infants who initiated nusinersen WCN19-1064 before symptom onset, emphasizing the value of early treatment and newborn screening. Updated analyses will provide further information. Originally presented at CureSMA 2019. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1276 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Longitudinal gait analysis in patients with chronic inﬂammatory WCN19-1063 demyelinating polyradiculoneuropathy

Poster shift 03 - Autonomic nervous system disorders/history of I. Bozovic, S. Peric, I. Basta, V. Rakocevic-Stojanovic, A. Kacar, D. Lavrnic, Z. Stevic, S. Radovanovic neurology/motor neuron disease/movement disorders/MS & Neurology Clinic, Neuromuscular and Spinal Cord Disorders, Belgrade, demyelinating diseases/neuromuscular disorders/vestibular dis- Serbia orders and nutritional disorders or intoxications Introduction С linical feature of sensorimotor manifestations as a leading Chronic inﬂammatory demyelinating polyradiculoneuropathy predictor of radiological isolated syndrome in multiple sclerosis (CIDP) is a chronic disorder which may lead to many functional impairments, including gait abnormalities. Our aims were to analyse I. Ganieva, Y. Parpieva, K. Khalimova, M. Yakubova gait characteristics in patients diagnosed with CIDP compared to Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan healthy controls (HC) and to determine changes of gait parameters after six months follow-up period. Relevance To date, the clinical and prognostic signiﬁcance of the clinical Method symptoms in patients with Radiologically Isolated Syndrome (RIS) Twenty-four CIDP patients and 24 HCs performed a basic walking remains controversial. However, it is irrefutable that patients with task, dual-motor task, dual-mental task, and combined (motor and RIS belong to the group with an increased risk of developing reliable mental) task using the GAITRite System. MRC sum score (MRC-SS) Multiple Sclerosis and this problem remains one of the most pressing and INCAT scores were assessed in all patients. Fourteen patients in neurology. were retested after six-month.

Objective Results To analyze the clinical sensory-motor manifestation as an early The most consistent ﬁndings in CIDP patients vs. HCs in all predictor in radiologically isolated syndrome. walking tasks were shorter stride length (SL) and prolonged cycle ARTICLE IN PRESS

256 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx time (CT) due to the prolonged double support time (DS), as well as increased variation of SL and of swing time (ST) (p b 0.01). After six- month, none of gait parameters changed signiﬁcantly (p N 0.05). The changes of CT and its variation in combined task signiﬁcantly differentiated patients with improved vs. non-improved INCAT (p b 0.01). Change in lower limb INCAT score correlated with the change of CT variation during combined task (rho=+0.75, p b 0.01). In subgroup of patients with improved INCAT, improvement in MRC-SS correlated with decreased variation of CT during basic walking task and decreased variation of SL during combined task (rho=−0.84 and rho=−0.91, p b 0.01).

Conclusion CIDP patients had slower gait with prolonged DS and with shorter SL compared to HCs. Functional improvement was in correlation with the shorter CT and with less variation of CT. doi:10.1016/j.jns.2019.10.1278

Conclusion The results of the healing processes show that the method could WCN19-1065 be applied not harmfully on other patients. Once triggered the healing processes mobilize a chain of recoveries in organs and Poster shift 03 - Autonomic nervous system disorders/history of systems throughout the entire human body. neurology/motor neuron disease/movement disorders/MS & Keywords: Methods, Treatment, Translational neuroscience demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1279

The true sight the best discovery in the history in the word not only for the medicine, for the people, at all WCN19-1072 G. Iliychovski Neurology and Private research. Sofia- Bulgaria, Neurology and Brain Diseases, Sofia, Bulgaria Poster shift 03 - Autonomic nervous system disorders/history of neurology /motor neuron disease/movement disorders/MS & Introduction demyelinating diseases/neuromuscular disorders/vestibular dis- Although the rapid progress in neuroscience in the recent years orders and nutritional disorders or intoxications there are still many unresolved problems in this field. In the current work is presented a newly developed method of Gender aspects of patients with multiple sclerosis in the treatment applied personally on the author. conditions of Uzbekistan

Objective I. Ganieva, Y. Parpieva, K. Khalimova, M. Yakubova, R. Userbaeva Demonstrate the effectiveness of a newly applied healing method Tashkent Medical academy, Neurology, Tashkent, Uzbekistan in the translational neuroscience and induce its wider application.

Methods The purpose of this study was to assess gender characteristics of A new treatment method considering intentional movement of multiple sclerosis in the conditions of Uzbekistan mini-particles throughout the human body, directed consciously by the brain is presented. The string-based movements of the mini- Material and methods particles accomplish healing “removals” from the affected organs and Were examined in38 patients with multiple sclerosis(MS)of systems. The mobilization of the brain triggering the inner healing Uzbek nationality. The age of patients ranged from 18 to 56 years, potential of the body, based on expanded brain functional capacity. average age was 36,0 ± 0.12 years (16 men,22 women). All patients were studied in clinical and neurological, laboratory, para clinical Findings data. The treatment results in healing and recovery of different organs and systems throughout the human body. It led to improvement and Results normalization of the activity of glands of internal secretion. The cyst, The study of the onset of the disease revealed that in all men the located in the center of the cerebrum near the pineal gland, was disease begins with weakness in the limbs and unsteadiness of gait, moved into the lower part of the pelvis. Another one was moved and in women these symptoms was observed in only 64% of patients. from the upper part of the cerebellum. Improvements were observed In addition, in women often the onset of the disease was impaired ’ also in other organs and systems. (husband s(H)19%, wives(W) 24%), and men dizziness ((H)25%, (W) The indicated healing processes, from the relocation of the 14%)The men have slightly less than was observed paresthesia (19%), nervous system to the cysts, were completed in a few days but the headache (19%)and speech disorder (14%),and women paresthesia, healing process is a long-term. headache and dizziness for 14%. Rarely, men and women, the disease ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 257 began with pain in the limbs (respectively 7 and 9%), swallowing (6 WCN19-1083 and 5%), and speech disorders only in men (2%). Further, the study of the average speed of disease progression have shown that MS fast progressed in women (4,9 ± 0,5 years) than in men (6,8 ± 0,8 Poster shift 03 - Autonomic nervous system disorders/history of years)The average duration of first remission in men and women is neurology /motor neuron disease/movement disorders/MS & approximately the same (respectively:10,8 ± 1,5 11,0 ± 1,4years) demyelinating diseases/neuromuscular disorders/vestibular dis- The average number of remissions was also about the same orders and nutritional disorders or intoxications (respectively 4,8 ± 0,7 and 4,4 ± 0,6 years) Among MS patients regardless of gender were more patients from the city ((H),(W) 63– Differential diagnosis of acute shoulder girdle weakness 64%) than from villages(husband is 37 and women – 36%). B. Lee, J.R. Kim, D.H. Sung Conclusion Samsung Medical Center, Department of Physical Medicine and Debut MS men of Uzbek nationality often begins with motor Rehabilitation, Seoul, Republic of Korea and cerebellar disorders.2.In the conditions of Uzbekistan MS progresses faster in women than in men.3.MS as in men, and in Neuralgic amyotrophy (NA), also known as Parsonage-Turner women, more common in those of the urban population than the syndrome, is a disease characterized by pain and subsequent muscle rural. weakness. NA appears in the form of mononeuropathy such as anterior interosseous nerve syndrome, posterior interosseous nerve doi:10.1016/j.jns.2019.10.1280 syndrome, long thoracic neuropathy, and isolated neuropathy of suprascapular nerve, or multiple mononeuropathy, a combination of these. It is also present in aspects of cervical nerve root involvement. It is a diagnosis of exclusion which does not have a clear diagnosis or WCN19-1079 classification criteria. Therefore, the objective of this study was to compare clinical features, images, and electrodiagnostic studies Poster shift 03 - Autonomic nervous system disorders/history of (EDX) of compressive C5 radiculopathy and NA, and to establish the basis for diagnostic criteria of NA. neurology/motor neuron disease/movement disorders/MS & Electronic medical records of 131 patients with shoulder girdle demyelinating diseases/neuromuscular disorders/vestibular dis- weakness without trauma at our institution between August 2011 orders and nutritional disorders or intoxications and January 2019 were reviewed. According to the grouping criteria, we analyzed 12 patients with C5 radiculopathy and 15 patients with A case report of an overlap between motor neuron disease and NA Parkinson disease We found that left-side weakness and pain were more common in NA group and the range of pain was wider in compressive C5 A. Hussein, K.A. Hussein, H. Abbashar, A. Siddig, Y. Alseddig, M. radiculopathy group significantly. However, there was no laboratory, Abbashar, R. Tofaha AlHusseini neurophysiological, or image findings to help differentiate compres- Daoud Research Group, Neurology, Khartoum, Sudan sive C5 radiculopathy and NA. In conclusion, neither MRI nor EDX were significantly helpful in A case report of an overlap between motor neuron disease and distinguishing compressive C5 radiculopathy and NA. The abnormal- Parkinson disease. H. M is a 72 years old Sudanese male. During the ity of distal peripheral nerve may present on MRI in patients with last two years he used to complain of upper and lower limbs compressive radiculopathy. NA should be considered if the proximal weakness. This was not preceded by neck pain, sphincteric portion of nerve root is normal or morphologic change or nerve is disturbance or numbness of extremities. During the course of the found. disease his elderly son noticed that he had slurred speech and difficulty in swallowing. He has no family history of similar condition doi:10.1016/j.jns.2019.10.1282 and his past medical history was insignificant. He looks unwell. The abnormalities were confined to the central nervous system. He is fully conscious, although he was a little bit emotionally labile. He had obvious tongue fasciculation. Both upper WCN19-1087 limbs showed distal muscle wasting and remarkable fasciculation. The tone was hypertonic in both upper limbs while the power was grade two. The reflexes were exaggerated. All modalities of Poster shift 03 - Autonomic nervous system disorders/history of sensations were intact. Coordination couldn't be demonstrated due neurology/motor neuron disease/movement disorders/MS & to the pronounced weakness of the distal parts of both limbs. Both demyelinating diseases/neuromuscular disorders/vestibular dis- lower limbs showed wasting, fasciculation, hypertonia and hyperre- orders and nutritional disorders or intoxications flexia and the power was grade two. All modalities of sensations were intact. Both coordination and the gate cannot be demonstrated Baló's concentric sclerosis: A case report due to the weakness. A clinical diagnosis of motor neuron disease fi was made and this was supported by EMG ndings. During the V.R. Konzena, M.S. Medeirosb, M.C. Augustinc, R.T. Martinsb, H.L. course of the disease the patient showed features of Parkinson Moser Filhob disease on the top of motor neuron disease). aSanta Casa de Misericórdia de Porto Alegre, Neurology, Porto Alegre, Brazil doi:10.1016/j.jns.2019.10.1281 bHospital Nossa Senhora da Conceição, Neurology, Porto Alegre, Brazil cHospital de Clínicas de Porto Alegre, Neurology, Porto Alegre, Brazil ARTICLE IN PRESS

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Background Fig. 1 Typical tumefactive lesion characteristic of BCS over right Balo's Concentric Sclerosis (BCS) is a rare variant of Multiple cerebellum. Sclerosis (MS) characterized by alternating rings of demyelination Fig. 2 Bilateral periventricular and juxtacortical lesions compat- and spared myelin. Traditionally, clinical course of BCS was ible with MS. considered to have a poor outcome. However, recent literature states that BCS often follows a benign course with prolonged doi:10.1016/j.jns.2019.10.1283 survival. Moreover, BCS and MS-like lesions are being increasingly reported to occur simultaneously, indicating that they may represent a continuum of the same disease process. WCN19-1090 Objective We report a case of BCS with clinical and radiological criteria for MS. Poster shift 03 - Autonomic nervous system disorders/history of Material and Methods neurology/motor neuron disease/movement disorders/MS & A 19-year-old man was admitted to hospitalization due to a demyelinating diseases/neuromuscular disorders/vestibular dis- progressive course of right hemiataxia and gait instability, right orders and nutritional disorders or intoxications hemiparesis, diplopia and dysarthria during the prior days. There was no history of fever, infectious diseases, seizures, traumatic brain Late onset of pompe disease, involvement of three brothers: A injury, bladder dysfunction, visual loss or dysphasia. He reported case report similar symptoms with spontaneous resolution three years before. K. Lebeisa, M. Beiral Hämmerleb, C. Bittar Braunec, E. Mellob,R. Results Beltréb, D. Souzab Patient performed an MRI brain scan with contrast that revealed aFederal University of the State of Rio de Janeiro, Internal Medicine, Rio multiple focal brain lesions, one of which highly suggestive of BCS. de Janeiro, Brazil Cerebrospinal fluid was unremarkable, viral and bacterial serologic bFederal University of the State of Rio de Janeiro, Neurology, Rio de tests were normal. He was treated with methylprednisolone for 7 Janeiro, Brazil days with rapid improvement of symptoms. Patient was discharged cFluminense Federal University, Neurology, Niteroi, Brazil from hospital with mild right hemiparesis and hemiataxia and no gait instability. A 49-year-old Brazilian male, experienced progressive weakness to walk, especially to climb stairs and get up since 2013. He denied Conclusion any instances of myalgias, paresthesia, dysphagia, dysarthria, or Patient’s clinical features and MRI findings are highly suggestive dysautonomia. of BCS and MS. Clinicians should consider BCS as a differential diagnosis of tumefactive demyelinating lesions since it may have a Family history better outcome. His two brothers reported difficulty getting up from the ground.

Physical examination Increased lordosis, bilaterally winged scapulae, quadriceps atrophy, Gowers sign, and hypoactive tendon reﬂexes globally. He showed tongue weakness, muscular strength in the lower limbs grade 3/5 during ﬂexion. The muscle strength in the upper limbs was of grade 4/5 during the abduction. He showed normal sensitivity.

Investigation Creatine kinase, lactate dehydrogenase, and aldolase were increased. An electrocardiogram revealed an incomplete right bundle branch block and left atrial hypertrophy. His acid alpha-glucosidase activity measured was 1.4 μmol/L/h (normal N 4), while his sister’s (62 years old ) enzyme level was 0.98 μmol/L/h and his brother’s (51 ARTICLE IN PRESS

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WCN19-1095

Poems’ syndrome associated with autonomic dysfunction: An atypical presentation

K. Lebeisa, M. Beiral Hämmerleb, M. Beiral Hämmerlec, C. Bittar Brauned, M. Ribeiro Caetanoe, L. Bergierf aFederal University of the State of Rio de Janeiro, Internal Medicine, Rio de Janeiro, Brazil bFederal University of the State of Rio de Janeiro, Neurology, Rio de Janeiro, Brazil cFederal University of Rio de Janeiro, Internal Medicine, Rio de Janeiro, Brazil dFluminense Federal University, Neurology, Rio de Janeiro, Brazil ePrivate Clinic, Neurophysiology, Rio de Janeiro, Brazil fFederal University of the State of Rio de Janeiro, Hematology, Rio de Janeiro, Brazil years old) was 1.31 mol/L/h. Enzyme replacement was initiated and the patient showed a slight improvement in muscle strength. A 52-year-old Brazilian man had no previous comorbidities. Four years ago, he exhibited bilateral gynecomastia, sexual dysfunction, Discussion and final comments and weight loss of 20 kg during that period. He exhibited edemigenic Pompe disease (PD) is caused by the partial or total deficiency of syndrome and plantar paresthesia that evolved with tetraparesis. In the enzyme acid alpha-glucosidase activity causing glycogen accu- addition, he exhibited dysautonomia with arterial hypotension, mulation in the cells, with muscular involvement.1 In the United xerostomia, urinary retention, and constipation. States, the prevalence of PD is one in every 28,000 births.2 It is an autosomal recessive disease. The risk of recurrence in the offspring is Physical examination approximately 25%. The diagnosis of PD justifies the active investi- Digital clubbing and lymph node enlargement with mobile and gation of the disease among the siblings.1 We report a case of late- elastic lymph nodes. Gynecomastia, normal testicles, abdominal onset PD with involvement of two relatives in addition to the distension, and absence of hepatosplenomegaly. The tetraparesis indexed patient. was more distally accentuated. The patient experienced tactile and painful hypoesthesia in the lower limbs. Reflexes, sensation to doi:10.1016/j.jns.2019.10.1284 vibration and joint position sense were absent. Fundoscopic examination showed no papilledema. ARTICLE IN PRESS

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Complementary exams remained enough to limit daily activities until one year after stroke. Thrombocytosis, basic biochemistry, and normal serologies. Modified Rankin scale more than 1 point at discharge was Cerebrospinal fluid tests showed increased protein content. Electro- independent poor prognostic factor for post-stroke dizziness. neuromyography revealed sensorimotor polyneuropathy with a demyelinating pattern and less axonal loss. Computed tomography Conclusion revealed lymphadenopathy in several chains, and many sclerotic The present study suggested that a majority of patients with bone lesions. A bone marrow biopsy contained 10% of plasma cells. posterior circulation stroke was suffered from post-stroke dizziness. Protein electrophoresis of the urine showed a lambda monoclonal Modified Rankin scale at discharge is a most reliable predictor for peak. The vascular endothelial growth factor (VEGF) was 300 times poor outcome. higher than the reference value. doi:10.1016/j.jns.2019.10.1286 Discussion and final comments The association of demyelinating polyneuropathy with a monoclonal peak in the electrophoresis results and findings of sclerotic bone lesions, the elevation of VEGF, lymph node organomegaly, and WCN19-1103 endocrinopathy, confirm the diagnosis of POEMS Syndrome. In this syndrome, neurological damage usually develops with a demyelinating pattern, greater axonal loss, and autonomic preserva- Poster shift 03 - Autonomic nervous system disorders/history of tion. However, we report an atypical case, of neurological impair- neurology/motor neuron disease/movement disorders/MS & ment with less axonal loss; and relevant dysautonomia. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1285 Somatosensory psychophysic losses in inhabitants of riverside communities of the Tapajós river basin, Amazonas, Brazil: The possible involvement of exposure to methylmercury WCN19-1102 M. Moreno Domingues, E. Dirce Torres Cury, A. Ayako Kamogari de Poster shift 03 - Autonomic nervous system disorders/history of Araújo, J. Maria Farah Costa Junior, M. da Conceição Nascimento Pinheiro neurology/motor neuron disease/movement disorders/MS & Federal University of Pará, Postgraduate Program in Tropical Diseases, demyelinating diseases/neuromuscular disorders/vestibular dis- Belem, Brazil orders and nutritional disorders or intoxications The objective of this study was to evaluate the somatosensory Post-stroke dizziness: Natural course and prognostic factor system of riparian fish exposed to the diet of fish in two communities of the Tapajós river basin, and to compare the same measurements a b a a J. Lee , I. Jung , J.Y. Choi , J.S. Kim performed in the inhabitants of the Tocantins river basin. Mercury a Seoul National University Bundang Hospital, Neurology, Seongnam, analysis was performed on hair samples through atomic absorption Republic of Korea; spectrophotometry. Three somatosensory psychophysical tests were b Korea University Ansan Hospital, Neurology, Ansan, Republic of Korea performed: tactile sensation threshold (Semmes-Weinstein 20 monofilaments), duration of vibration sensation (128 Hz tuning Background & objects fork) and two-point discrimination (two-point discriminator). The Dizziness is one of main symptoms of posterior circulation concentration of mercury was significantly higher in the Tapajós strokes. However, a prognosis of dizziness in those strokes has not riparian communities than in the Tocantins riparian communities (p been evaluated. In this study, we investigated post-stroke dizziness b 0.01). The tactile sensation threshold is greatest in all parts of the in terms of their temporal characteristics and prognostic factors. body, except in the left breast; the duration of the lower vibration sensation in all parts of the body, except in the upper sternum; and a Methods threshold of discrimination of two points greater in all the parts of We recruited 37 consecutive patients who had posterior circula- the body were observed in riverside of the Tapajós, when compared tion stroke within January 2014 to December 2015. Dizziness to the riverside of the Tocantins. The rivers exposed to mercury in handicap inventory (DHI), UCLA dizziness questionnaire (UCLA- the Tapajós region presented impaired somatosensory function in DQ), and short-form vertigo symptom scale (sf-VSS) were used to comparison with individuals from the control region. The long-term evaluate post-stroke dizziness. The questionnaires were taken at 1 exposure to mercury in riparian communities in the Tapajós basin is week, 1 month, 3 months, 6 months, and 12 months after onset of a possible cause, however, new studies should be encouraged in stroke. order to definitively confirm the somatosensory psychosocial losses observed in this population. Results DHI, UCLA-DQ, and sf-VSS scores were 39.1, 14.9, and 10.7 at 1 week, 21.4, 15.6, and 10.9 at 3 months, 17.6, 9.4, and 3.4 at 12 doi:10.1016/j.jns.2019.10.1287 months. The post-stroke dizziness decreased along time but ARTICLE IN PRESS

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WCN19-1104

Poster shift 03 - Autonomic nervous system disorders/history of neurology /motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Pure neural leprosy as differential diagnosis of demyelinating polyneuropathies: Case report

K. Lebeisa, M. Beiral Hämmerleb, C. Bittar Braunec, C.B. Nogueiraa,R. Barbosa Limad, R. Salvador Vivas Cardosoe aFederal University of the State of Rio de Janeiro, Internal Medicine, Rio de Janeiro, Brazil bFederal University of the State of Rio de Janeiro, Neurology, Rio de Janeiro, Brazil cFluminense Federal University, Neurology, Niteroi, Brazil upper extremities (hands and forearms) symetric anesthesia and dFederal University of the State of Rio de Janeiro, Dermatology, Rio de paresthesia were observed. Ulnar and tibial nerves were thickened. Janeiro, Brazil At the first consultation, no cutaneous lesions, except for old trauma eFederal University of the State of Rio de Janeiro, Medical Student, Rio de related scars were present. Janeiro, Brazil Fifteen months after early symptons, and three months after first consultation, appear barely discernible borders hypochromic macu- A 36-year-old male, brazilian, with no comorbidities, presented in lar lesions on trunk and lower limbs, whose senstivity to thermal, early 2017 with bilateral plantar hyposthesia, evolving to complete painful and tactile stimuli was impaired, besides a ucler at right hand and feet anesthesia. He denied vertigo, dysphagia or sphincter plantar region, are noted. disturbance. Ancillary exams Physical examination Complete blood count, basic metabolic panel and cerebrospinal Muscle strength, tone, gait and reflexes unaltared. Lower fluid analysis showed unaltered values. Electroneuromyography extremities (feet, lower legs and the distal third of the thighs) and revealed sensorimotor demyelinating polyneuropathy and serum ARTICLE IN PRESS

262 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx protein electrophoresis detected polyclonal WCN19-1114 hypergammaglobulinemia. IgM anti-myelin-associated glycoprotein antibody was absent. Lymphocytic inflammatory infiltrate and granulomatous inflammatory infiltrate were demonstrated on sural Poster shift 03 - Autonomic nervous system disorders/history of and skin lesiosn biopsies, respectively. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Discussion and final comments orders and nutritional disorders or intoxications Although the low prevalence of the pure neural leprosy, it should still be among the differential diagnosis of demyelinating Balance evaluation by dynamic posturography and minibestest: polyneuropathies. In these cases, the sural nerve and area with Comparison between patients with dizziness and imbalance due impaired sensitivity biopsies may help the diagnosis. to different neurological syndromes In addition to the skin lesions on the late phase of the disease course, the thicknened tibial and ulnar nerves, as well as E.R. Da Silva Aquino, V. Gonçalves Lopes, S. Nardy Nader, C. Borges the results of skin and sural nerve biopsies, support the diagnosis of Pereira leprosy. University of São Paulo Medical School, Clinical Neurology, São Paulo, Brazil doi:10.1016/j.jns.2019.10.1288

Introduction Objective tools were studied to quantify the impairment in patients with dizziness and imbalance. WCN19-1111 Objective ’ Poster shift 03 - Autonomic nervous system disorders/history of To evaluate the patient s balance using posturography and a clinical tool (MinBESTest). neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Methods orders and nutritional disorders or intoxications We included 113 evaluations of 52 patients with five syndromes: unilateral peripheral vestibulopathy, bilateral vestibulopathy, central Population of the river margin exposed to the mercury in the vertigo, cerebellar ataxia and polyneuropathy. Amazon: Evaluation of equilibrium, motor coordination and Posturography (ProBalance Master®) uses the sensory organiza- muscular force tion test in four conditions: 1 – eyes opened and 2 – eyes closed with fixed platform; 3 – eyes opened and 4 - eyes closed with sway- M. Moreno Dominguesa, E. Dirce Torres Khourya, A. Ayako Kamogari referenced platform. For each condition and at the end (composite), de Araújob, J. Maria Farah Costa Juniora, S. Fujiharaa, M. da Conceição the range of sway angle results in an equilibrium score. 100 Nascimento Pinheiroa represents no sway and zero represents maximum sway or fall. aFederal University of Pará, Postgraduate Program in Tropical Diseases, MiniBESTest is a 14-item clinical balance test (total score: 28) and Belem, Brazil includes anticipatory adjustments, reactive postural control, sensory bFederal University of Pará, Posgraduate Program in Tropical Diseases, orientation and gait stability. Belem, Brazil Results and discussion The objective of this study was to verify the association between Patients with peripheral vestibular impairment presented pre- mercury concentrations and neurological alterations related to served balance under conditions 1 and 2, while patients with central balance, motor coordination and muscle strength in adult riverside impairment have a poor balance in all conditions. Unlike patients residents in communities located on the banks of the Tapajós and with vestibular and cerebellar syndromes, polyneuropathy patients Tocantins rivers in the Brazilian Amazon. Concentrations of total presented worse balance at conditions 2 and 4 and normal scores at mercury were quantified in hair samples by atomic absorption 1 and 3 (graph 1). spectrophotometry and the neurological evaluation was performed Patients with unilateral vestibulopathy have higher MiniBESTest by conventional examination. The concentrations of mercury in the scores (average 20) (graph 2). The strongest correlation between communities of the Tapajós basin were higher than that of Tocantins (p b 0.0001). In the evaluation of the balance gait change was detected in eight cases in the Tapajós region (16.7%), while no case in Tocantins was observed (p b 0.05). The motor coordination examination showed alteration only in the diadochokinesis test, characterized as uncertain in four cases (Tapajós), whereas in Tocantins no alteration was detected. Regardless of the locality, no change in muscle strength was observed. In spite of the low frequency of changes in the three aspects studied, the effects at low concentrations of mercury should be monitored, since exposure in the Amazon region is chronic, as well as to collaborate in public health measures. The use of other quantitative methods may be used in future research to ratify these results. doi:10.1016/j.jns.2019.10.1289 ARTICLE IN PRESS

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doi:10.1016/j.jns.2019.10.1290

WCN19-1118

Clinico-pathological correlation in peripheral neuropathy

P. Eswaradassa, B. NamasivayamaT. Rb

aConsultant Neurologist SIMS Chellum, Neurology, Salem, India bConsultant Neuropathologist SIMS Chellum, Pathology, Salem, India composite and MiniBESTest score is observed in central disorders. Objectives There is no correlation in unilateral vestibulopathy (graph 3). To evaluate the diagnostic yield of nerve biopsy in patients with peripheral neuropathy of undetermined cause despite extensive Conclusion diagnostic workup. Different syndromes lead to different impairment patterns in posturography and MiniBESTest. This study also suggests that, in Methods some situations, the MiniBESTest, easily performed, can be reliable in Thirty-ﬁve consecutive patients with peripheral neuropathies from detecting functional impairment. a single center underwent relevant blood investigation, electrophysiological testing and nerve biopsy. The nerve specimen was ﬁxed in 2.5% glutaraldehyde and histopathological examination done.

Results The patient cohort consisted of 22 men and 13 women. Electrophysiologically neuropathy was classiﬁed as sensorimotor in ARTICLE IN PRESS

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29, purely sensory in 6, as axonal in 28, demyelinating in 4 and Results mixed axonal/demyelinating in 3 patients. Definitive diagnosis was The sample size was 67 consisting of 32 (52.23%) males and 35 possible in 25 patients (Hansen’s neuropathy in 14, vasculitic (47.77%) females with mean age (±SD) 67.83(±7.454). The mean neuropathy in 9 and Giant axonal neuropathy in 2). In six patients, MHY stage was 2.5 (±0.52) which was classified into mild (7.5%), the histopathology was supportive (hereditary neuropathy in 4 and moderate (61.2%) and severe (31.3%) off treatment. Among all, CIDP in one). The etiology of neuropathy remained unclear in 4 97.02% (65) patients had at least one NMS with mean number of patients. The duration of symptoms before nerve biopsy ranged from NMS of 8.64 ((±4.89) and range 2-38; the commonest NMSs 3 weeks to 5 years. All patients with asymmetric symptoms or signs reported were depression (56.71%), nocturia (56.71%), and insomnia (18/18) had definite diagnosis after nerve biopsy whereas the (55.22%) while bowel incontinence (2.98%) was the least reported. etiology could be defined in only 41% (7/17) of patients with There was a significant positive correlation between MHY and symmetric involvement. In 69% of patients, the clinical and number of NMS reported (p=0.006). histological diagnosis concurred, in 20% the histology changed the diagnosis and remained undiagnosed in 11%. Conclusion The types of NMS in PD varied widely in this cohort of patients Conclusions and nocturia, memory loss, loss of concentration, depression, The histopathological diagnosis was definitive in 72%, supportive insomnia and restless legs were identified to be predominant. in 17% and non contributory in 11% of patients. The contribution of nerve biopsy to the diagnosis of peripheral neuropathy was highest doi:10.1016/j.jns.2019.10.1292 in asymmetric neuropathy. The main indication for nerve biopsy remains the diagnosis of Hansen’s and vasculitic neuropathy, potentially treatable disorders. WCN19-1127 doi:10.1016/j.jns.2019.10.1291

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-1124 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Poster shift 03 - Autonomic nervous system disorders/history of Psychometric properties of the Croatian version of the multiple neurology/motor neuron disease/movement disorders/MS & sclerosis international quality of life questionnaire Demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Z. Uzarevica, A. Soldo Korugabc, I. Kampicb, Z. Popijacb, S. Butkovic Soldobc Non-motor symptoms of Parkinson’s disease and their correla- aFaculty of Education-University of Osijek, Department of Natural tion with motor manifestations among patients from northern Sciences, Osijek, Croatia Sri Lanka bClinical Hospital Centre Osijek, Clinic for Neurology, Osijek, Croatia cFaculty of Medicine-University of Osijek, Department of Neurology, A. Keshavaraja, S. Kunathilagamb Osijek, Croatia aTeaching Hospital of Jaffna, Neurology Department, Jaffna, Sri Lanka bNorthern Central Hospital, Neurology Unit, Jaffna, Sri Lanka Background Multiple sclerosis (MS) is a chronic progressive disease of the Introduction central nervous system that results in a wide range of clinical Non-motor symptoms (NMS) of Parkinson’s disease (PD) are less manifestations and has a great impact on patient health-related appreciated than motor symptoms but are the key determinants of quality of life (HRQoL). health, quality of life and social cost of PD. Objective Aim The aim of this study was to determine the psychometric To identify the prevalence of NMS and determine its association properties of the Croatian version of the Multiple Sclerosis Interna- with motor manifestations among PD patients of two movement tional Quality of Life (MusiQoL) questionnaire. disorder clinics in Jaffna, Sri Lanka Patients and methods Methodology In this research was analysed data of 82 MS patients (51 A hospital-based cross sectional study, in which data were women and 31 man, average 42.6±11.9 years) which underwent collected from consented PD patients regularly attending the neurological care at Clinical Hospital Centre Osijek, Croatia. Our movement disorder clinics of Teaching Hospital, Jaffna and Northern research problem was examined using the multidimensional and Central Hospital (pvt) ltd for a period of one year. A self- self-administrated MusiQoL questionnaire. The data was descrip- administered questionnaire consists of 26 items was used to collect tively analysed and internal consistency reliability was assessed by data on NMSs while the disease severity (Modified Hoehn and Yahr Chronbach’s alpha coefficient. Pearson’s correlation was performed Score-MHY) was measured using Unified Parkinson Disease Rating on the MusiQoL index score and dimension scores of the MusiQoL Scale (UPDRS). questionnaire. The level of significance was set to pb0.05. ARTICLE IN PRESS

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Results WCN19-1151 Cronbach’s alpha for the MusiQoL total score using the entire sample was 0.93, indicating a high internal consistency. Also, all MusiQoL dimensions demonstrated a high internal consistency Poster shift 03 - Autonomic nervous system disorders/history of (Chronbach’s alpha: 0.75-0.95). The MusiQoL index score signiﬁ- neurology/motor neuron disease/movement disorders/MS & cantly correlated with all MusiQoL dimension scores (Pearson’s demyelinating diseases/neuromuscular disorders/vestibular dis- correlation: 0.43-0.86). orders and nutritional disorders or intoxications

Conclusion 2/5000 interpretation of the Chinese guidelines for the diagnosis The Croatian version of the MusiQoL questionnaire can be a valid and treatment of tumefactive demyelinating lesions of central and reliable instrument for measuring QoL in Croatian-speaking MS nervous system patients and constitutes a useful instrument to measure HRQoL in the clinical setting. J. Liu, X. Qi The Six Medical Center of PLA General Hospital, Department of Keywords: Multiple sclerosis, MusiQoL, Quality of life, Questionnaire Neurology, Beijing, China doi:10.1016/j.jns.2019.10.1293 Tumefactive demyelinating lesions (TDLs) are relatively special type of immune mediated inflammatory demyelinating lesions in the central nervous system. TDLs are so named because it mimics brain WCN19-1128 tumors with large lesions with perilesion edema, mass effect and /or enhancement on neuroimmaging, and easily misdiagnosed as brain tumors. Poster shift 03 - Autonomic nervous system disorders/history of Although brain biopsy with pathological study is a gold standard neurology/motor neuron disease/movement disorders/MS & for the diagnosis of TDLs, it has limitations, including the risk of demyelinating diseases/neuromuscular disorders/vestibular dis- biopsy, dilemma of pathological results ( atypical pathology). orders and nutritional disorders or intoxications Currently, the diagnosis of TDLs is mainly clinical in addition to neuroimaging study. Because no diagnostic criterion or expert census Diagnostic utility of intraoperative squash cytology in central about this disorder is available. Many patients did not receive nervous system tumors with histopathological correlation appropriate treatment due to the misdiagnosis, and some even received tumorectomy or gamma knife therapy. During the recent years, studies of TDLs in China provided B. Kc, G. Sayami increasing evidence for the diagnosis of the disorder, and the Grande International Hospital, Pathology, Kathmandu, Nepal experience for the diagnosis of this disorder is becoming mature. Thus, the experts in the communities of Neuroimmunology and Intra-operative squash cytology is an important diagnostic tool neuropathology formulated the guidelines for the diagnosis and for rapid and accurate diagnosis of central nervous system (CNS) management of the TDLs as a reference for various levels of hospitals tumors. It has shown to play a vital role in determining further in our country. surgical management of patients undergoing surgery and can be Recent years, these guidelines were drafted by us, which were ascertained as an alternative method to frozen section in resource authorized by the above societies. In this article, the main viewpoints limited settings. including the diagnosis criteria, treatment program, etc., and these The study was done to evaluate the value of squash smear guidelines were completely discussed in the National Neu- cytology in rapid intra-operative diagnosis of CNS tumors and its roimmunological Summit Forum for three times.It is especially correlation with final histopathological diagnosis. A total of 57 valuable and can be used as a reference for managing those patients patients with various nervous system tumors were enrolled in the for whom the biopsy cannot be performed. study. Patients undergoing surgery at Department of Neurosurgery, TUTH, Kathmandu, Nepal and whose samples were sent intra- doi:10.1016/j.jns.2019.10.1295 operatively and post-operatively were included in the study. Intra- operative squash cytology diagnosis was performed and was correlated with histopathology diagnosis as gold standard. The frequency of nervous system tumor increased with increasing age with a peak incidence at fifth decade. Glial tumors were the most WCN19-1161 common (42%) followed by meningioma (21%) and schwannoma (19%). Glioblastoma was the most common glial tumor as well as the Poster shift 03 - Autonomic nervous system disorders/history of most common central nervous system tumor, both in squash neurology/motor neuron disease/movement disorders/MS & cytology and histopathology. Grade I tumors constituted the largest demyelinating diseases/neuromuscular disorders/vestibular dis- group accounting for 59.6% followed by grade IV (22.8%), II (10.5%) orders and nutritional disorders or intoxications and III (07%) tumors respectively. Out of 57 cases, 46 cases showed complete correlation between squash cytology and histopathology diagnosis. Thus, diagnostic accuracy of intra-operative squash Management of cervical dystonia with dry needling cytology irrespective of lesion and site was 80.7%. S. Chandra, H. Sugiharto, S. Marisdina, D. Susan doi:10.1016/j.jns.2019.10.1294 Mohamad Hosein Hospital Palembang, Neurology, Palembang, Indonesia ARTICLE IN PRESS

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Introduction and chronic phase(≥7 weeks after onset) were completed after Cervical dystonia is characterized by involuntary muscle contrac- enrollment. The characteristics of contrast-enhanced MRI in different tions of the neck and abnormal head positions that affect daily life clinical stages were compared and the evolutional characteristics activities. Botulinum toxin treatment continues to be the ﬁrst choice were summarized. treatment for most types of focal dystonia. Unfortunately, botulinum toxins are expensive and have limitations. Dry needling has been Results used widely to relieve muscle spasms and restore its length. In 35 TDLs, acute phase: the patterns of enhancement mainly patchy (74.3%, n = 26), nodule (34.3%, n = 12), closed ring (14.3%, n Case report = 5) and open ring (11.4%, n = 4), and for subacute phase, were A 34 years-old man developed severe axial neck dystonia mainly open ring (40.6%, n = 13), closed ring (31.3%, n = 10), gradually. His symptoms began approximately 6 months earlier with patchy (25.0%, n = 8) and irregular edge of enhancement (21.9%, n painless pulsing in his neck followed by involuntary neck movement = 7), and for chronic phase, mainly pale patchy (66.7%, n = 10). In and rotated of the neck with spasm. There was severe right head 35 Gliomas and 35 lymphomas, there were no signiﬁcant different rotation and laterocollis with mild retrocollis. Head CT-scan was enhancement form in different clinical stages. normal but cervical spine x ray showed retrolisthesis and narrowing intervertebral disc gap of C4-C5 and C6-C7. We used the Toronto Conclusions Western Spasmodic Torticollis Rating Scale (TWSTRS) to monitor the The manifestation of the TDLs lesions on contrast-enhanced MRI progress of dry needling in this patient. Before dry needling was may have dynamics of evolution with different clinical stages. In performed, the TWSTRS was 47 and after 5 times dry needling in one acute phase, the patterns of enhancement were mainly patchy and week interval, the score became 2 and persisted at 6 months nodular. In subacute phase, the enhancement gradually evolved into reassessment. Dry needling was done on the myotendinous junction edge of the lesions. In chronic phase, the degree of enhancement and/or muscle belly of ipsilateral sternocleidomastoideus muscle, faded evidently which showed mainly pale patchy and even spinalis capitis muscle and upper trapezius muscle. disappeared. The dynamic evolution may help to differentiate TDLs from Glioma and lymphoma. Conclusions Few treatment options are available and dry needling may be doi:10.1016/j.jns.2019.10.1297 useful in facilitating a rapid reduction in disability and severity. However, management using dry needling technique may require further assessment and re-needling after 3–6 months. WCN19-1174 Keywords: Cervical dystonia, dry needling

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1296 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Peripheral neuropathy among Bangladeshi Type 2 diabetic WCN19-1171 subjects

Poster shift 03 - Autonomic nervous system disorders/history of P.C. Banik, R. Mondal neurology/motor neuron disease/movement disorders/MS & Bangladesh University of Health Sciences BUHS, Noncommunicable demyelinating diseases/neuromuscular disorders/vestibular dis- Diseases, Dhaka, Bangladesh orders and nutritional disorders or intoxications Aims To determine the prevalence of DPN among a group of type 2 Dynamic features observation of contrast-enhanced MRI in the diabetic (T2DM) subjects, Bangladesh. different clinical stages of pathologically confirmed tumefactive demyelinating lesions and gliomas and lymphomas Materials and methods A cross-sectional study was conducted among 640 urban (M/F, J. Liu, X. Qi, D. Song 244/396; age in years, 52.23611.8; BMI, 25.9563.9 Kg/m2) and 560 The Six Medical Center of PLA General Hospital, Department of rural (M/F, 244/396; age in years, 50.89611.9; BMI, 24.1364.6). T2DM Neurology, Beijing, China subjects, selected purposively from various health care facilities in Dhaka and Northern Districts of Bangladesh. Diagnosis of T2DM and Objective DPN was done by WHO Study Group Criteria (following a 2-sample To explore the dynamic evolution rules of tumefactive demyelin- OGTT) and IWGDF recommendation respectively. DPN was assessed ating lesions (TDLs) in comparison to Glioma and lymphoma on by using 5.07 (10-g) Semmes-Weinstein monofilament, 128 Hz contrast-enhanced MRI with the different clinical stages, in order to tuning fork (for vibration sense), and two types of sensation test find the new differential imaging features of TDL and brain tumors. (fine, crude touch).

Methods Results 35 TDLs, 35 Gliomas and 35 lymphomas proven by pathological DPN was present among 21.1% (95% CI, 18.8–23.4) of T2DM studies were prospectively recruited from 2015 to 2017. Brain subjects. Regarding the sex speciﬁc, women (24.1%; CI, 21.0–27.2) contrast-enhanced MRI of different clinical stages including acute showed about 1.5 times higher prevalence compared to that of men phase(≤3 weeks after onset), subacute phase(4–6 weeks after onset) (16.0%; CI, 12.6–19.4) and the difference was highly signiﬁcant (p b ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 267

0.001). The prevalence of DPN was much higher among rural at their autopsied time. Respiratory insufﬁciency was the main cause of subjects (24.5%; CI, 20.9–28.1) compare to the urban subject death in 6 of these patients. Sepsis was another main cause of death in 4 (18.1%; CI, 15.1–21.1) and the difference was highly signiﬁcant (p patients. There were no pulmonary embolism in this series. b 0.001) between the two demographic locations. Conclusion Conclusions From this retrospective study, TIV could markedly improve A large proportion of T2DM subjects in Bangladesh, both men and prognosis of pathologically proved ALS patients. Sepsis and respira- women, irrespective of urban or rural origin, suffer from neuropathy. tory failures were the main cause of death in ALS-TIV. doi:10.1016/j.jns.2019.10.1298 doi:10.1016/j.jns.2019.10.1299

WCN19-1184 WCN19-1185

The cause of death in pathologically confirmed amyotrophic Toxic optic neuropathy- An uncommon complication of com- lateral sclerosis with invasive tracheostomy ventilation, a retro- monly used antibiotics spective institute-based study D. Radhakrishnana, N. Kumara, S. Ramanujb, R. Shreec, M. Rajatc,S. K. Komaia, C. Ishidaa, K. Takahashia, A. Tagamib, Y. Motozakia,A. Kumarib Kawashimac aAIIMS- RISHIKESH- INDIA, Neurology, Rishikesh, India aNational Hospital Organization Iou National Hospital, Hokuriku Brain bAIIMS- Rishikesh, India, Ophthalmology, Rishikesh, India and Neuromuscular Disease Center- Department of Neurology, Kana- cAIIMS- Rishikesh India, Neurology, Rishikesh, India zawa, Japan b National Hospital Organization Iou National Hospital, Hokuriku Brain Progressive painless bilateral vision loss with colour vision and Neuromuscular Disease Center- Department of Respiratory Medi- impairment and relatively symmetrical central or cecocentral cine, Kanazawa, Japan scotomas are characteristic of toxic optic neuropathy (TON). We c National Hospital Organization Kanazawa Medical Center, Pathology describe a case of 30 year old gentle man who developed TON in the and Laboratory Medicine, Kanazawa, Japan setting of prolonged use of chloramphenicol and ciprofloxacin for osteomyelitis. He presented with one week history of progressive Objective painless loss of vision in both eyes (right N left) associated with We performed retrospective institute-based prognosis study to impairment of colour vision. Clinical examination on day 8 of illness clarify the cause of death and the clinical course in pathologically revealed best corrected visual acuity of 6/18 in right and 6/12 in left confirmed amyotrophic lateral sclerosis (ALS) patients with trache- eye. His colour vision was significantly reduced in both eyes ostomy invasive ventilation (TIV). (rightNleft). Fundus examination revealed bilateral mild blurring of disc margins and mild tortuosity of retinal vessels at posterior pole. Patients and methods Visual field testing showed bilateral centro cecal scotoma. Laboratory To elucidate the clinical characteristics of pathologically con- work-up including complete blood count, inflammatory markers, firmed ALS, we retrospectively reviewed medical records of 96 folate and vitamin B12 levels were found normal. Visual evoked consecutive autopsied patients between 2008 and 2016. Pathological potentials showed prolonged P 100 latency bilaterally. Optical diagnosis was confirmed when the following two histopathological coherence tomography revealed retinal nerve fibre layer thickness features were observed. First of them was systematic both upper and on the higher side. CEMRI of the brain including orbit and CSF lower motor neuronal degeneration, and second was the existence of analysis were unremarkable. Suspecting, a diagnosis of TON second- TDP-43 inclusion body pathology and/or Bunina bodies. ary to chloramphenicol and ciprofloxacin both drugs were stopped immediately. High doses of B vitamins including B12, B6 and folic Results acid were prescribed resulting in improvement of symptoms. At 3 We identified 11 patients of pathologically diagnosed ALS with TIV months of follow up he had normal visual acuity and colour vision (ALS-TIV) in this period. Female to male ratio was 4 to 7. Average age of bilaterally. Our case represents a rare but serious complication of death was 71.1 y.o. and median survival time was 123 months. Age of commonly used antibiotics as chloramphenicol and ciprofloxacin. onset ranged from 43 to 82 years old, and age of death ranged from 56 Careful history taking is the key in reaching diagnosis. to 90 years old. According to initial symptom, 5 cases were upper limb onset, 3 cases were bulbar onset, 1 case was respiratory onset, and a doi:10.1016/j.jns.2019.10.1300 case was lower limb onset. All ALS-TIV patients had bronchopneumonia ARTICLE IN PRESS

268 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-1198 can ﬂare up owing to certain predisposing factors, causing severe symptoms, named myasthenia crisis.

Poster shift 03 - Autonomic nervous system disorders/history of Objective neurology/motor neuron disease/movement disorders/MS & The aim of this study is to ascertain any relation of variability of demyelinating diseases/neuromuscular disorders/vestibular dis- Myasthenia auto-antibodies to myasthenia clinical features espe- orders and nutritional disorders or intoxications cially crisis.

Tullio phenomenon and drop attacks – An unsuspected cause Methods It is a cross sectional study, carried out in Rashid Hospital, Dubai. H. Malhotraa, N. Kumara, T. Patila, K. Malhotra Preetb, R. Garg Kumara MG patients between 2004-2010 were recruited and followed for aKing George's Medical University, Neurology, Lucknow, India minimum 5 years period. Relationship between disease severity, bRam Manohar Lohia Institute of Medical Sciences, Pathology, Lucknow, diversity, immunology and other variables was made through India chisquare test mainly.

Result Tullio phenomenon or loudness intolerance is most commonly A total of 143 patients were included in the study and 102 associated with superior semicircular canal dehiscence. We describe completed the follow up. Females predominated in number and their a patient presenting solely with this phenomenon and found to have onset was relatively earlier than males (pb0.001). Total 58.8% of an unsuspected cause. patients (67% AchR-Ab+ patients, all MuSK-Ab+ and 33% of A 43-years-old gentleman presented to us complaining of seronegative) experienced crisis. Seropositive patients had 30% more intolerance to loud noise, occasionally leading to falls, for six tendency to develop crisis (pb0.05). Moreover, myasthenia crisis months. These complaints were insidious in onset and steadily directly proportional to disease severity at presentation. progressing. Interestingly, the intolerance in heavy trafﬁc used to subside on wearing a helmet. There was no history of hearing loss, Conclusion tinnitus, earache, or ear discharge. Clinical tests of hearing including Seropositive MG is severe form of MG and further in this group Rinne, Weber and absolute bone conduction test were within MuSK-Ab MG showed higher MGFA grading at presentation and normal limits. The neurologic as well as systemic examination was more frequent MG crisis. seronegative patients develop crisis at normal. relatively late age. Seropositivity, mechanical ventilation, old age and Pure tone audiometry (PTA) showed mild conductive hearing loss high starting MGFA grade indicated poor outcome after crisis. in left ear and brainstem auditory evoked response (BAER) was normal. Patient’s skull radiograph showed multiple large lytic lesions doi:10.1016/j.jns.2019.10.1302 giving the appearance of “geographical skull”. Chest X-ray showed a lytic lesion with pathological fracture involving posterior part of in left sided rib. Computed Tomography of head with bone window also showed multiple lytic lesions in skull (Figure 1,2). Brain parenchyma was normal. Incisional biopsy of one of the skull lesions was WCN19-1207 performed, which was consistent with a diagnosis of Langerhans cell histiocytosis (LCH) (Figure 3). The patient showed considerable Poster shift 03 - Autonomic nervous system disorders/history of improvement after being initiated on Etoposide and Vinblastine. neurology/motor neuron disease/movement disorders/MS & LCH is a rare cause of Tullio phenomenon. It appears that the loss demyelinating diseases/neuromuscular disorders/vestibular dis- of skull tissue due to lytic lesions leads to impairment of “modiﬁer” function of intact skull, resulting in “volume conduction” and noise orders and nutritional disorders or intoxications intolerance. The effects of 6-hydroxydopamine on iron metabolish in astro- doi:10.1016/j.jns.2019.10.1301 cytes are mediated by hypoxia-inducible factor- 2Α

M. Xu, J. Wang Qingdao University, Department of Physiology and Pathophysiology, WCN19-1203 Qingdao University, China

Poster shift 03 - Autonomic nervous system disorders/history of Disrupted iron homeostasis in the substantia nigra (SN) is involved in Parkinson's disease (PD). In astrocytes, 6-OHDA neurology/motor neuron disease/movement disorders/MS & increased both DMT1 and FPN1 expression, increased iron traffic. demyelinating diseases/neuromuscular disorders/vestibular dis- However, the mechanisms remain elusive. Hypoxia-inducible factors orders and nutritional disorders or intoxications (HIFs) are known to be important in regulating iron homeostasis. To explore that the mechanism of iron metabolism in astrocytes treated Role of myasthenia gravis auto-antibodies as predictor of with 6-OHDA, using primary cultured rat astrocytes, we observed myasthenic crisis and clinical parameters that protein levels of HIF-1α and HIF-2α in astrocytes were significantly increased by treatment with 6-OHDA, whereas expres- Y. Malik, J. Dar, M. Alkaylani, J. Inshasi, A. Almadani sion did not change in ventral mesencephalic (VM) neurons. Rashid Hospital- Dubai Health Authority, Neurolgoy, Dubai, United Arab Furthermore using inhibitors of HIF-1α and HIF-2α, we observed Emirates that HIF-2α inhibitor markedly reduced the up-regulation of DMT1 and FPN1 by 6-OHDA and decreased ferrous iron influx and efflux. Myasthenia Gravis (MG) is an autoimmune disease of the However, there was no effect following treatment of astrocytes with neuromuscular junction. Like any other autoimmune disorder, it HIF-1α inhibitor. Activation of HIF-2α by Phorbol 12-myristate 13- ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 269 acetate (PMA) and 6-OHDA was blocked by bisindolylmaleimide I truncal movements and voice after 2 weeks of oral salbutamol and hydrochloride (Bisl) and N-acetyl-l-cysteine (NAC) or Nω-Nitro-L- pyridostigmine. arginine methyl ester hydrochloride (L-NAME) preventing downstream activation of DMT1 and FPN1. NAC and L-NAME both block Conclusion the activation of PKCδ phosphorylation by 6-OHDA in primary This is the ﬁrst case report of VAMP1 pre-synaptic CMS from cultured astrocytes. Our data indicate that HIF-2α, but not HIF-1α, south-east Asia. regulates expression of DMT1 and FPN1 in astrocytes, and PKC pathway is involved in 6-OHDA activation of HIF-2α in astrocytes, doi:10.1016/j.jns.2019.10.1304 which may be associated with ROS and NO activation of PKCδ. doi:10.1016/j.jns.2019.10.1303 WCN19-1223

Poster shift 03 - Autonomic nervous system disorders/history of WCN19-1216 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Poster shift 03 - Autonomic nervous system disorders/history of orders and nutritional disorders or intoxications neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Adult onset opsoclonus-myoclonus-ataxia syndrome, clinical orders and nutritional disorders or intoxications features and diagnostic ﬁndings

First report of a VAMP1 associated pre-synaptic congenital S. Daoud, S. Sakka, N. Farhat, O. Hdiji, H. Haj Kacem, M. Damak, C. myasthenic syndrome from South-East Asia Mhiri Habib Bourguiba University Hospital- Sfax -Tunisia, Neurology Depart- S. Vengalila, K. Polavarapua, V. Preetish-Kumara, G. Arunachalb,N. ment, Sfax, Tunisia Prakash Mahajana, S. Nashia, K. Muddasua, C.P.C. Reddya, A. Nalinia a National Institute of Mental Health and Neurosciences, Department of Introduction Neurology, Bengaluru, India Opsoclonus-myoclonus-ataxia (OMA) is a rare neurological b National Institute of Mental Health and Neurosciences, Department of disorder, of paraneoplastic, infectious, post-infectious, post-vaccinal Genetics, Bengaluru, India or idiopathic origin.

Background Case report Biallelic mutations in VAMP1, a member of synaptobrevin family We report a 20-year-old woman who was presented with vertigo, have been reported previously to cause a rare form of pre-synaptic nausea, gait unsteadiness and movement disorders of limbs and eyes. congenital myasthenic syndrome (CMS) in two middle-eastern On examination, she had OMA syndrome. Brain MRI and total body families. scan were normal. Standard biological examinations and cerebrospinal fluid (CSF) analysis were normal. Serum and CSF viral serology Objective and method (EBV, VZV, HSV, CMV) were negative. Onco-neural antibodies were We present a case of pre-synaptic CMS from Southern India with negative. Neuron specific enolase was elevated (50,7ng/ml then 60 VAMP1 novel mutation identified by clinical exome sequencing ng/ml after 2 weeks). The dosage of urinary catecholamines was (CES).Infomed consent obtained. negative. Our patient received a steroid therapy and had a good outcome. One month later, our patient presented a disseminated Results intravascular coagulation leading rapidly to death. The autopsy A 5-year-old girl born to consanguineous parentage and delivered showed a severe and complete loss of Purkinje cells of the cerebellar by LSCS for absent labour pains, developed respiratory distress and cortex. It showed also a macroscopic lesion, and the feeding difficulty postnatally requiring oxygen supplementation. anatomopathological study showed adenomatous nodular hyperpla- Profound hypotonia, muscle weakness and ophthalmoparesis was sia of the adrenal glands. This aspect is highly suggestive of a observed during neonatal period. After 9 months of ryle’s tube neuroblastoma. feeding, percutaneous endoscopic gastrostomy (PEG) was performed. Fatigable ptosis, hypophonia and frequent lower respiratory Discussion/conclusion tract infections were noticed from 2 years of age. She did not attain OMA syndrome is a rare neurological disorder witch affects motor mile stones but attained normal mental milestones. Examina- mainly children. It is an autoimmune disease caused by dysfunction tion revealed myopathic facies, ophthalmoparesis, bifacial and bulbar in Purkinje cells. The etiology varies, although it is a paraneoplastic weakness, contractures at ankle and knee, kyphoscoliosis and manifestation (mainly of neuroblastoma) in 40% to 80% of cases. The areflexia. Her elder sister died in neonatal period following pathophysiology is thought to be immunological. Therapeutic benefit meconium aspiration and respiratory distress. Her maternal cousin, has been described with steroids, intravenous immunoglobulin, a 12-year-old boy is reported to have fatiguable ocular, limb and cyclophosphamide, azathioprine, and rituximab. Successful treat- bulbar weakness. Creatine kinase level was 366 IU/L. Child did not ment of the tumor, when present, does not usually improve co-operate for repetitive nerve stimulation test. CES revealed a neurological outcome. homozygous frameshift mutation c.66delT (p.Gly23AlafsTer6) in exon 2 of VAMP1 resulting in loss of v-SNARE domain important doi:10.1016/j.jns.2019.10.1305 for vesicle docking at NMJ. Child showed mild improvement in neck, ARTICLE IN PRESS

270 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-1226 C. Thursina Srie Setyaningrum, E. Fitri, F. Rachmad, D. Nugraha, V. Nathanael, R. Anindia Gadjah Mada University, Department of Neurology, Yogyakarta, Poster shift 03 - Autonomic nervous system disorders/history of Indonesia neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Abstract orders and nutritional disorders or intoxications Background Batik workers have occupational risk for developing neuromus- Extraterritorial motor function in carpal tunnel syndrome: A cular disorders. The process of Canting requires carefulness and study with angular rate measurement system based on precision which forces them to look down while sitting for a long gyrosensor period of time. Neuromuscular disorders in this case are Neck pain, Low back pain, Carpal tunnel syndrome and Vertigo can arise if the H.Y. Seoka, D.Y. Kwonb batik workers do their work with the wrong posture and less aKeimyung University School of Medicine, Neurology, Daegu, Republic of ergonomic position continuously. Korea bKorea University Ansan hospital, Neurology, Ansan-city, Republic of Purpose Korea Prevalence of neck pain, low back pain, carpal tunnel syndrome and vertigo in batik workers at Lendah Subdistrict, Kulon Progo Regency, Yogyakarta. Introduction Although extramedian sensory symptoms have been studied Method extensively, extraterritorial motor symptoms have not been of great Descriptive study using primary data with Cross-Sectional design interest to clinicians. The objective of this study was to investigate which the study population included 60 batik workers. The sampling the extraterritorial motor function in patients with carpal tunnel technique uses purposive sampling technique. The research instru- syndrome (CTS) using a gyrosensor. ments used were symptom based history taking, physical examination. Methods Angular velocity signal was measured during forearm pronation/ Result supination movement in 52 mild-to-moderate CTS and 45 controls. The prevalence of neuromuscular disorders in batik workers are Four forearm pronation/supination performance (FPS) parameters, Neck pain 51,1%, Carpal tunnel syndrome 43,96%, low back pain root-mean-squared (RMS) velocity, RMS angle, peak power, and 35,17%, and vertigo 11,5%. The subjects in the age groups of 36–50 total power) were derived from the signal. All participants com- years old, female sex, duration of work as pecanting more than 5 pleted the Boston-Questionnaire comprising 11 items of the years, normal body mass index. symptom severity scale (SSS) and eight of the functional severity scale (FSS). Conclusion Study can be concluded that the highest prevalence of neuro- Results muscular disorders in batik workers is Neck pain, at Lendah Sub- All FPS parameters were signiﬁcantly lower in CTS than in district, Kulon Progo Regency, Yogyakarta. controls (RMS velocity and total power, P b 0.001; RMS angle, P = 0.001; peak power, P = 0.022). In addition, all FPS parameters had a Keywords: Neuromuscular disorders, Pecanting, Batik, Kulon Progo signiﬁcant negative correlation with FSS scores (RMS velocity, r = – Regency 0.369, P = 0.023; RMS angle, r = –0.420, P = 0.009; peak power, r = –0.339, P = 0.038; total power, r =– 0.419, P = 0.009).

Discussion doi:10.1016/j.jns.2019.10.1307 This study identiﬁed extraterritorial proximal motor dysfunction in mild to moderate CTS patients. It suggests that extraterritorial motor dysfunction can occur in the early stages of CTS as well as extramedian sensory symptoms. Further studies are needed to identify its precise mechanism. WCN19-1238 doi:10.1016/j.jns.2019.10.1306 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications WCN19-1231 Parkinsonism and its clinical sub types: A prospective observa- Poster shift 03 - Autonomic nervous system disorders/history of tional study in a tertiary care hospital neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- S.K. N S, S.S. Mamilla Narayana Medical College, Neurology, Nellore, India orders and nutritional disorders or intoxications

Introduction Neuromuscular disorders in Batik workers at Yogyakarta Parkinsonism deﬁned by combination of six speciﬁc motor Indonesia features: tremor at rest, bradykinesia, rigidity, loss of postural ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 271 reflexes, flexed posture,freezing phenomenon. Parkinsonism sub Observations classified into Idiopathic Parkinson’s disease, (IPD) secondary 56 consecutive patients with HLGD, forming 3.38% of new Parkinsonism, Parkinsonism Plus syndromes, heredodegenerative referrals, were included. 23 patients (41%) fulfilled criteria for Parkinsonism. Progressive Supranuclear Palsy (PSP) (Höglinger 2017), 11 (20%) for vascular parkinsonism (VaP) (Winikates 1999), 9 (16%) for Normal Methodology pressure hydrocephalus (NPH) (Relkin 2013), and 2 (4%) had a Study prevalence and clinical profile of Parkinsonism clinical multisensory gait disorder. 11 cases (20%) remained unclassified. subtypes. Hospital based, prospective, Observational study done. Patients with PSP (Table 1) had REM Sleep behavior disorder (RBD), Patient with Parkinsonism, Akinetic rigid state were enrolled. extraocular abnormalities (EOM), axial rigidity, masked facies, gait History, clinicalfeatures, staging were recorded in proforma. freezing, inability to arise from chair unsupported, severe postural instability on pull test, and midbrain atrophy on MRI brain. Patients with Results VaP had ≥2 vascular risk factors, pyramidal signs, broad-based 120 studied (100 male/20 females). Prevalence of Parkinsonism is asymmetric gait, and leukoaraiosis on MRI brain. NPH was a diagnosis 42 per 10,000 population. 75 Patients were IPD. Prevalence of IPD is of exclusion (Table 1) with ventriculomegaly on MRI, and improvement 26/10,000 population. Progressive supra nuclearpalsy is 7/10000 at lumbar tap test (LTT) which eventually predicted VP Shunt population. Vascular Parkinsonism is 5/10,000 population. Multi responsiveness. system atrophy is 1/10,000 population. 4 cases of MSA of which MSA P (2) MSA C (2). Cortico basal degeneration is 1/10,000 population. Conclusion Drug induced Parkinsonism is 0.6/10,000 population. 2 cases of drug PSP is the commonest HLGD. VaP and NPH are other common induced PD. 1 case of Dementia with lewy body was diagnosed. HLGD. Reaching a diagnosis requires assessment of all features in The mean age presentation of IPD is 63.5 years. CBD 59.5 years, unison. Clinical phenotype evolves with time on follow up. CBD PSP 65 yrs, DLB 70 yrs, Drug induced PD is 49 yr. In MSA –C 60.5 yrs, MSA-P 63 yrs. PSP RS and PSP P is 65.4 and 65.2 years. doi:10.1016/j.jns.2019.10.1309 Mean duration of all subtypes of Parkinsonism is 2.37 yrs. CPD PSP is 3 yrs and IPD is 2.5 yrs. Drug induced Parkinsonism is 0.33 yrs. 32% patients of IPD in stage 1.5, 24 % stage -1, 21.33 % is stage – 2, stage 2.5 is 14.67% and stage 3 is 8%. WCN19-1269

Conclusion Knowing prevalence and clinicalfeatures disease burden and Poster shift 03 - Autonomic nervous system disorders/history of prognosis is guided. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- doi:10.1016/j.jns.2019.10.1308 orders and nutritional disorders or intoxications

Congenital myathenic syndrome associated with COLQ mutation: an interesting report WCN19-1264 D. Joshia, S. Patilb, P. Dashb, V. Mishrab, R. Chaurasiab, A. Pathakb a Poster shift 03 - Autonomic nervous system disorders/history of Neurology, Varanasi, India bInstitute of Medical Sciences- Banaras Hindu University, Neurology, neurology/motor neuron disease/movement disorders/MS & Varanasi, India demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Congenital myasthenic syndromes (CMS) are a rare inherited disorder of neuromuscular transmission associated with abnormal Clinical spectrum of higher level gait disorders (HLGD) in a weakness and fatiguability on exertion. Usually present from birth/ tertiary care movement disorder clinic - A prospective study childhood and are characterized by exercise induced weakness and easy fatigability (regular activities). We present rare and interesting A. Alshi, J.D. Desai, P.M. Wadia case of CMS. Jaslok Hospital and Research Centre, Neurology, Mumbai, India A 26 year old male, born out of non-consanguinous marriage with normal birth history, presented to us with motor developmental delay, Introduction was a floppy infant with poor suck and cry with bilateral ptosis, Gait disorders are classified into lower, middle, and higher gradually progressive bilateral lower limb weakness since 10 years of sensorimotor levels (Nutt 1993). HLGD are common and disabling age, bilateral upper limb weakness since 12 years of age with easy in the elderly. fatiguability on exertion, ptosis, neck weakness without any fasciculations, bladder bowel involvement or sensory complaints with a negative Objective history family with a worsening of symptoms on pyridostigmine trial. To study the burden, demographic profile and etiologic profile of Routine nerve conduction studies were normal. There was HLGD in a tertiary care center in India. decremental response in RNST. His Ach receptor antibody and Musk antibody status were negative. In view of above findings he was Methods subjected to genetic analysis where targeted gene sequencing was Patients presenting with Parkinsonism and predominant lower done, which revealed a homozygous mutation in the intron 12 of limb involvement in the form of gait difficulty (freezing, shuffling or COLQ gene which was pathogenic. slowness) and/or postural instability (imbalance or falls), not TheCOLQgeneislocatedattheshortarmofchromosome3.ItsAR completely accounted for by visual, vestibular or proprioceptive mutation causes synaptic form of CMS. The age of presentation is deficits, were studied. Follow up was done for 3 months. variable from early childhood to 4–5th decade with fatiguable weakness, ARTICLE IN PRESS

272 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx ptosis and respiratory crises. They have variable response to treatment WCN19-1272 and hence genetic characterization is of utmost importance!

Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1310 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-1271 An interesting case of Miller Fisher syndrome with ocular myasthenia Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & P. Ranganathan demyelinating diseases/neuromuscular disorders/vestibular dis- Pazhani Neuro Centre, Neurology, Chennai, India orders and nutritional disorders or intoxications Thirty five year old lady went for a holiday. She had a brief febrile illness. Subsequently she noticed numbness in the fingers and toes. Can we predict post-partum relapses in pregnant women with She also noticed unsteadiness while walking and restricted eyeball multiple sclerosis? movements. She was admitted and investigated to r/o Miller Fisher syndrome. Clinically she had ataxia, areflexia and opthalmoparesis. F. Kharrat, O. Hdiji, H. Hajkacem, S. Sakka, N. Farhat, M. Damak, M. As the diagnosis was made in the earlier phase, the nerve conduction Chokri study was normal. Antiganglioside antibodies were negative. MRI Habib Bourguiba-University Hospital, Department of Neurology, Sfax, brain showed faint demyelinating shadow in the brainstem. She was Tunisia treated with IV immunoglobulin for 5 days. Her ataxia and numbness subsided. Total opthalmoparesis not Background recovered. Her serum acetylcholine receptor antibody was positive Multiple sclerosis (MS) affects women of childbearing age. Thus, 0.8. The antiganglioside antibodies and serum vasculitic profile pregnancy is an important issue to discuss since pregnancy is known repeated were negative. to be associated with a high risk of post-partum relapses. She was started on oral steroid and pyridostigmine. Her opthalmoparesis recovered in 4 weeks time. It is unusual to see both Aims of the study Miller Fisher syndrome and ocular myasthenia in the same patient. it To identify predictors of post-partum relapses in MS patients. is possibly a post viral auto immune disorder and the patient developed both disorder. It is important to look for other auto Methods immune disorders in a situation like this, as the patient needed We conducted a retrospective study including relapsing- MS specific treatment for different disorders. patients being pregnant after disease onset and looked for factors associated with the occurrence of post-partum relapses. doi:10.1016/j.jns.2019.10.1312

Results We enrolled 42 patients with a total of 72 pegnancies. Post- partum relapses occured in 55% of the pregnancies, mainly in the ﬁrst three months after delivery. Post-partum relapses were WCN19-1273 associated with shorter disease duration, the occurrence of relapses 24 months before pregnancy and the rank of pregnancy (the ﬁrst Poster shift 03 - Autonomic nervous system disorders/history of one), but not with the other factors: relapses occuring during neurology/motor neuron disease/movement disorders/MS & pregnancy, aborted pregnancies, the delivery type, the use of demyelinating diseases/neuromuscular disorders/vestibular dis- epidural analgesia, resumption of disease modifying drugs within a orders and nutritional disorders or intoxications month after delivery and breastfeeding.

Video head impulse test findings in patients with Charcot-Marie- Discussion Several prior studies showed similar outcomes. Others suggested tooth disease 1A further predictive factors such as high EDSS prior to pregnancy and ab c d e d d annualized relapse rate during pregnancy. We add to those findings Z. Calic , V. Rakocevic , S. Peric , M. Vujnic , B. Bjelica , I. Bozovic , fg the rank of pregnancy. This may be explained by the fact that M. Welgampola a immunotolerance mechanisms acquired in mid-first-pregnancy are Department of Neurophysiology, Liverpool Hospital, Liverpool, Austra- memorized and set up in early stages of subsequent pregnancies. lia bSouth Western Sydney Clinical School, University of New South Wales, Conclusion Sydney, Australia c Knowing predictors of post-partum relapses, makes clinicians Neurology Clinic Clinical Centre of Serbia, School of Medicine University more aware of this risk. All pregnancies should be planned. of Belgrade, Belgrade, Serbia d Conception should be avoided in patients with recent onset and Neurology Clinic Clinical Centre of Serbia School of Medicine, University active course of the disease in the two previous years. of Belgrade, Belgrade, Serbia eFaculty of Medicine, University of Banja Luka, Banja Luka, Bosnia and Herzegovina doi:10.1016/j.jns.2019.10.1311 fInstitute of Clinical Neurosciences, Royal Prince Alfred Hospital, Camperdown, Australia ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 273 gCentral Clinical School Sydney Medical School, University of Sydney, Objective Camperdown, Australia Our objective is to demonstrate the benefits of palliative care services (PCS) in an interdisciplinary ALS team in documentation of Aim code status, enrollment in hospice services and location of death. Charcot-Marie-Tooth disease 1A (CMT1A) is the most common inherited nerve disorder. This study examines vestibulo-ocular reflex Methods (VOR) characteristics as measured by the video head-impulse test Data from the Veterans Affair Connecticut Healthcare System (v-HIT). (VACT) ALS database includes clinical data on 134 veterans with ALS. The current analysis includes 80 deceased patients. Database was Method reviewed for documentation of DNR/DNI status, enrollment in 22 patients (age 51.9 ± 13.8, 17F, 5M) with CMT1A with a mean hospice programs and location of death. disease duration of 25.2 ± 17.5years, mean MRC Score of 47.4 ± 6.8, were recruited from an Outpatient Neurology Clinic. Three-dimen- Results sional v-HIT was performed. VOR gain, catch-up saccade prevalence, The majority of patients (84%) had palliative care assessments. 1st saccade amplitude, onset latency and duration were examined Patients who chose DNR/DNI status were more likely to do so when and compared against age-matched controls. palliative care services were involved (79% with PCS, 49% without Results PCS). Patients with PCS were more likely to die at home versus those 6/22 (27.3%) patients reported vertigo or dizziness, 18/22 (82%) without PCS (54% vs 39%). Patients without PCS were more likely to severe imbalance resulting in recurrent falls in 14/22 (64%) patients. die in a hospital setting as compared to those with PCS. Mean Overall Neuropathy Limitations Scale Score (ONLSS) lower limbs was 2.1. Horizontal, anterior and posterior canal (HC, AC, PC) Conclusion v-HIT gains were 1.08 ± 0.1, 0.96 ± 0.2, 0.69 ± 0.1 for CMT1A 0.99 Discussions of code status and end of life care increase the ± 0.1, 0.97 ± 0.1, and 0.78 ± 0.1 for controls. Refixation saccade likelihood of home death and avoid unwanted care while maintain- prevalence for HC, AC, PC were 52%, 22%, 46% in CMT1A and 59%, 11% ing patient autonomy. PCS embedded within an interdisciplinary ALS and 53% in controls. 1st saccade onset latency was prolonged (N50 clinic will assist in assuring a patient’s end of life preferences are ms) for HC and PC in CMT1A group compared to controls (p b 0.05). followed. Further research to determine if PCS improves quality of There was no significant difference in 1st saccade amplitude and life is needed. duration. Higher ONLSS was associated with larger 1st saccade b amplitude in HC and PC (p 0.05). doi:10.1016/j.jns.2019.10.1314 Conclusion Refixation saccades are common and tend to occur later in patients with CMT1A. These findings may relate to demyelinating processes involving the VOR pathways. Further studies of otolith ocular pathways and correlations with oculomotor function may WCN19-1288 help better characterisation of the origins of these changes. Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1313 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-1274 Hunting the one: a genetically-proven sporadic case of Huntingtons disease in the Philippines, a case report Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & L.K. Batino demyelinating diseases/neuromuscular disorders/vestibular dis- Baguio General Hospital and Medical Center, Neurology, Baguio, Philippines orders and nutritional disorders or intoxications

A healthy 39-year old, male, with no family history of any End of life decision making and palliative care services in a movement disorder presented with a 10 year history of progressive multidisciplinary amyotrophic lateral sclerosis clinic purposeless, involuntary, brief, arrhythmic, dance-like movement of the left forearm evolving to gait disturbance, prominent writhing of H. Patwaa, C.J. Coffeeb, T. Shamasb all extremities and bulbar symptoms. He developed behavioral aYale University/VA Connecticut Healthcare System, Neurology, New change, emotionally lability, irritability with cognitive decline. He Haven- CT, USA presented at the emergency department for inability to swallow and bVA Connecticut Healthcare System, Nursing, West Haven- CT, USA slurred speech. He had mild cognitive impairment, vertical gaze palsy, slurred speech with involuntary random facial grimaces & Background intermittent tongue protrusions. Random purposeless involuntary, The care of patients with Amyotrophic Lateral Sclerosis (ALS) brief, writhing (proximal to distal) movements of extremities ’ should include goals of care discussions to assure a person s wishes including the trunk and bilateral hung up knee jerk reﬂexes are are honored throughout the trajectory of the illness. Effective noted. communication in end-of-life is critical in preserving patient MRI done, revealing atrophy of putamen and caudate, focal autonomy. Early dialogue assists in making informed decisions on dilatation of the frontal horns, cerebral atrophy and signal changes at how an individual wants to deal with a terminal illness. ARTICLE IN PRESS

274 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx attributed to neuronal loss and gliosis. CSF analysis, ASO titer, ESR, 2- WCN19-1309 D Echo revealed normal results. Genetic testing (PCR) revealed full penetrance with a 47 CAG repeat sequence. He is currently maintained on Clonazepam, Lamotrigine, Valproic acid and Risper- Poster shift 03 - Autonomic nervous system disorders/history of idone which decreased the frequency of the involuntary movements. neurology/motor neuron disease/movement disorders/MS & He is now feeding per orem with resolution of bulbar symptoms and demyelinating diseases/neuromuscular disorders/vestibular dis- requires less assistance in ADL’s. He has 2 children, both are orders and nutritional disorders or intoxications currently asymptomatic. This is the second genetically proven case of HD in the Philippines Onasemnogene abeparvovec gene-replacement therapy (GRT) in and the first sporadic case confirmed to date. High clinical suspicion presymptomatic spinal muscular atrophy (SMA): SPR1NT study based on history and physical exam warrants genetic testing even if update the disease is rare because of its penetrance and progressive debilitating natural history. K.A. Straussa, K.J. Swobodab, M.A. Farrarc, H.J. McMilland, J. Parsonse,J. M. Kruegerf, S.T. Iannacconeg, C.A. Chiribogah, J.M. Kwoni, K. Saitoj,M. doi:10.1016/j.jns.2019.10.1315 Scotok, F. Baldinettil, M. Schultzm, E. Kernbauern, M. Farrowo, F.G. Ogrincp, S. Kavanaghq, D.E. Feltnero, B.E. McGillo, S.A. Spectoro,J. L’Italienr, D.M. Sprouleo, F. Muntonik aPediatrics, Clinic for Special Children, Strasburg, USA WCN19-1290 bDepartment of Neurology, Massachusetts General Hospital, Boston, USA cDepartment of Neurology, Sydney Children’s Hospital Network, Randwick, Australia Poster shift 03 - Autonomic nervous system disorders/history of dDepartment of Pediatrics, Children’s Hospital of Eastern Ontario, neurology/motor neuron disease/movement disorders/MS & Ottawa, Canada demyelinating diseases/neuromuscular disorders/vestibular dis- eDepartment of Pediatrics, University of Colorado School of Medicine, orders and nutritional disorders or intoxications Aurora, USA fDepartment of Neurology, Helen DeVos Children’s Hospital, Grand A case of proximal muscle weakness and areflexia with post Rapids, USA exercise facilitation of deep tendon reflexes gDepartment of Pediatrics, University of Texas Southwestern Medical Center, Dallas, USA F. Chacko, R. Pozhekadavil, M.A. Poovathingal hDivision of Pediatric Neurology, Columbia University Medical Center, Jubilee Mission Medical College and Research Institute, Neurology, New York, USA Thrissur, India iDepartment of Neurology, University of Wisconsin School of Medicine and Public Health, Madison, USA j Lambert-Eaton syndrome (LES) is a rare presynaptic neuromus- Institute of Medical Genetics, Tokyo Women's Medical University, cular junction disorder which is characterised by proximal limb Tokyo, Japan k muscle weakness, decreased deep tendon reflexes and various The Dubowitz Neuromuscular Centre, University College London- Great autonomic symptoms. Ormond Street Institute of Child Health & Great Ormond Street Hospital, A 60 year old gentleman presented with complaints of progres- London, United Kingdom l sive proximal muscle weakness of both upper limbs and lower limbs EMEA Medical Affairs, Avexis- Inc., Bannockburn, USA m since 6 months. He had significant weight loss and anorexia. He gave Translational Medicine, Avexis- Inc., Bannockburn, USA n history of dryness of mouth, difficulty in swallowing and erectile Clinical Operations, Avexis- Inc., Bannockburn, USA o dysfunction since past six months. On examination there was Clinical Development, Avexis- Inc., Bannockburn, USA p symmetrical, proximal more than distal, lower limb more than upper Biostatistics, Avexis- Inc., Bannockburn, USA q limb weakness. No wasting or fasciculation was noted. Gait was Clinical Development- Biostatistics, Avexis- Inc., Bannockburn, USA r waddling. All deep tendon reflexes were absent. However, the Regulatory and Quality, Avexis- Inc., Bannockburn, USA reflexes showed a post exercise facilitation (after 10 s of tonic contraction) especially the knee jerk (video available). High SMA is a rapidly progressing neurologic disease caused by frequency RNS performed showed an incremental response. Chest biallelic survival motor neuron 1 gene (SMN1) deletion/mutation. X ray showed a non-homogenous opacity in right middle zone. CECT Genomic copies of a similar gene (SMN2) modify disease severity. In thorax showed a heterogeneously enhancing lesion in right hilum a phase 1/2a study, the one-time SMN GRT onasemnogene involving mediastinum. A guided biopsy from lesion showed features abeparvovec (AVXS-101) improved outcomes in symptomatic SMA of small cell neuroendocrine carcinoma. Anti-VGCC antibody was type 1 patients (2xSMN2) dosed ≤6 months. This study evaluated tested and it was positive confirming diagnosis of LES. Patient was AVXS-101 in presymptomatic patients. transferred to oncology department and initiated on chemotherapy. I SPR1NT is a multicenter, open-label, phase 3 study. Asymptom- have obtained patient and Institutional Review Board approval. atic SMA patients (≥27; 2–3 x SMN2) aged ≤6 weeks will receive In conclusion, post exercise facilitation of deep tendon reflexes intravenous AVXS-101 (1.1 x 10e14 vg/kg). Safety and efficacy are can be considered as the telltale sign of LES. LES is considered a assessed through study end (2 x SMN2: 18 months; 3 x SMN2: 24 harbinger of malignancy, especially small cell lung cancer (SCLC). A months). Primary outcomes: independent sitting ≥30 s (2 x SMN2), diagnosis of LES may lead to the timely detection of SCLC, assisted standing (3xSMN2); exploratory outcomes: motor function significantly improving patient prognosis and survival. improvement (CHOP INTEND, Bayley-III). As of 8 March 2019, 18 infants have received AVXS-101 (aged 8– doi:10.1016/j.jns.2019.10.1316 40 days [mean: 23]; 11 female; 2 x SMN2, n = 8; 3 x SMN2, n = 9; 4 x SMN2, n = 1). Mean baseline CHOP INTEND score in patients with ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 275

SMA (2 x SMN2) was 44.0 points (maximum 64), which increased by Keywords: Pesticide exposure, Cholinesterase level, Tremor a mean of 8.9 (n = 8) and 14.4 (n = 7) points at 1 and 3 months post-dosing, respectively; 6 patients achieved a score of ≥60 points. 4/8 patients (2 x SMN2, median [range] age: 6.1 [1.7–9.1] months) achieved sitting without support for ≥30 s; 1 patient achieved doi:10.1016/j.jns.2019.10.1318 standing with assistance. Early increases in mean Bayley-III Gross Motor score were observed in SMA patients (2 x SMN2 and 3 x SMN2). Follow-up will be presented. Preliminary data from SPR1NT show rapid motor function WCN19-1320 improvements in presymptomatic SMA patients. Prompt diagnosis and early treatment intervention may minimize motor neuron loss Poster shift 03 - Autonomic nervous system disorders/history of and maximize patient beneﬁt. neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1317 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Comparison of a novel instrument for diagnosing HIV-associated WCN19-1318 neuropathic pain with the single-question neuropathy screen

A. Iyagbaa, M. Owolabib, A. Ogunniyib Poster shift 03 - Autonomic nervous system disorders/history of aUniversity of Port Harcourt Teaching Hospital, Medicine, Port Harcourt, neurology/motor neuron disease/movement disorders/MS & Nigeria demyelinating diseases/neuromuscular disorders/vestibular dis- bUniversity College Hospital, Medicine, Ibadan, Nigeria orders and nutritional disorders or intoxications Background Blood cholinesterase level is associated with tremor of farmer Distal symmetrical peripheral neuropathy (DSPN) and neuropathic with pesticide exposure in Indonesia pain (NP) are common in human immunodeficiency virus/acquired immune deficiency syndrome (HIV-AIDS) infection. The HIV-associ- A. Dyah Kusumawati, S. Sutarni, S. Subagya, I. Setyopranoto, R.G. ated neuropathic pain questionnaire (HANP-Q) is a novel instrument Malueka for diagnosing HIV-associated peripheral neuropathy and neuropathic Gadjah Mada University, Neurology, Yogyakarta, Indonesia pain. It was compared with the single question neuropathy screen (SQNS). The Douleur Neuropathique en Four Questions (DN4) is the Background gold standard questionnaire for diagnosing NP. Organophosphates and carbamates are among the most commonly used insecticides worldwide and are known to suppress the Aim activity of acetylcholinesterase (AChE) that will result in nervous To compare simple clinical screening tools for diagnosing HIV- system toxicity. Previous studies described positive associations DSPN between concurrent AChEactivity, a stable marker of cholinesterase inhibitor pesticide exposure, with tremor in farmer. This study was Method aimed to identify association between cholinesterase level with Ethical approval was obtained. It was a descriptive cross-sectional tremor in farmer exposed to pesticide exposure in Magelang study using convenience sampling method. A total of 100 HIV-positive Regency, Indonesia. adults were recruited. All participants were administered the HANP-Q, SQNS and the DN4 questionnaires. The SQNS diagnosed HIV-DSPN Methods from a simple 'yes' or 'no' response to the presence of numbness. The This was a cross-sectional study. The subjects were the farmer DN4 comprises 10 items. Of these, three items were findings from with history of pesticide exposure in Ngablak, Magelang Regency, clinical examination. A total score of 4/10 diagnosed HIV-DSPN and Central Java, Indonesia. Cholinesterase level was analyzed from NP. The HANP-Q consists of 3 sub-scales each comprising 4 items. A blood. Tremor was assessed using Tremor Rating Scale (TRS). total score ≥2 was diagnostic of HIV-DSPN and NP. Data were analyzed using Statistical Package for Social Sciences-21 software. Results In total, 120 subjects aged average 45.8 ± 13.43 years old were Results included. The average of blood cholinesterase level was 8.73 ± The frequency of diagnosis of HIV-DSPN were 8(8.0%), 17(17.0%) 1.80 kIU/L, with 70 subjects had low level of blood cholinesterase and 4(4.0%) by the HANP-Q, SQNS and DN4 respectively. The and 86 subjects had tremor. Bivariate analysis showed that blood sensitivity and specificity of the HANP-Q relative to the DN4 were cholinesterase level was associated with tremor (p = 0.005; 80.0% and 95.8% respectively. The sensitivity and specificity of the 95% CI = 1.39-7.23). Further analysis showed that cholinesterase SQNS relative to the DN4 were 80.0% and 86.3% respectively. level was associated with tremor of arm (p = 0,02; 95%CI = 1.58- 6.88) Conclusion Overall, the HANP-Q had a superior diagnostic accuracy compared Conclusions to the SQNS. It will be a useful diagnostic tool in resource-limited Blood cholinesterase level, a stable marker of cholinesterase settings inhibitor pesticide exposure, was positively associated with tremor in farmer as assessed by TRS, especially tremor of arm. doi:10.1016/j.jns.2019.10.1319 ARTICLE IN PRESS

276 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-1328 bNational Institute of Mental Health and Neuro Sciences- Bengaluru, Department of Pyschiatric Social Work, Bengaluru, India cNational Institute of Mental Health and Neuro Sciences, Department of Poster shift 03 - Autonomic nervous system disorders/history of Neurology, Bengaluru, India neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Background orders and nutritional disorders or intoxications Multiple Sclerosis is increasingly being identiﬁed as a debilitating illness with signiﬁcant comorbid mental health concerns. The current Effects of transcranial direct current stimulation on action-verb study attempts to understand the psychosocial correlates and quality processing in patients with Parkinson's disease of life (QoL) of Women with multiple sclerosis (WLMS).

J. Cardona, D. Suarez Method Universidad del Valle, Instituto de Psicología, Cali, Colombia A sample of 34 WLMS attending multiple sclerosis clinic in National Institute of Mental Health and Neuro Sciences (NIMHANS), – Objective Bengaluru between August 2018 February 2019 were cross- The aim of this study is to investigate the effects of transcranial sectionally assessed using Hospital Anxiety and Depression Scale direct current stimulation (tDCS) combined with cognitive training (Zigmond & Snaith, 1983), Brief Cope (Carver et al, 1989), Multiple (CT) on action-verb processing in patients with Parkinson’s disease Sclerosis Quality of Life 54 (Vickery et al, 1997), Family Burden (PD). Inventory (Pai & Kapur, 1981) and Expanded Disability Status Scale (EDSS) (Kurtzke, 1983) and secondary data from case files. Methods A pilot, randomized double-blind controlled, clinical trial was Results conducted with 30 patients with PD assigned to two groups: (I) The mean age of the WLMS was 30.5±8.5 years with mean active anodal tDCS over M1 and IFG plus CT (experimental group), or duration of illness of 4.3±4.0 years and 1.9 ± 0.8 mean score on (II) sham tDCS plus CT (control group). All PD participants who met EDSS. Anxiety was found to be the common comorbidity among ρ b UK Parkinson Disease Society Brain Bank criteria were evaluated WLMS ( = .349, p 0.005). The most affected MS-QoL domains using part III of the Unified Parkinson’s Disease Rating Scale (UPDRS) were health distress with median of 32.5 (20.00,66.25) and health and Hoehn and Yahr’s stages I or II. The patients were undergoing perception at 42.5 (28.70,60.00) and the least affected domains were antiparkinsonian therapy and were evaluated during the “on” phase sexual function 100 (72.90,100.00) and cognitive function 80 of their medication, and were evaluated with the Addenbrooke (40.00,92.50). Further results will be presented. Cognitive Examination- revised and the INECO Frontal Screening. The primary outcome (action-verb processing) was assessed by time- Conclusion picture word paradigm. Motor impairment, bradykinesia and There is a dearth of literature regarding depression, anxiety, balance, were analyzed as secondary outcomes. fatigue, cognitive and physiological impairments are known to be prevalent among persons with MS in India. Thus, the findings of the Results study can be used as a baseline to create psychosocial intervention We found that active vs. sham tDCS led to an increase in the rate for women with Multiple Sclerosis taking treatment at NIMHANS. of correct responses in the time-picture word paradigm. This doi:10.1016/j.jns.2019.10.1321 improvement were more notable in verbs with high manipulability versus verbs with low manipulability.

Conclusion Our results showed that active stimulation of left IFG and M1 WCN19-1340 resulted in improvements in action-verb processing. Thus, our ﬁndings encourage further investigation exploring tDCS as an Poster shift 03 - Autonomic nervous system disorders/history of adjuvant therapy for cognitive treatment in PD. neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1320 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Gene-replacement therapy (GRT) in spinal muscular atrophy type WCN19-1331 1 (SMA1): Long-term follow-up from the onasemnogene abeparvovec phase 1/2A clinical trial

Poster shift 03 - Autonomic nervous system disorders/history of J.R. Mendella, R. Shellb, K.J. Lehmana, M. McCollya, L.P. Lowesa, L.N. neurology/motor neuron disease/movement disorders/MS & Alfanoa, N.F. Millera, M.A. Iammarinoa, K. Churcha, S.A. Spectorc, F.G. demyelinating diseases/neuromuscular disorders/vestibular dis- Ogrincd, H. Ouyange, E. Kernbauerf, S. Shahf,J.L’Italieng, D.M. orders and nutritional disorders or intoxications Sproulec, D.E. Feltnerc, S. Al-Zaidya aNationwide Children’s Hospital, Center for Gene Therapy, Columbus, Psychosocial correlates and quality of life among women living USA b with multiple sclerosis Nationwide Children’s Hospital, Section of Pulmonary Medicine- Department of Pediatrics, Columbus, USA c C. Vasanthraa, P.T. Thomasb, M. Netravathic AveXis-Inc., Clinical Development, Bannockburn, USA d aNational Institute of Mental Health and Neuro Sciences, Department of AveXis-Inc., Biostatistics, Bannockburn, USA e Psychiatric Social Work, Bengaluru, India AveXis-Inc., Biostatistics- Clinical Development, Bannockburn, USA ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 277 fAveXis-Inc., Clinical Operations, Bannockburn, USA fundus examination were normal. His random blood sugars were gAveXis-Inc., Regulatory and Quality, Bannockburn, USA 306 mg, glycosylated haemoglobin levels were 12.1 and fasting and post prandial sugars were 230 mg and 290 mg respectively. In view SMA1 is a rapidly progressing neurologic disease caused by of atypical presentation a CSF examination was done which revealed biallelic survival motor neuron 1 gene (SMN1) deletion/mutation. an albuminocytological dissociation. Nerve conductions revealed The SMN GRT onasemnogene abeparvovec (AVXS-101) treats the severe pure motor axonal neuropathy. genetic root cause of SMA and is designed for immediate, sustained Patient was started on IVIg along with good glycemic control and SMN protein expression. In a phase 1/2a trial (CL-101; on day 3 he started showing improvement, and by day 7 his power NCT02122952), SMA1 patients received a one-time AVXS-101 had improved to 3/5 proximally and 4-/5 distally. infusion at low dose (Cohort 1, n = 3) or proposed therapeutic dose Thus our case had a dramatic improvement with IVIg and (Cohort 2, n = 12), and demonstrated improved outcomes versus symptomatic control of blood sugar. The AMAN variant of GBS and natural history. anti GM1 positivity has a faster clinical recovery as compared to its SMA1 patients in CL-101 could rollover into a long-term follow- demyelinating counterpart. Thus we highlight an interesting associ- up (LTFU) study (Study LT-001; NCT03421977). The primary ation of a potentially treatable neuropathy in uncontrolled diabetes objective is long-term safety. Patients have annual visits (5 years), mellitus. then phone contact (additional 10 years). Patient record transfers are requested. Safety assessments include medical history/record review, doi:10.1016/j.jns.2019.10.1323 physical examination, clinical laboratory evaluation, and pulmonary assessments. Efﬁcacy assessments include developmental milestone evaluation to determine maintenance of the highest achieved milestone in the parent study. WCN19-1345 As of 8 March 2019, 10 patients from Cohort 2 (proposed therapeutic dose) had enrolled in Study LT-001. All patients remain Poster shift 03 - Autonomic nervous system disorders/history of alive, with a mean (range) age of 3.9 (3.4–4.8) years at 3.7 (3.3–4.3) years post-GRT; no additional requirement for ventilatory/nutritional neurology/motor neuron disease/movement disorders/MS & support was observed. No developmental milestones achieved in the demyelinating diseases/neuromuscular disorders/vestibular dis- CL-101 study were lost, supporting the durability of AVXS-101. No orders and nutritional disorders or intoxications long-term treatment-related adverse events have been observed. Updated data will be presented. Measurement of cortical thickness and volume of subcortical One-time AVXS-101 administration at the proposed therapeutic structures in multiple sclerosis: A single center retrospective dose continues to provide prolonged and durable efﬁcacy with study milestone development up to 4.3 years post-GRT in LT-001. Z. Abuzaid, O. Salih, T. Alharbi, E. Nassim, B. Shahid doi:10.1016/j.jns.2019.10.1322 King Fahad Specialist Hospital, Neurosciences Center, Dammam, Saudi Arabia

Background WCN19-1343 Alterations of the cortex during development and disease can be captured by measuring the cortical thickness across the whole Poster shift 03 - Autonomic nervous system disorders/history of brain. In addition, various studies have pointed towards a role of cortical thinning as a reliable index of atrophy in neurodegenerative neurology/motor neuron disease/movement disorders/MS & and neurological disease [1–3]. We aimed to evaluate whether demyelinating diseases/neuromuscular disorders/vestibular dis- cortical thickness and the volume of subcortical structures mea- orders and nutritional disorders or intoxications sured with FreeSurfer correlated with functional outcome of disease progression assessed by EDSS scores in Multiple Sclerosis (MS) An interesting association of Guillain Barre syndrome in uncon- patients. trolled diabetes mellitus Methods D. Joshia, R. Prasadb, P. Dashb, V.N. Mishrab, R.N. Chaurasiab,A. MRI data were retrospectively analyzed for 31 MS. FreeSurfer Pathakb software package was used to compute cortical thickness based on aDepartment of Neurology, Institute of Medical Sciences- Banaras Hindu the three dimensional T1-weighted images. University, Varanasi, India bInstitute of Medical Sciences- Banaras Hindu University, Neurology, Result Varanasi, India Modest correlation was observed between cortical thickness and clinical measures that included the age and EDSS. Diabetic neuropathy most commonly presents with a lengh dependant sensorymotor neuropathy. A predominantly/pure motor Conclusion and a non length dependant presentation of neuropathy in a diabetic Our results indicate that cortical thickness could serve as a useful is atypical and prompts us to search for other causes. measure in identifying subtle changes in MS patients. A 35 year old gentleman presented to us with us with acute flaccid quadripareisis which progressed over a period of 48 h causing References him to become bed bound. He was found to have proximal power of 0/5 and distal 1/5 in all 4 limbs, normal sensory and complete [1] Bradford C. Dickerson, Akram Bakkour, David H. Salat, et al., The arreflexia. There was no neck muscle/craniobulbar/respiratory in- cortical signature of Alzheimer's disease: regionally specific volvement. His CBC, potassium, CK/LDH, HIV, HBsAg status and cortical thinning relates to symptom severity in very mild to ARTICLE IN PRESS

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mild ad dementia and is detectable in asymptomatic amyloid- WCN19-1349 positive individuals, Cerebral Cortex 19 (3) (2009) 497–510. [2] Arnaud Charil, Alain Dagher, Jason P. Lerch, et al., Focal cortical atrophy in multiple sclerosis: relation to lesion load and Poster shift 03 - Autonomic nervous system disorders/history of disability, Neuroimage 34 (2) (2007) 509–517. neurology/motor neuron disease/movement disorders/MS & [3] Stefano Magon, Laura Gaetano, M. Mallar Chakravarty, et al., demyelinating diseases/neuromuscular disorders/vestibular dis- White matter lesion ﬁlling improves the accuracy of cortical orders and nutritional disorders or intoxications thickness measurements in multiple sclerosis patients: a longitudinal study, BMC Neuroscience 15 (1) (2014) 106. Alazami syndrome due to LARP7 gene mutation: Expanding the phenotype doi:10.1016/j.jns.2019.10.1324 A. Bajafar, F. Rifai, Y. Al-Said, E. Cupler King Faisal Specialist Hospital and Research Center, Neurosciences, Jeddah, Saudi Arabia

Introduction and the rationale WCN19-1348 Alazami syndrome is a rare autosomal recessive genetic disorder, characterized by severe mental retardation, speech delay, short Poster shift 03 - Autonomic nervous system disorders/history of stature and skeletal deformities. The genetic basis of the disease neurology/motor neuron disease/movement disorders/MS & consists of homozygous or compound heterozygous deletion of demyelinating diseases/neuromuscular disorders/vestibular dis- LARP7 gene (also known as PIP7s, ALAZS) located on chromosome 4q25, which is an essential regulator of gene expression. The gene orders and nutritional disorders or intoxications product is a small nuclear ribonucleoprotein that that inhibits transcription factor b that is required resuming transcription by Wernicke-Korsakoff syndrome and catatonia: A case report RNA polymerase II. We report a new family with an expanded phenotype. C.B. Braune, G.R. Freitas, M.C. Ribas, M.R. Figueiredo, R.W. Murari, A.R. Monfredinho, M.N.F.L. Castro, A.S. Cardoso, T.D. Pinheiro, L.F.H. Maia, Clinical history L.D.A. Peixoto, I. Barboza Three Saudi Arabian sisters, aged 26, 21, 20 years, born first- Federal Fluminense University UFF, Neurology, Niterói, Brazil degree consanguineous parents, presented early in life with developmental delay. The prenatal, birth and postnatal periods were A 28-year-old woman was admitted in emergency room 45 unremarkable. The sisters had a Rett-like phenotype that included days after childbirth with subacute onset of diplopia, horizontal motor and speech delay, mild dysmorphic facial features, short nystagmus and vertigo. She had a medical past of recurrent stature, and sterotopies including hand washing. One had trichotil- vomiting during pregnancy, and an esophagectomy with partial lomania that resulted in partial baldness and bezoar formation. A gastrectomy due to idiopathic achalasia three years before mild decrease of head circumference and low weight were noted. presentation. Her brain MRI was unremarkable. She was evaluated MECP2 sequencing was negative. Whole exon sequencing (Saudi by otorhinolaryngology team and was discharged with diagnosis of Diagnostic Laboratory Riyadh) revealed a homozygous deletion of bilateral vestibular neuritis. After one week, she returned with LARP7 (LARP7:NM_015454:exon5:c.502_552del:p.168_184del), clas- behavioral change. Her neurologic examination revealed restricted sified as a pathogenic loss of function mutation. extraocular motility to lateral gaze, vertical nystagmus, Babinski sign and dysarthria. Cognitive deficits appeared days before Conclusion admission and she quickly became catatonic. Initial laboratory The three sisters, who were presented with a Rett-like phenotype, tests were normal. Brain MRI showed increased signal in FLAIR were found to have homozygous loss of function LARP7 mutations sequence involving mammillary bodies, periaqueductal area, and thus expanding the phenotype of Alazami Syndrome. Additional periventricular region of the third ventricle. A clinical suspicion of work is required to understand the genetic pathogenicity and thiamine deficiency was considered, treatment with parenteral misfunction in LARP7 leads to these specific symptoms. thiamine was started, and rapidly improvement in catatonia and ocular motility was seen. Although the classic neurologic manifes- doi:10.1016/j.jns.2019.10.1326 tation of thiamine deficiency is Wernicke encephalopathy triad, patients may not fulfill the classic phenotype of ocular dysmotility, gait ataxia, and mental status changes in early presentations of the disease. Additionally, MRI may not be abnormal and can change WCN19-1357 over the course of disease. The classic signs of catatonia, which were lush in this case report, may be found in neurologic metabolic disorders, but is not described as a classic finding in Poster shift 03 - Autonomic nervous system disorders/history of thiamine deficiency. In this case report, in addition to catatonia as neurology/motor neuron disease/movement disorders/MS & an atypical manifestation, there was resolution of this phenotype demyelinating diseases/neuromuscular disorders/vestibular dis- after thiamine replacement. orders and nutritional disorders or intoxications

Mitochondrial membrane protein associated neurodegeneration (MPAN); First genetically conﬁrmed case in south Asia

a a b c doi:10.1016/j.jns.2019.10.1325 B. Senanayake , U. Dassanayake , K. Manju , R. De Silva aInstitute of Neurology, National Hospital, Colombo, Sri Lanka ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 279 bDevelopmental Neurosciences, UCL Great Ormond Street Institute of Department of Pediatrics, Singapore, Singapore Child Health, London, United Kingdom dFaculty of Medicine- Public Health and Nursing- Universitas Gadjah cEssex Centre for Neurological Sciences, Queen's Hospital, Romford, Mada, Department of Neurology, Yogyakarta, Indonesia United Kingdom eFaculty of Medicine- Public Health and Nursing- Universitas Gadjah Mada, Department of Surgery- Pediatric Division, Yogyakarta, Indonesia f Background/Introduction Faculty of Rehabilitation- Kobegakuin University, KNC Department of MPAN is an autosomal recessive disease of neurodegeneration Nucleic Acid Drug Discovery, Kobe, Japan with brain iron accumulation (NBIA) caused by homozygous mutations of the C19orf12 gene. MPAN accounts for 6–10% of NBIA Background cases, with no more than 80–100 genetically confirmed individuals Dystrophin protein, produced by DMD gene, has essential role to worldwide. We present a case of a young Sri Lankan with maintain the muscle integrity. Mutation occurs in DMD gene can Parkinsonism and cognitive decline which was genetically confirmed cause a complete absence of dystrophin, resulting progressive and as MPAN. severe muscle damage in Duchenne Muscular Dystrophy (DMD), or a partial functional protein, resulting in a milder phenotype, Becker Case report Muscular Dystrophy (BMD). DMD gene mutation profile of DMD and A 24 year old female presented six years ago with impaired gait BMD patients in Indonesian has never been reported. In order to and bilateral upper limb rest tremors. Examination revealed clinically apply mutation-specific therapies, such as exon skipping or extrapyramidal features. Metabolic screening including iron and read-through therapy, precise mutation analysis of DMD gene is copper studies and blood picture were normal. A diagnosis of young necessary. This study was performed to identify the mutation profile onset Parkinsonism with neuropsychiatric features was made. She is of the DMD gene in the Indonesian DMD and BMD patients so that Levodopa responsive but developed early dyskinesias. The response feasible therapeutic strategies can be developed. gradually waned with time. Psychiatric symptoms, impaired speech, dysphagia and loss of ambulation followed. Initial MRI showed Methods increased iron deposition in the head of the caudate, putamen, Forty-three male patients with high serum creatine kinase and globus pallidus and substantia nigra. Whole genome exome positive Gower sign were recruited from Sardjito Hospital and UGM sequencing revealed likely homozygous C19orf12 variants, findings Academic Hospital. Multiplex Ligation-Dependent Probe Amplifica- confirmed by Sanger sequencing. tion (MLPA) was performed to detect mutations in DMD gene.

Discussion Results MPAN characteristically presents in childhood with gait changes. Study of 43 patients exhibited that age of disease onset varied Later progressive dystonia, parkinsonism, cognitive decline, and from 1 to 9 years old (mean 5.1 years old). Nineteen patients neuropsychiatric symptoms develop in more than 50%. Axonal motor (44.18%) were wheelchair bound, starting at 9 to 13 years old (mean neuropathy and optic atrophy are associated. Life expectancy is age of 11 years old). MLPA result showed 30 deletions (69.77%) and variable. MRI shows increased iron accumulation in the globus 5 duplications 11.63% cases, meanwhile 8 cases (18.6%), showing pallidus and substantia nigra. Treatment is aimed at symptom DMD phenotype, had no deletion nor duplication. control with regular follow-up to detect complications. Conclusion Conclusion MLPA is practical technique to identify deletion and duplication Despite the rarity, NBIAs is an important differential in young in DMD gene. The mutation proﬁle depicted in this study provides patients presenting with progressive dystonia/extrapyramidal symp- better picture of DMD or BMD cases in Indonesian population. toms. To our knowledge, there has been no previous description from the South Asian littoral. doi:10.1016/j.jns.2019.10.1328 doi:10.1016/j.jns.2019.10.1327

WCN19-1365

WCN19-1361 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Poster shift 03 - Autonomic nervous system disorders/history of demyelinating diseases/neuromuscular disorders/vestibular dis- neurology/motor neuron disease/movement disorders/MS & orders and nutritional disorders or intoxications demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Analysis of the characteristics of some patients with hereditary transthyretin amyloidosis hATTR that do no stabilize after DMD gene analysis of Duchenne and Becker muscular dystrophy Patisiran treatment patients in Indonesia L. Galan, L. Silva, A. Guerrero-Sola, A. Horga, V. Pytel, J. Matias-Guiu E. Dwianingsiha, K. Iskandarb, L.C. Pingc, R.G. Maluekad,G.Fnue,L. Hospital Clinico San Carlos IdISSC, Neurology, Madrid, Spain Pratiwia, S. Hapsarab, M. Matsuof, L.P. Sanc a Faculty of Medicine- Public Health and Nursing- Universitas Gadjah Introduction Mada, Department of Anatomical Pathology, Yogyakarta, Indonesia Hereditary Transthyretin (TTR) Amyloidosis is a progressive b Faculty of Medicine- Public Health and Nursing- Universitas Gadjah systemic autosomal dominant disease, causing neuropathy and Mada, Department of Pediatric, Yogyakarta, Indonesia cardiopathy as main manifestations. Without treatment, it is c Yong Loo Lin School of Medicine- National University of Singapore, potentially mortal. Recently, a clinical trial has shown that patisiran, ARTICLE IN PRESS

280 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx a SiRNA, stabilizes or improves the disease in 57% of patients. Results However, 43% patients still progress, maybe in a lesser degree than 60% were male. Mean age was 57.26 (males) and 58.3 (females). natural history. We have recently presented our experience with 8 Duration of disease was (p = 0.0035*) higher in males. No patients with patisiran in “real life”. The objective of this work is to significant variation in mean absolute L-dopa response was observed analyze the characteristics of the patients that did not stabilize with in both. Genotype association with AUC of L-dopa, the COMT gene the treatment. SNP at the promoter region rs6269 (G/A) (GG VS AA = 3811 ± 1010, N = 2; 1662 ± 306.6, N = 12; P = 0.02) and one nonsynonymous Methods change rs4680 (G/A: Val158Met) (GG VS AA = 2881 ± 361.5, N = In the CT benefit was assessed according to mNIS+7. However, as 12; 1237 ± 321.4, N = 9; P = 0.004) have shown a significant this is not feasible in clinical practice, we considered a patient as not association. The two synonymous changes rs4633 (C/T) and rs4818 stabilized when in 6 months: (G/C, Leu136Leu) were not significant. In genotype association with dyskinesia score, the COMT gene SNP at the promoter region rs6269 1. NIS or Compass31 increased N5 points or and the non synonymous change rs4680 have shown a significant 2. RODS reduced b5 points or association. Conclusion: The AUC of Levodopa was found signifi- 3. mBMI reduced N5% or cantly high with variant alleles of rs4633 (C/T), rs4680 (G/A) of 4. Pain increased. COMT polymorphisms and are significantly associated with Dyskinesias. Results Of a total of 8 patients, 4 patients treated for 12–18 months doi:10.1016/j.jns.2019.10.1330 showed no long-term stabilization [age range 68-82 yrs, mutations Val50Met (3), initial NIS 45-125, stage II Coutinho (4), PND IIIb (4), TTR block N80% (4)]. Two had received previously tafamidis and 3 of them skipped N2 doses of patisiran. Three of them still referred some benefit with the treatment WCN19-1371

Discussion Poster shift 03 - Autonomic nervous system disorders/history of Although this is a small sample, an advanced disease stage at the neurology/motor neuron disease/movement disorders/MS & beginning of treatment appears to be a common denominator. demyelinating diseases/neuromuscular disorders/vestibular dis- Skipping doses could also had affected response to Patisiran. A better tool to measure treatment effect as well as better predictors of orders and nutritional disorders or intoxications response are needed. Diet quality and multiple sclerosis severity: Is there a link? doi:10.1016/j.jns.2019.10.1329 A. Souissia, S. Mrabetab, F. Larnaouta, A. Nasriab, M. Ben Jebaraab,A. Gargouriab, I. Kacemab, R. Gouiderab aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia b WCN19-1370 University of Tunis El Manar, Faculty of Medicine of Tunis, Tunis, Tunisia

Poster shift 03 - Autonomic nervous system disorders/history of Background and objectives neurology/motor neuron disease/movement disorders/MS & The contribution of diet to Multiple Sclerosis (MS) risk and course demyelinating diseases/neuromuscular disorders/vestibular dis- was of great interest. Diet could be a modifiable contributor to orders and nutritional disorders or intoxications disease progression. Our goal was to assess the impact of diet on severity of remitting MS. Association of genetic polymorphism with pharmacokinetics of Levodopa in South Indian Parkinson's disease population Materials and methods We conducted a prospective study, from February to May 2019, in R. Borgohaina, R. Kandadai Mridulab, T.F. Sdc, T.F. Syedb, V.K. Kutalac the department of Neurology of Razi Hospital in Tunisia. We included aNeurology, Hyderabad, India patients who were diagnosed with remitting MS. Adherence to bNizam's Institute of Medical Sciences, Neurology, Hyderabad, India Mediterranean dietary was assessed via the Mediterranean adher- fi cNizam's Institute of Medical Sciences, Clinical Pharmacology and ence score (MAS). Totals of six or higher de ned the range of highest fi Therapeutics, Hyderabad, India bene t. Body mass index (BMI) was calculated to categorize patients into four groups: underweight, normal weight, overweight, or obese. We quantified MS severity using the Multiple Sclerosis Severity Score Objective (MSSS). Data was collected and analysed using SPSS-23. To evaluate the role of COMT gene variants on the pharmacokinetics (PK) of L-dopa in South Indian PD population. Results We included 62 patients consisted of 51 women and 11 men Method (sex-ratio F/M = 4.63). Mean BMI was 25 ± 4 (range = [16-36]). 30 PD patients were recruited based on UKPDS Brain Bank MSSS was independent from cigarette smoking and coffee or tea criteria. The motor activity improvement following L-dopa adminis- consumption. Alcohol drinking was associated to a mild MSSS (p = tration was reported as L-dopa response (aLR, %LR). Five hours (0–4 0.032). Mean MAS was 3.3 ± 1.5 (Range = [1-6]). Seven patients h after L-dopa administration) blood samples were collected and had a highest benefit diet. A MAS under 6 was associated with a peak plasma concentrations were analysed by HPLC method. DNA higher number of relapses during the first year (p = 0.03) but was was isolated. Genotyping was done by RFLP method and validated by independent from MSSS. Sanger’s sequencing. ARTICLE IN PRESS

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Conclusions WCN19-1387 Mediterranean diet was not associated with MS severity in our cohort. However, low Mediterranean adherence score was linked to an active disease during the ﬁrst year. Our results were partly consistent Poster shift 03 - Autonomic nervous system disorders/history of with other studies and need to be conﬁrmed in larger series. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- doi:10.1016/j.jns.2019.10.1331 orders and nutritional disorders or intoxications

Pantothenate kinase associated neurodegeneration (PKAN); An atypical late-onset presentation with genetic conﬁrmation WCN19-1385 B. Senanayakea, U. Dassanayakea, M. Kurianb, R. De Silvac aInstitute of Neurology, National Hospital, Colombo, Sri Lanka Poster shift 03 - Autonomic nervous system disorders/history of bDevelopmental Neurosciences, UCL Great Ormond Street Institute of neurology/motor neuron disease/movement disorders/MS & Child Health, London, United Kingdom demyelinating diseases/neuromuscular disorders/vestibular dis- cEssex Centre for Neurological Sciences, Queen's Hospital, Romford, orders and nutritional disorders or intoxications United Kingdom

Autonomic dysfunction in patients with essential tremor Background/Introduction PKAN is the commonest form of neurodegeneration with brain A. Rekika, A. Nasriab, I. Kacemab, S. Mrabetab, A. Gargouriab, M. Ben iron accumulation (NBIA). It is early onset (b10 years) and rapidly Djebaraab, R. Gouiderab progressive in the typical form and the atypical late onset aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia (N10 years) form is slowly progressive. We report probably the bUniversity of Tunis El Manar, Faculty of Medicine of Tunis, Tunis, ﬁrst genetically conﬁrmed case of late onset disease in a Sri Tunisia Lankan.

Background Case report Essential tremor (ET) is henceforth considered as multi-systemic A 32-year-old woman presented with progressively worsening neurodegenerative disorder characterized by a combination of motor and right arm dystonia of one year with a reasonable response to non-motor symptoms including autonomic dysfunction. Our aim was to anticholinergics. Over 10 years, her cervical dystonia required explore neurophysiologic autonomic dysfunction in patients with ET. regular Botulinum toxin injections. Also dysarthria, dysphagia and oro-lingual dystonias developed. A metabolic screen including Methodology copper and iron studies were unremarkable. T2-weighted brain A prospective study was conducted in the department of MRI showed bilateral symmetrical central hyperintensities sur- Neurology in Razi Hospital (January2018-April 2019) including rounded by hypointense signal in the globus pallidi (“eye-of-the- consecutive patients with ET according to the Tremor Investigation tiger” sign). Genetic testing by exome sequencing suggested Group (TRIG) criteria. The sympathetic autonomic system (AS) was compound heterozygous variants of PANK2, findings replicated by assessed with the sympathetic skin response (SSR) evaluated at the Sanger sequencing. One of the mutations (c.1441CNT p.Arg481*) has four limbs. Absent SSR at either one or more extremities was previously been reported in hyperprebetalipoproteinemia, regarded as abnormal. Parasympathetic AS was assessed according to acanthocytosis and retinitis pigmentosa (HARP) syndrome. Ewing’s battery (Heart rate variation to deep breathing, to Valsalva, heart rate response to standing). Discussion In this case slowly evolving segmental dystonia was present Results accompanied by “typical” brain MRI appearances of PKAN. The Fifty ET patients were included with a sex-ratio of 1,08 (26 men and atypical presentation and slow progression in this patient may be 24 women) and a mean age of 64.5years [20–86]. Neurophysiologic explained by the compound heterozygosity. Atypical PKAN accounts dysautonomia was found in 74% of ET. Heart rate variability was for 25% of cases. In both forms treatment remains symptomatic with reduced in 60% of ET, mostly to standing in third of the patients. no clinical benefit from iron chelation. Parasympathetic dysfunction was early in 86,7% and definite in 13,3%. Sympathetic dysautonomia was found in 30%. Only 9 patients (18%) Conclusion had both sympathetic and parasympathetic autonomic dysfunction. NBIA is an important differential in progressive extrapyramidal disease with supportive MRI findings. Atypical presentation and slow Conclusions progression may be explained by interallelic compensation in the Our study showed the high prevalence of autonomic dysfunction compound heterozygous variant. on electrophysiological assessment in ET affecting mostly parasympathetic but also sympathetic autonomic system.

doi:10.1016/j.jns.2019.10.1333 doi:10.1016/j.jns.2019.10.1332 ARTICLE IN PRESS

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WCN19-1398 Hiccups are reﬂexes characterized by the onset of sudden diaphragmatic and intercostal muscle contraction immediately followed by laryngeal closure leading to the production of a “hic” Poster shift 03 - Autonomic nervous system disorders/history of sound. Hiccup is experienced a number of times through life, it’s neurology/motor neuron disease/movement disorders/MS & usually self-limiting and resolves on its own. However, when it demyelinating diseases/neuromuscular disorders/vestibular dis- becomes intractable (lasting more than a month), it poses a serious orders and nutritional disorders or intoxications chronic ailment that causes exhaustion, dehydration, weight loss, poor sleep and eating patterns. Intractable hiccup is a common complica- Experience in using azathioprine for multiple sclerosis in tion of brain surgery but little is known about its pathophysiology and developing country: A preliminary study exact cause, although a number of factors has been implicated with the autonomic system playing a major role in one such involvement. A M.B. Rahmawati case of intractable hiccup in Dammam, Saudi Arabia was explored. In Faculty of Medicine Diponegoro University, Neurology, Semarang, this study we reviewed the literature and found the role of the CNS Indonesia and PNS in the onset of hiccups as well as known treatment methods for intractable hiccups and future prospects. Background doi:10.1016/j.jns.2019.10.1335 Incidence of multiple sclerosis has recently increased in developing countries, including Indonesia. Currently FDA-approved drugs for modifying disease are still expensive and not covered by insurance in Indonesia. Therefore Azathioprine is promising as alternative treatment to prevent and reduce relaps of multiple sclerosis in developing country. WCN19-1402

Aim Poster shift 03 - Autonomic nervous system disorders/history of To evaluate the use of azathioprine in multiple sclerosis and neurology/motor neuron disease/movement disorders/MS & review the relevant literature demyelinating diseases/neuromuscular disorders/vestibular dis- Methods orders and nutritional disorders or intoxications Three patients treated with azathioprine 0.7–2.5 mg/kg per day for 24 months were evaluated in a retrospective follow-up study. Stiff person syndrome - An unusual paraneoplastic neurological phenomenon in carcinoid tumour Results Two patients were free from relaps during two years treatment with V. Sahathevana, T. Herathb, D. Gamlakshac, N. Yogendranathana,A. Azathioprine. One patient suffered from relaps after tapering off of Kulatungaa, B. Senanayakeb Azathioprine (after two years treatment). Side effects were few and aNational Hospital Sri Lanka, Department of Medicine, Colombo 10, Sri were well-tolerated. Hematologic profile was unremarkedly influenced. Lanka bNational Hospital Sri Lanka, Department of Neurology, Colombo 10, Sri Conclusion Lanka Azathioprine might be an effective and cheaper alternative in the cNational Hospital Sri Lanka, Department of Pathology, Colombo 10, Sri treatment of multiple sclerosis, especially in developing country. Lanka Long-term use might be needed to prevent or minimize relaps of multiple sclerosis, but its long-term toxicity remains unclear. Stiff person syndrome (SPS) is a rare neurologic disorder characterised by rigidity of the truncal and proximal limb muscles Keywords: Multiple sclerosis, Azathioprine, Relaps with intermittent superimposed spasms. It’s unique because it lacks similarity to any other neurologic disorder. Possibly tetanus is the closest related condition with both inhibiting central gamma- aminobutyric (GABA) systems. SPS is extremely rare with less than doi:10.1016/j.jns.2019.10.1334 20 cases reported from South Asia which has a population of nearly 2 billion. In its classic form, it is associated with the presence of high titres of glutamic acid decarboxylase (GAD) antibodies. Par- aneoplastic SPS comprising of around 5% of the patients has been WCN19-1401 reported with malignancies of the breast, colon, lung, thymus and Hodgkin’s lymphoma. Antibodies against amphiphysin and gephyrin Poster shift 03 - Autonomic nervous system disorders/history of are detected in paraneoplastic SPS. We report a 58 year old Sri Lankan male with SPS with a high GAD antibody titre and classical neurology/motor neuron disease/movement disorders/MS & electro myographic (EMG) changes, who was found to have an demyelinating diseases/neuromuscular disorders/vestibular dis- underlying carcinoid tumour. We postulate that SPS was a par- orders and nutritional disorders or intoxications aneoplastic phenomenon secondary to the carcinoid in this case. Although neurological syndromes such as sensory neuropathy, The role of autonomic nervous system in intractable hiccup: A limbic encephalitis and, myelopathy have been described as review paraneoplastic features in carcinoid, we believe this is the first report of SPS associated with carcinoid tumour. A. Idris-Agbabiakaa, A. Jhaa, S. Sarkara, M. Millaneisea, I. Agbabiakab aTexila American University, College of Medicine, East Bank Demerara, Guyana doi:10.1016/j.jns.2019.10.1336 bMinistry of Health/Eye Unit, Dept. of Surgery, Dammam, Saudi Arabia ARTICLE IN PRESS

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WCN19-1407 WCN19-1411

Neurophysiological study of autonomic nervous system in Onasemnogene abeparvovec gene-replacement therapy (GRT) for amyotrophic lateral sclerosis spinal muscular atrophy type 1 (SMA1): Global pivotal phase 3 study program (STR1VE-US, STR1VE-EU, STR1VE-AP) M. Ben Mahmouda, I. Kacemab, A. Nasriab, S. Mrabetab, M. Djebaraa, A. Gargouri-Berrechidab, R. Gouiderab E. Mercuria, G. Baranellob, J.W. Dayc, C. Brunod, S. Cortie, C.A. aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia Chiribogaf, T.O. Crawfordg, B.T. Darrash, R.S. Finkeli, A.M. Connollyj, bUniversity of Tunis El Manar, Faculty of Medicine, Tunis, Tunisia S.T. Iannacconek, N.L. Kuntzl, R. Massonb, L.D.M. Peñam, F. Baldinettin, M. Schultzo, P.B. Shiehp, E.C. Smithq, K. Saitor, M. Scotos, S.A. Spectort, u v w s Introduction A. Authors Truncated , D.M. Sproule , J.R. Mendell , F. Muntoni a Autonomic nervous system (ANS) involvement is increasingly Catholic University, Department of Paediatric Neurology and Nemo recognized in Amyotrophic Lateral Sclerosis (ALS). However, only Clinical Centre, Rome, Italy b few studies had demonstrated the value of ANS investigations. Fondazione IRCCS Istituto Neurologico Carlo Besta, Developmental Neurology Unit, Milan, Italy c Objective Stanford University Medical Center, Department of Neurology, Stanford, The aim of this neurophysiological study was to assess ANS in USA d ALS. Istituto Giannina Gaslini, Translational and Experimental Myology Centre, Genoa, Italy e Methods University of Milan, Dino Ferrari Centre- Neuroscience Section- We performed a prospective population-based study of patients Department of Pathophysiology and Transplantation DEPT- Neurology with ALS followed in the department of Neurology of the Razi Unit- IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, hospital of Tunis from 2017 to 2019. Each patient had a clinical Milan, Italy f examination. ANS was assessed using the Ewing’s battery which Columbia University Medical Center, Division of Pediatric Neurology, included standardized tests. Tests of parasympathetic function New York, USA g included heart rate variation with deep breathing, Valsalva maneu- Johns Hopkins Medicine, Department of Neurology, Baltimore, USA h ver and heart rate response to active standing. Tests of sympathetic Bostoen Children’s Hospital, Department of Neurology, Boston, USA i adrenergic function included analysis of skin response. Nemours Children’s Hospital, Division of Neurology- Department of Pediatrics, Orlando, USA j Results Nationwide Children’s Hospital, Department of Neurology, Columbus, Thirty-two patients (9 women, 23 men) were enrolled in our USA k study. Mean age at disease onset was 53.5 years. Spinal-onset was University of Texas Southwestern Medical Center, Department of predominant (81%). ANS was impaired in 88% of cases: early Pediatrics, Dallas, USA l parasympathetic dysautonomia (63%), defined dysautonomia (9%), Ann and Robert H. Lurie Children’s Hospital of Chicago, Division of and sympathetic dysautonomia (19%). 16% patients had both Neurology, Chicago, USA m sympathetic and parasympathetic dysfunction. We did not find a Cincinnati Children’s Hospital, Division of Human Genetics, Cincinnati, correlation between the ANS impairment and clinical features USA n related to ALS. AveXis- Inc., EMEA Medical Affairs, Bannockburn, USA o Discussion AveXis- Inc., Translational Medicine, Bannockburn, USA p Our results confirm findings reported by several authors and David Geffen School of Medicine at UCLA, Department of Neurology, Los revealed that ALS involvement is not limited to motor system but Angeles, USA q also disturbs the ANS, predominantly affecting parasympathetic Duke University School of Medicine, Department of Pediatrics, Durham, function. These findings suggest that ALS disease should be USA r considered as multisystem degenerative disorder. Tokyo Women's Medical University, Institute of Medical Genetics, Tokyo, Japan s Conclusion University College London- Great Ormond Street Institute of Child Dysautonomia might worsen neurological course during ALS Health & Great Ormond Street Hospital, The Dubowitz Neuromuscular disease, it should be looked for and treated to improve quality of Centre, London, United Kingdom t life of these patients. AveXis- Inc., Clinical Development, Bannockburn, USA uAvexis- Inc., Medical Affairs, Bannockburn, USA v doi:10.1016/j.jns.2019.10.1337 Clinical Development, Bannockburn, USA wNationwide Children’s Hospital, Center for Gene Therapy, Columbus, USA ARTICLE IN PRESS

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SMA1 is a rapidly progressing neurologic disease caused by loss of Material and methods function of the survival motor neuron 1 gene (SMN1). Two groups of study were identified: 30 patients with typical Onasemnogene abeparvovec (AVXS-101), a one-time, intravenous, CIDP and 30 patients with atypical CIDP. All the functional and investigational GRT treats the genetic root cause of SMA and is clinical tests mentioned above were performed in both groups. designed for immediate, sustained neuronal SMN expression. In a phase 1/2a study, AVXS-101 demonstrated exceptional improve- Results ments in survival and motor function. We report data from the global We found only medium level correlations in the range 0,4-0,7 with phase 3 STR1VE program evaluating AVXS-101 in the United States statistical importance or no correlation at all. The duration of the (US; NCT03306277), European Union (EU; 2017-000266-29/ disease and the decrease in muscle strength in the upper or lower NCT03461289), and Asia Pacific (AP; NCT03837184). limbs increases the degree of disability in patients with typical CIDP. In The STR1VE trials are multicenter, open-label, single-arm studies contrast, in the atypical CIDP group, the degree of disability is higher in symptomatic SMA1 patients b6 months (biallelic SMN1 deletion/ when conduction blocks are present on nerves conduction studies. mutations, 1–2 SMN2 copies). Primary outcomes: independent sitting ≥10 (EU, AP) or ≥30 s (US) at 18 months; event-free survival Conclusions (no death/permanent ventilation) at 14 months (US only; secondary The damage of profound sensitivity and proprioception plays a outcome in EU, AP). Secondary/exploratory outcomes: independence greater role in atypical CIDP then muscle weakness. The correlation of ventilator support; motor function improvements (Bayley-III, equations established by multiple regression can predict the CHOP INTEND). evolution of disability in CIDP patients. US enrollment is complete (N = 22). As of 8 March 2019, survival was improved compared with natural history among patients who doi:10.1016/j.jns.2019.10.1339 could have reached 13.6 months of age at datacut; 11/22 patients (median age at datacut: 14.4 months) achieved sitting without support for ≥30 seconds; 21/22 patients have achieved a CHOP INTEND score ≥40 points (maximum 64). As of 24 April 2019, 27 WCN19-1437 patients have been enrolled in STR1VE-EU (30 planned). STR1VE-AP is currently enrolling patients (6 planned). Updated data (pooled) will be presented. Poster shift 03 - Autonomic nervous system disorders/history of In STR1VE, AVXS-101 has demonstrated significant therapeutic neurology/motor neuron disease/movement disorders/MS & benefit in prolonging survival and improving motor function in demyelinating diseases/neuromuscular disorders/vestibular dis- symptomatic infants with SMA1. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1338 Prognosic factors associated with disability in multiple sclerosis

O. Shulga Volyn RegionalClinical Hospital, Neurology, Lutsk, Ukraine WCN19-1424 Background Poster shift 03 - Autonomic nervous system disorders/history of Predicting of long-term prognosis of clinically isolated syndrome ﬁ neurology/motor neuron disease/movement disorders/MS & (CIS) of Multiple Sclerosis (MS) remains dif cult task. It has been established that demography, level of disability, emotions, presence demyelinating diseases/neuromuscular disorders/vestibular dis- of oligoclonal bands can inﬂuence the course, as well as long-term orders and nutritional disorders or intoxications outcome of MS.

Pecularities of correlations between functional and laboratory Objective ﬂ tests in atypical forms of chronic in ammatory demyelinating To explore factors of disability and prognosis over 5 years in polyneuropathies patients with CIS

E. Gavriliuc, V. Lisnic, V. Nemtsan, L. Munteanu Methods Nicolae Testemitsanu State University of Medicine and Pharmacy, We conducted a longitudinal study of 30 patients who attended Neurology, Chisinau, Republic of Moldova Volyn Regional Clinical Hospital. We examined the associations between demographic, clinical, psychosomatic, imaging and labora- Background tory factors in patients with CIS using nonparametric Spearman’s There are still not yet known all clinical and laboratory correlation coefficient, Kruskal-Wallis test and Unifactor dispersion peculiarities of atypical chronic inflammatory demyelinating poly- analysis of variance ANOVA for multivariate analysis. To assess neuropathy (CIDP), ranging from only sensitive symptoms without atrophic processes in patients with CIS, measurement of 23 linear weakness to asymmetric motor deficit. In our study we tried to parameters and 14 indices was performed for each patient at least analyse the relationship between the data obtained using the scores twice in dynamics. of disabilities in CIDP (Overall Neuropathy Limitation Scale, 9 holes peg test, INCAT sensory score and 10 meters test) and clinical Results (muscle strength in limbs, absence of deep-tendon reflexes, etc.), 30 patients with CIS aged 32,07 ± 8,54 years, were observed paraclinical (nerves conduction studies of motor and sensory during 33.10 ± 12.81 months. The median time to second attack was nerves, level of proteins in CSF, superficial peroneal nerve biopsy) 6.33 ± 3.47 months. Corpus callosum index was the most predictive results. index of 0.4 ± 0.32 and correlated with shock reaction (p b 0,001) ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 285 and EDSS step (p b 0,001). There were no signiﬁcant difference for Conclusions patients with anxiety and relief reaction at the time of CIS. Our results suggest that pareidolic illusions were associated with loss of attention as frontal lobe dysfunction and decline of visual Conclusions memory in demented state. We identiﬁed that emotional reaction of the diagnosis at the time of CIS and corpus callosum index are the strongest predictors of disability in CIS. doi:10.1016/j.jns.2019.10.1341 doi:10.1016/j.jns.2019.10.1340

WCN19-1448

WCN19-1441 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Poster shift 03 - Autonomic nervous system disorders/history of demyelinating diseases/neuromuscular disorders/vestibular dis- neurology/motor neuron disease/movement disorders/MS & orders and nutritional disorders or intoxications demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications A case series of Lewis-Sumner syndrome

Correlation with Pareidolia and neuropsychological state in T. Mguidich, O. Hdiji, H. Haj Kacem, S. Sakka, N. Farhat, M. Damak, M. Parkinson's disease and other syndrome: A retrospective study Chokri Habib Bourguiba Hospital, Neurology, Sfax, Tunisia T. Tokashiki, H. Akamine National Hospital Organization- Okinawa National Hospital, Neurology, Introduction Ginowan, Japan Lewis and Sumner syndrome (LSS) is a clinical and electrical entity distinguished from chronic polyradiculoneuropathies (PIDC) Objective and pure motor neuropathies with multifocal conduction blocks The aims of this study are to investigate the positive rate of the (NMM). We report 3 cases of LSS. Pareidolia test in Parkinson’s syndrome (Psyn) with or without dementia and the responsible lesion for Pareidolia using by the Patients and methods neuropsychological tests. We included all patients diagnosed with LSS in the department of Neurology, at Habib Bourguiba Hospital between 1992 and 2018. We Background reported clinical, electrophysiological and therapeutic characteristics Pareidolia is observed as visual illusion in patients with dementia of these patients. with Lewy bodies (DLB). In Parkin^son's disease without dementia (PDwoD), It was reported that the correlations between pareidolic Results illusions and hypometabolism measured by 18F-FDG-PET were seen Three patients were included (mean age: 37 ± 15.6 years; sex in the bilateral temporal, parietal and occipital cortexes. But the ratio: 3). All of them had no medical history. Two patients had an responsible lesion of the Pareidria with or without dementia is not upper limb onset. Paresthesias were inaugural in two patients. Mean well known. disease duration was 7.3 ± 1.3 months. Clinical examination showed sensorimotor involvement in 2 patients, and purely motor involve- Methods ment in 1 patient. Cerebrospinal fluid protein level was elevated in 1 We enrolled 160 cases (73 ± 8.9 years old, 87 females) with patient (0.7 g/L). Electrophysiological studies showed features of Parkinson's syndrome with or without dementia. The Pareidolia test asymmetrical and multifocal sensorimotor demyelinating neuropa- and other neuropsychological tests (Mini mental state examination thy with persistent conduction blocks. Steroids were used as first- Japanese version (MMSE-J), Frontal Assessment Battery (FAB), line therapy in 2 patients and were insufficiently effective requiring Benton Visual Retention test (Benton) and Clinical Assessment for the use of immunosuppressive therapy in 1 patient. The third patient Attention (CAT)) were undertaken. We divided them into 5 groups was unsuccessfully treated with intravenous immunoglobulin but (DLB, Parkinson disease with dementia (PDD), PD without dementia had dramatically improved after steroids. (PDwoD), other Dementia (DMT) and Psyn) and analysed. Statistical analysis was performed by JMP version 11. Conclusion LSS is an entity that should be well known and distinguished Results from other neuropathies. Its treatment is not well codified and needs The positive ratio of Pareidolia is highest in DLB and PDD, 81.3% to be discussed on a case-by-case basis. and 79.6%, respectively. That of PDwoD was 28.6% and lower than DMT. In psychological test, The positive pareidolic responses were related to lower value of MMSE-J, FAB, Benton and attention ablility doi:10.1016/j.jns.2019.10.1342 (SDMT, PASAT and Position stroop test). ARTICLE IN PRESS

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WCN19-1449 WCN19-1458

Improving neuroprotective effects of resveratrol by brain New methods of treatment for vascular Parkinsonism and targeting through chitosan glutamate nanoparticles in MPTP Parkinson's disease: Tempo-rhythmal correction combined with induced Parkinsonism special exercise and music

M. Rahman, V. Kumar D. Akramova, G. Rakhimbaeva FHS- SHUATS, Pharmaceutical Sciences, Allahabad, India Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan

Objective Methods Resveratrol is a naturally obtained polyphenol and phytoalexin, We have created a new treatment and diagnostic program for widely received significant attention in neurodegenerative disorders, Parkinson's disease (PD). The program treats and diagnoses perva- i.e, Parkinsonism. Its poor solubility, photostability and lower sive disorders of PD and Vascular parkinsonism (VP) with music and bioavailability limited their immense potential. Conventional based various exercises in order to s used method of Tempo-rythmal formulation doesn’t meet the optimum therapeutic effects. To correction (TRC). overcomes the challenges, our study aims to develop and evaluate All patients were divided into 4 group.1st group -25 patients the neuroprotective effects of resveratrol (RV) using chitosan who have VP and they have received both medicamentous glutamate nanoparticles (RV-CG-NPs) in a mouse model with MPTP treatment (MT) and TRC with music and exercises. 2st In the induced Parkinsonism. group-25patients were patients with PD, they have also received MT and TRC with music and exercises. Group 3 was a group of 25 Methods VP patients they have received only MT. 4th group of 25 PD The nanoparticles were prepared by ionic gelation method using patients they only MT. Patients walked in the morning under quiet chitosan and tripolyphosphate, and characterized for particle size music, on the midday under quickened and on the evening under distribution, encapsulation efficiency and in vitro drug release. fast rythm music. Moreover, the optimized nanoparticles were administered via intranasal route in C57BL/6 mice induced with parkinsonism by 1- Results methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). In st group the maximal positive result according “vital activity” and was 2.87 ± 1.36. According to a special survey, "quality of life" Results was 50% higher than the initial result/in the first days of TRC in all The optimized nanoparticles exhibited particle size of 131 nm, patients this index was 25or28%and 31or33%on the last day of polydispersity index (PDI) of 0.21 and entrapment efficiency of 89%. TRC. Pharmacodynamically, MPTP made significant impairment on social In patients of 2nd group according the maximal positive outcome recognition memory along with generation of striatal oxidative stress was on "vital activity" and is 2.23 ± 1.16.The average steps in and reduced expression of tyrosine hydroxylase in striatum. patients with PD was 21or 23% on the first day of the TRC, and 26% in Biodistribution study of nanoparticle formulation in brain of C57BL/ the last days of the TRC. According to a special survey, "quality of 6 mice showed significantly higher brain levels of resveratrol as life" was 40%. Groups 3 and4 only MT and correction was not compared to the plain resveratrol solution. The Cmax (890.12 ng/ml) conducted in group patients. and AUC (1986.51 ng h/ml) of said optimized nano-formulation (i.n) were found to be significantly higher in the brain at all time points Conclusion compared to RV solution (i.n) and chitosan glutamate-RV-NPs (i.v). TRC with music is method of treatment and rehabilitation, which is effective in each type of VP and PD also in eco- Conclusion nomical aspect that patient can use both in the hospital and in the Overall, the results from the current work showed enhanced home. brain levels and neuroprotective effect resveratrol when administered through nanoparticles, which could be a substantial achievement of direct nose-to-brain targeting in Parkinson's disease. doi:10.1016/j.jns.2019.10.1344 doi:10.1016/j.jns.2019.10.1343 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 287

WCN19-1466 Introduction Following our work on the validation of onboard smartphone sensors for the assessment of abnormal tremors and bradykinesia, Poster shift 03 - Autonomic nervous system disorders/history of we wanted to assess compliance when users exploited a feature of neurology/motor neuron disease/movement disorders/MS & MyTremorApp where multiple recordings per day during multiple demyelinating diseases/neuromuscular disorders/vestibular dis- days was setup. orders and nutritional disorders or intoxications Method Phenotypic variability of GNE myopathy Data from fifteen individuals with Parkinson’s disease and six healthy persons were examined for the study. MyTremorApp was ’ N. Al Talai, P. Sarathchandran, A.B. Al Madani installed on each participant s personal smartphone and setup to Rashid Hospital- Dubai Health Authority, Neurology, Dubai, United Arab perform either 1, 2, or 3 days of testing. Each day was comprised of Emirates three scheduled tests; one in the morning, one around noon and one in the evening. Postural, kinetic and rest tremor, as well as bradykinesia during a rapid pronation-supination task were assessed GNE myopathy is a rare autosomal recessive disease with an during each test. Compliance was calculated by identifying the estimated prevalence of 1 in 1.000.000 worldwide. It is caused by number of performed tests over total scheduled tests. These were mutation in GNE gene that encode, UDP-N-acetylglucosamine 2- averaged for all participants in each group, to be compared with a t epimerase/N-acetylmannosamine kinase (GNE), a rate limiting test (p ≤ 0.05). enzyme in the biosynthesis of Salic Acid. GNE myopathy classically manifests during adulthood as distal Results muscle weakness predominantly affecting anterior tibial muscles Overall compliance was 95% for the healthy group and 96% for the that later on involves other muscle groups. Another encountered patient group. Compliance was close to 100% for the 1 & 2-day phenotype is limb-girdle spectrum. Quadriceps muscles sparing is a testing, but dropped to 90% for the 3-day testing. No statistical characteristic findings promoting the diagnosis of GNE myopathy. differences were found between compliance from the two groups. We present two Middle Eastern siblings with atypical clinical presentation of GNE myopathy. Both patients presented with Conclusion proximal muscle weakness involving quadriceps and significant Our study demonstrate that patients showed high compliance in myalgia that interfere with Activity of Daily Living. Serum CPK was performing the tests. In combination with the validity of the analysis elevated in both. Histopathological findings of muscle biopsy showed package, these preliminary results strongly suggest that necrotic fibers with fiber size variation. MyTremorApp can be used to monitor tremor and bradykinesia Genetic testing showed homozygous mutation on GNE gene longitudinally, offering an easy and free method to track the c.2228TNC (p.Met743Thr) on chromosome 9. The disease progression evolution of tremor and bradykinesia symptoms over time. was more rapid in the younger brother who also had clinically stable Fanconi aplastic anemia status post allogenic bone marrow transplantation from his brother who has GNE myopathy. Intravenous doi:10.1016/j.jns.2019.10.1346 immunoglobulin (IVIG) was started and showed modest effect mainly in the symptoms of weakness, pain and fatigue. In conclusion, we demonstrate that same genetic variants can present with variable phenotypes. Recognize a wide spectrum of GNE myopathy help early recognition of the disease hence allows WCN19-1474 providing the patients with trial of IVIG which might alleviate some of the patient symptoms and improved their quality of life. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1345 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Severity of motor symptoms among Parkinson’s disease patients WCN19-1473 and its association with other variables; A descriptive study

Poster shift 03 - Autonomic nervous system disorders/history of A. Keshavaraja, S. Kunathilagamb, N. Prathapana, J. Anpalagana a neurology/motor neuron disease/movement disorders/MS & Teaching Hospital of Jaffna, Neurology Department, Jaffna, Sri Lanka b demyelinating diseases/neuromuscular disorders/vestibular dis- Northern Central Hospital, Neurologiy Unit, Jaffna, Sri Lanka orders and nutritional disorders or intoxications Parkinson’s disease (PD) is a progressive neurodegenerative disorder characterized by cardinal motor features of bradykinesia, Using a smartphone application to monitor longitudinally tremor resting tremor and rigidity. This hospital-based study was carried out and bradykinesia in persons with movement disorders at two movement disorder clinics in Jaffna, Sri Lanka to describe the motor severity, duration, treatment response and association be- C. Duvala, J.F. Daneaultb, S. Bogardc tween these variables among PD patients (December 2015- May aUniversité du Québec à Montréal, Sceinces de l'activité physique, 2016). Demographic details and motor severity were assessed using Montréal, Canada an interviewer-administered questionnaire and Uniﬁed Parkinson’s bRutgers University, Kinesiology and Health, New Brunswick, USA Disease Rating Scale – Part III (UPDRS III) respectively. The sample cCentre de recherche Institut universitaire de gériatrie de Montréal, consisted of 144 PD patients (Male -52.1% and female -47.9%) with Gériatrie, Montréal, Canada the mean age of 65.42 (±9.12) years. The average age at onset was ARTICLE IN PRESS

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61.75 (±9.47) years; disease duration was 3.67 (±2.9) years. Conclusion Majority of the patients 56.5% (86) reported tremor as their first Our study suggests the possible role of MM genotype of PON1- symptom whereas 24.8% (36) reported hypokinesia. Of all, 87.6% 55M/L gene Polymorphism as genetic risk factor for Parkinson’s (127) had no history of falls. BMI was negatively correlated with disease when presented simultaneously with other risk factors of motor severity off treatment (p b 0.05). Motor severity (UPDRS III) Parkinson’s disease. Further studies are required before PON-55M/L off treatment ranged from 17 to 82 (Mean ± SD: 43.27 ± 14.78) gene Polymorphism can be considered as genetic risk factor for while on treatment (dopamine agonist and replacement therapy) it Parkinson’s disease. was 0–62 (Mean ± SD: 23.324 ± 12.83); the mean difference between on and off treatment was statistically significant (p b doi:10.1016/j.jns.2019.10.1348 0.05). Treatment response (reduction in motor severity score- UPDRS III) was negatively correlated with age of onset, but it was not statistically significant (p N 0.05). Gender, duration, age at onset, current age or BMI did not have statistically significant WCN19-1498 impact on treatment response. Therefore, it is important to identify the factors influencing treatment response to improve the outcomes. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1347 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Fatigue in multiple sclerosis: A Tunisian hospital cohort WCN19-1490 M. Ben Mahmoud, H. Derbali, A. Riahi, D. Azzouz, J. Zaouali, M. Poster shift 03 - Autonomic nervous system disorders/history of Mansour, R. Mrissa Military Hospital of Tunis, Department of Neurology, Tunis, Tunisia neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Introduction orders and nutritional disorders or intoxications Fatigue is a severe underestimated problem in multiple sclerosis (MS) that still not predicted by objective measures. PON1-55M/L (paraoxonase 1) gene polymorphism in Parkinson’s disease in North Indian population Objective Our study aimed to estimate the prevalence of fatigue in MS a a b R.K. Dhamija , B. Kumar , K. Saraswathy Tunisian population and evaluate its statistical links and impact on a Lady Hardinge Medical College and Associated Hospitals, Neurology, daily functioning. New Delhi, India b University of Delhi, Anthropology, New Delhi, India Methods We performed a cross-sectional population-based study of A number of genetic markers are well established in the causation patients with MS followed in the department of Neurology of of Parkinson's Disease such as alpha synuclein gene, Parkin gene, Military Hospital of Tunis from 2004 to 2019. All patient underwent LRRK2 gene etc. Recent studies also suggest role of newer genetic an interview that consisted on assessment of the level of disability factors like PON1 etc. in the causation of Parkinson’s disease. The (EDSS), the modified Ashworth score, the Hamilton Depression score PON 1L55M allele is correlated with decreased m-RNA and protein (HADS) and pain. Self-administered tests “Fatigue severity scale” levels and therefore carriers of PON1 L55M allele may have an (FSS), “EMIF SEP” and Epworth scale were given to patients. The rest inherited defect in detoxification of environmental toxins and this of data was collected from medical records. allele constitutes one possible candidate for PD susceptibility. Hence the role of PON1-55M/L gene Polymorphism in Parkinson’s disease Results was studied. We have included 60 MS patients. The prevalence of fatigue was 65%. Fatigue was the most disabling symptom for 39% of cases. Methods Fatigue was not statistically related to age, gender, MS duration, MS In the present study, the PON1-55M/L gene polymorphism was drugs, spasticity, daytime sleepiness or pain. Progressive MS form analyzed in 74 Parkinson’s disease patients and in 74 age and sex and higher EDSS scores were statistically correlated to the presence matched controls, by polymerase chain reaction amplification and of fatigue. Fatigue was statistically associated with temporal restriction digestion. Ethical clearance was obtained from Ethics demyelinating lesions and frontal cortical atrophy. The FSS was committee for Human Research, Lady Hardinge Medical College New moderately correlated to HADS and strongly correlated to EMIF-SEP. Delhi. Conclusion Result The high prevalence of fatigue in MS and its considerable The PON1-L55M gene Polymorphism was not found to be functional impact require special attention which should be of associated with Parkinson’s disease in this study. However the MM concern to all patients given the difficulty of identifying of group of genotype had odd ratio of 2.85 in patients as compared to controls, higher risk. after adjusting for all the confounders. Well water consumption and exposure to pesticides were significantly more among Patients of doi:10.1016/j.jns.2019.10.1349 Parkinson’s disease as compared to control group. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 289

WCN19-1503 WCN19-1515

Brain volume data in Brazilian patients with opticspinal and Harlequin syndrome: A case report in Uyo, southern Nigeria conventional multiple sclerosis B. Ekeha, D.O. Okekeb, E.M. Nyoyokoc R. Alvarengaa, A.C.B. Nascimentob, A.C.R.D.A. Araújoa, N.S. aUniversity of Uyo Teaching Hospital, Internal Medicine, Uyo, Nigeria Meneguetteb, M.P.A. Alvarengac, F. Carolino Pereirab, H. Alvarenga bUniversity of Uyo Teaching Hospital, Family Medicine, Uyo, Nigeria Filhod, C. Vasconcelosb, N.S. Meneguetteb, L.A. Brandâoe, P.P. Raffuf,C. cUniversity of Uyo Teaching Hospital, Family Medicine, Uyo, Nigeria D.M. Rimkusg a Universidade Federal do Estado do Rio de Janeiro- Hospital Federal da Background Lagoa, Neurology, Rio de Janeiro, Brazil Harlequin syndrome is a rare autonomic disorder characterized b Universidade Federal do Estado do Rio de Janeiro, Neurology, Rio de by ipsilateral loss of sweating or flushing of the face, neck and upper Janeiro, Brazil thoracic region in response to heat, exercise or emotional stress. It c Universidade Federal do Estado do Rio de Janeiro - UNESA, Neurology, may be primary or secondary and occurs at any age. Very few cases Rio de Janeiro, Brazil have been reported in Africa and this to the best of our knowledge is d Universidade Federal do Estado do Rio de Janeiro - UNESA, Physical the first case report in Nigeria. Education, Rio de Janeiro, Brazil e IRM, Radiology, Rio de Janeiro, Brazil Case report f UFRJ, Radiology, Rio de Janeiro, Brazil The patient is a 29 year old cinematographer who presented with g USP, Radiology, Rio de Janeiro, Brazil complaints of sweating only on the right side of the face, neck and trunk for four months. He first noticed that he was sweating on the Opticspinal Multiple Sclerosis (OSMS) is a MS phenotype right side of his face, neck and trunk while taking pictures and videos described in Asian population characterized nowdays by recurrent outside on an extremely hot day. Since then he had noticed this optic neuritis and transverse myelitis small spinal cord lesions and characteristic sweating. There was associated darkening of the right AQP4 – Ab negative. side of the body. There was no history of trauma to the neck, back or The objective of the study was to analyze and compare Brain MRI neck surgery. He had no associated gustatory sweating, erectile by MSmetrix reports in OSMS and Conventional MS patients (CMS) dysfunction, palpitations, syncopal attacks or nocturnal diarrhoea. from Rio de Janeiro where the majority of the population is Afro Past medical history was not contributory. descendent. Examination revealed sweating with associated hyperpigmenta- tion of the right side of the face with anhidrosis of the left side. Method A clinical diagnosis of Harlequin Syndrome was made. The 58 MS patients were scanned on HDxt GE 1,5 T. For each scanner routine investigations were all normal. Further investigations to a 3D T1 and 3D FLAIR were acquired and analysed blinded in exclude secondary causes were hampered by lack of funds. He is icometrix platform. Data on whole brain volume (WBV), gray matter regular on follow-up and has no further complaints. volume (GMV), total load volume 3DT1, 3DFLAIR image and white matter lesions segmentation were analyzed and compared blinded Conclusion between OSMS and RRMS groups. Rare diseases still occur and require a high index of suspicion and good clinical acumen to make a diagnosis. Results OSMS and CMS groups had similar demographic and long term doi:10.1016/j.jns.2019.10.1351 disability (Median EDSS b1.5). OSMS had a longer time of disease (19 versus 13 years) . As shown at Table 1, the total load volume 3DFLAIR and periventricular and deep white matter load lesions were significantly higher in CMS than OSMS. Periventricular WCN19-1516 lesions were identified in 100% of the participants but infratentorial lesions ocurred in 50% of OSMS and 27,9 of CMS and deep WM lesions in 100% of CMS and 30% of OSMS. Cerebral Poster shift 03 - Autonomic nervous system disorders/history of atrophy measured by the WBV and GMV not differed between the neurology/motor neuron disease/movement disorders/MS & groups, however, the 3DT1 volume was significantly higher in demyelinating diseases/neuromuscular disorders/vestibular dis- CMS. orders and nutritional disorders or intoxications

Conclusion Multiple sclerosis with adrenal insufﬁciency mimicking MSmetrix records showed different T2 and T1 patterns of Brain adrenoleucodystrophy lesions between OSMS and CMS. F. Kharrat, O. Hdiji, H. Hajkacem, N. Farhat, S. Sakka, M. Damak, M. doi:10.1016/j.jns.2019.10.1350 Chokri Habib Bourguiba-University Hospital, Neurology, Sfax, Tunisia ARTICLE IN PRESS

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Background Materials and methods The diagnosis of multiple sclerosis (MS) is based on clinical, 143 patients who were clinically diagnosed as Huntington disease radiological and biological findings. A misinterpretation of non from various tertiary neurology clinics were referred to our centre specific clinical and radiological findings may contribute to mis- for HTT-CAG screening and genetic investigations. CAG- 11-83TNR of diagnosing MS mimickers such as inherited leucoencephalopathies. the HTT and CTG 13-24 TNR of JPH3 gene were analyzed by fragment analysis from DNA samples of the patients. The work conforms to Case report approval from IGIB ethics Committee. Mrs NS, a 28 year-old woman was referred to our unit for vertigos and gait disturbances evolving since 15 days. Neurological assessment Results showed central vestibular syndrome and deep sensory alterations. In our analysis, 82 out of 143 (57%) patients were found to have Brain and spinal cord MRI showed non enhancing T2 and Flair expanded HTT-CAG-TNR in the range 39–83 repeats. The distribution hyperintensities in the parieto-occipital periventricular white matter, of normal alleles in HD patients varied from 11–33 CAG repeats cerebellar pedoncules and the cervical portion of the spinal cord. (mean, 20). None of our patient had shown JPH3-CTG expansion in Lumbar puncture showed oligoclonal bands in cerebrospinal fluid. pathogenic range. The observed allele distribution was, range; 13-24 The visual evoked potentials showed bilateral optic demyelinating CTG with bimodal distribution, CTG-20 (50%) and CTG-23 (36%). The neuropathy. Biochemistry revealed adrenal insufficiency. The diag- normal range of JPH3-CTG alleles were 19-25 in healthy controls (n nosis of adrenoleucodystrophy in a carrier woman was plausible all = 180 chromosomes). the more we noted high Lactate-to-pyruvate ratio. However the dosage of Very-Long-Chain-Fatty-Acids was normal. Hence, the Conclusion diagnosis of MS was made. The patient received five days pulsed- Overall, we have found HTT-CAG expansion mutation as a major methylprednisolone and experienced rapid improvement. determinant of HD phenotype in Indian patients. HDL-2 causing JPH3-CTG mutation was not observed in our group of HD patients. Discussion HDL-2 might be rare in Indian patient with HD-Like phenotype Many patients are misdiagnosed with multiple sclerosis, and might (small sample size in this study is a limitation to conclude exact be given disease modifying therapies while they suffer from metabolic occurrence of HDL2). fl or systemic disorders mimic^king in ammatory demyelinating diseases. There are many overlapping factors mainly: inflammation which is the doi:10.1016/j.jns.2019.10.1353 underlying mechanism of X-linked adrenoleucodystrophy (X-ALD), demyelinating optic neuritis, the presence of oligoclonal bands in CSF which were reported in X-ALD patients. WCN19-1525 Conclusion In our case we ruled out the diagnosis of X-ALD but this should be considered in patients with atypical MRI features or family history of Poster shift 03 - Autonomic nervous system disorders/history of adrenomyelopathy mainly in males. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- doi:10.1016/j.jns.2019.10.1352 orders and nutritional disorders or intoxications

Atypical course of Charcot-Marie-Tooth disease: what shall we be thinking of? WCN19-1523 F. Kharrat, O. Hdiji, N. Farhat, H. Hajkacem, S. Sakka, M. Damak, C. Poster shift 03 - Autonomic nervous system disorders/history of Mhiri Habib Bourguiba-University Hospital, Neurology, Sfax, Tunisia neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Introduction orders and nutritional disorders or intoxications Charcot-Marie-Tooth disease (CMT) is an inherited polyneuropathy with a gradually progressive course. Atypical features Screening of HTT and JPH3 trinucleotide repeat expansion in such as acute or subacute deterioration and unusual electrophysio- Indian patients with Hungtington disease phenotype logical ﬁndings should be alarming.

a b c d S. Parveen , V. Suroliya , A.K. Srivastava , M. Faruq Case report a Institute of Genomics and Integrative Biology, New Delhi, India A 54 year-old woman with medical history of hypothyroidism, b All India Institute of Medical Sciences, New Delhi, India was referred to our department for weakness of her 4 limbs with a c All India Institute of Medical Sciences, New Delhi, India subacute onset and rapid worsening. The family history was marked d Institute of Genomics and Integrative Biology, New Delhi, India by feet deformities. The pedigree was suggestive of autosomal dominant inheritance. On examination the proband had steppage Background gait, with distal muscle weakness in four limbs, absent tendon Huntington disease (HD) and Huntington Disease like -2 are an reflexes, hummer toes and hollow feet. Nerve conduction studies autosomal dominant progressive adult-onset neurodegenerative showed homogeneous demyelination but with temporal dispersion disorder. HD and HDL-2 are caused due to expansion of CAG in motor nerves. We noted slight increase in protein level in trinucleotide repeats (TNR N39) in the HTT (4p16.3) and CTG cerebrospinal fluid (CSF). The patient was diagnosed with autosomal expansion (N40) in JPH3 (16q24.2). The aim of the present study dominant CMT type I based on family history, neurological was to determine the occurrence of HD and HDL-2 among patient examination of the proband and her relatives, and electrophysiolog- manifesting Huntington disease phenotype. ical findings, associated with chronic inflammatory demyelinating ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 291 polyneuropathy. The patient was treated with high-dose steroids and subdivided in groups along the course, light predimentary states than moderately improved. were more characteristic of primary and secondary progressive currents than remitting currents. Discussion Further reported features suggesting a co-existent hereditary and doi:10.1016/j.jns.2019.10.1355 inflammatory process are: conduction blocks in motor nerve conduction studies, high protein levels in CSF, evidence of inflammatory process in nerve biopsy and nerve root enhancement in spinal imaging. WCN19-1540 Conclusion Atypical course and unusaul biological, electrical and imaging features in CMT disease should be alarming about the co-existence of Poster shift 03 - Autonomic nervous system disorders/history of inflammatory neuropathy. Immunomodulatory treatment may sub- neurology/motor neuron disease/movement disorders/MS & stancially improve the outcome. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1354 Increased expression of endoplasmic reticulum stress-related caspase-12 and CHOP in the hippocampus of EAE mice

WCN19-1539 M.H. Harirchiana, M. Kamareheib, S. Kabudanian Ardestaniac,M. Firouzib, H. Zahednasabc a Poster shift 03 - Autonomic nervous system disorders/history of Tehran University of Medical Sciences, Iranian Center for Neurological neurology/motor neuron disease/movement disorders/MS & Research, Neurology, Tehran, Iran bInstitute of Biochemistry and Biophysics, University of Tehran, Tehran, demyelinating diseases/neuromuscular disorders/vestibular dis- Iran orders and nutritional disorders or intoxications cInstitute of Biochemistry and Biophysics, University of Tehran, Tehran, Iran

The study of the of cognitive disfunctions using to the test The role of endoplasmic reticulum (ER) stress has been proposed in “ ” MoCa in patients with multiple sclerosis several neurodegenerative and autoimmune diseases and may contribute to neural apoptosis. The complex role of ER stress-mediated I. Ganieva, Y. Parpieva, K. Khalimova, M. Yakubova apoptosis introduces a novel angle on multiple sclerosis (MS) research. Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan Nevertheless, the mechanisms through which ER stress intermediates apoptosis are not entirely understood. To this aim, we examined the Relevance expression of C/EBP homologous protein (CHOP), caspase-12, and Cognitive impairments are hidden to clinicians, therefore not protein disulfide isomerase (PDI) in mice with experimental autoim- diagnosed and not included in rating scales, such as EDSS. In mune encephalomyelitis (EAE). We found the upregulated expression addition, impaired cognitive activity can have a significant impact of CHOP, caspase-12, and PDI in the brain of EAE mice in comparison to on the quality of life and the condition of patients, even in the healthy mice. Furthermore, elevated levels of caspase-12/CHOP in absence of marked focal disorders. astrocytes, oligodendrocytes, and microglia were found in the hippocampus section of EAE mice. This study highlighted the presence Material and methods of ER stress and increased activation of caspase-12 in the hippocampus We examined 78 patients with multiple sclerosis. Age varied from of mice in response to EAE induction. Higher levels of caspase-12/ 24 to 48 (34,3 ± 1,43). Of these, 55% (43) women, 45% (35) men. All CHOP in hippocampal oligodendrocytes as compared with microglia patients underwent MRI scans and using to the test MoCa to the and astrocytes denote that oligodendrocytes are particularly sensitive study of the cognitive disfunctions. to ER-mediated apoptosis. In conclusion, the regulation of ER stress pathway would be beneficial for the survival of oligodendrocyte. Results All Patients^were divided into groups depending on the clinical forms doi:10.1016/j.jns.2019.10.1356 and course, all patients were tested MoCaând the following data were points (p) obtained. Cerebral form (CF)-32%(25)-MoCa 18,6 ± 1,66, cerebrospinal form (CSF) -15,3% (12), MoCa 21,3 ± 1,3 , cerebellar form (CF) -21% (16) MoCa 23,2 ± 2,3, atypical form ( AF) -5% (4) 24,3 ± 1,7, WCN19-1541 radiological-isolated form (RIS) –15% (12) MoCa 25,3 ± 2,1, clinical isolated form (CIS) -12% (9) MoCa 22,3 ± 1,9 and primary progressive (PP)-5(6,4%) MoCa 23,3 ± 2,08, secondary progressive (SP) 12(15%) Poster shift 03 - Autonomic nervous system disorders/history of 24,3 ± 2,18, remitting course(RC) 40(51%) MoCa 25,2 ± 2,09. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Conclusion orders and nutritional disorders or intoxications 1. Impaired cognitive dysfunction by the definition of the MoCa test showed that pronounced cognitive impairment was not Congenital muscular dystrophies and congenital myopathies: A observed in more than one group. Mild dementia was observed in study of mutational pattern and phenotypic variability from a the group of cerebral and cerebrospinal forms in contrast to other tertiary care hospital in India groups that were closer to the norm (N26-30p) on a scale. 2. The ARTICLE IN PRESS

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K. Polavarapua, S. Nashib, H. Padmanabhab, V. Preethish-Kumara,S. DNM2 (P9,10): Onset:11-12 years with limb-girdle weakness. Vengalilb, N.P. Mahajanb, M. Keerthipriyab, C. Pradeep-Chandra Dominant(p.Glu368Lys, p.Val798Met) mutations. Reddyb, G. Arunachalc, S. Gunasekarand, M. Faruqe, A. Joshie,P. Thomas Treesaf, A. Nalinib Conclusion aNational Institute of Mental Health and Neuro Sciences NIMHANS, This study highlights the mutational pattern and challenging Neurology - Clinical Neurosciences, Bangalore, India conundrums of genotype-phenotype correlations in CMD and CM. bNational Institute of Mental Health and Neuro Sciences NIMHANS, Neurology, Bangalore, India doi:10.1016/j.jns.2019.10.1357 cNational Institute of Mental Health and Neuro Sciences NIMHANS, Human Genetics, Bangalore, India dNational Institute of Mental Health and Neuro Sciences NIMHANS, Neurology - Human Genetics, Bangalore, India WCN19-1545 eCSIR-Institute of Genomics and Integrative Biology, Functional Geno- mics, New Delhi, India fNational Institute of Mental Health and Neuro Sciences NIMHANS, Poster shift 03 - Autonomic nervous system disorders/history of Psychiatric Social Work, Bangalore, India neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Objective orders and nutritional disorders or intoxications To characterize the genetic subtypes of Congenital muscular dystrophies (CMD) and Congenital myopathies (CM). Difference of the role of high cortisol levels in the cognitive impairment Parkinson’s Disease and vascular Parkinsonism Methodology – fi A retrospective analysis of CMD and CM (2015 2019) con rmed D. Akramova, G. Rakhimbaeva by exome sequencing. Institutional Ethics Committee approval Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan obtained for the study. Objective Results We aimed to analyze the Parkinson's Disease (PD) related CMD group: 15 patients; M:F = 2:3. Onset was congenital in Ullrich hormonal factors and Vascular Parkinsonism(VP) hormonal factors CMD(UCMD), FKRP and LAMA2, adult in Bethlem myopathy(BM) in the cognitive impairment. and variable in LMNA. Common findings: Contractures, limb girdle and facial weakness, elevated serum creatine kinase. Calf hypertro- Patients and methods phy in LAMA2 and FKRP. We chose the cortisol to study the hormonal mechanisms to role of cognitive impairment, A total of 100 patients were participated in Collagenopathies. our investigation,46 patients of themare suffering from VP, whereas UCMD (P1,2): Recessive nonsense(p.Gln104ter, p.Gln27ter) mu- 53 patients are with PD. All patients were tested anamnestic, tations in Col6A2 and Col6A3. neurological and neuropsychological examination. We used the BM (P3-7): P3-5 had dominant missense mutations in Col6A2(p. MMSE and Hamilton, HADS scales methods; moreover we studied Gly564Arg), Col6A1(p.Gly269Ar) and Col12A1(p.Gly982Arg). Co6A3 cortisol levels and the concentration blood serum in the morning N nonsense(p.Arg59ter) and c.6309+3A G(5'splice) mutations in P6,7. using immunofluorescence methods. LAMA2 (P8,9): Compound heterozygous mutations(p.Lys1726ter| p.Arg2604ter; p.Arg2319ter|del 34-37ex): leukoencephalopathy and Results cerebellar cysts on imaging. According to the results of the investigation the concentration of LMNA (P10-14): Onset: infancy to 3rd decade. P10-13 had cortisol was doubled in VP 42%, 17.3% patients showed the increase heterozygous missense mutations(p.Arg453Trp, p.Leu102Pro, p. by 1,5 times, and the concentration of cortisol was not changed in Asn39His and p.Val440Met). Variable phenotypes: P10,11: EDMD, 38.6%. The patients with PD group, 11.6% showed the increase by P12: LGMD, P13: RSS with DCM. P14 with biallelic (p.Glu124Argfs*3| 1,5 times in cortisol concentration, the cortisol level doubled in p.Glu124Gln) mutations: EDMD with adult progeria features. 9.3% and was not changed in 79.5%. According to the results of FKRP (P15): Recessive(p.Pro448Leu) mutation: limb-girdle phenotype. neuropsychological tests and scales the higher levels of cortisol CM group: 12 patients; M:F = 2:1. Infantile onset with motor delay in concentration, the higher cognitive impairment was observed in RYR1, DES and SEPN1. Ptosis was common in all except RYR1. those patients RYR1 (P1-4): P1&2 had opthalmoparesis: compound heterozygous(p.Leu2963Pro| p.Ser783Leu; p.Glu4464Asp|p.Arg628His) muta- Conclusions tions. P1 biopsy showed centronuclear myopathy(CNM). P3 had The higher concentration of cortisol in blood serum, the higher heterozygous(p.Gly3288Arg) mutation. P4 with CM and bullous skin cognitive impairments in patients with VP. Cortisol levels always lesions: concomitant RYR1 frameshift(p.Leu108SerfsTer10) and play an important role in the pathogenesis of Cognitive impairments ’ N COL7A1 5 splice(c.8620+3G A) mutations. in patients with VP and PD. DES (P5,6): Recessive truncating(p.Arg150Ter, p.Glu320ArgfsTer2) mutations. Variable phenotypes: P5: CM with dilated cardiomyopathy doi:10.1016/j.jns.2019.10.1358 (DCM), P6: Congenital myasthenic syndrome(CMS). SEPN1 (P7,8): Rigid spine syndrome(RSS) with homozygous(p. Met525Arg, p.Ala195Thr) mutations. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 293

WCN19-1546 cUniversidade Federal do Estado do Rio de Janeiro-UNIG, Neurology, Rio de Janeiro, Brazil dUniversidade Federal do Estado do Rio de Janeiro, Hospital da Lagoa, Poster shift 03 - Autonomic nervous system disorders/history of Neurology, Rio de Janeiro, Brazil neurology/motor neuron disease/movement disorders/MS & eUniversidade Federal do Estado do Rio de Janeiro- UNESA, Neurology, demyelinating diseases/neuromuscular disorders/vestibular dis- Rio de Janeiro, Brazil orders and nutritional disorders or intoxications fUFMG, Neurology, Cuiabá, Brazil gUniversidade de Vassouras, Neurology, Vassouras, RJ, Brazil h Spinal dural fistula mimicking neuromyelitis optica spectrum Hospital Federal da Lagoa- UNESA, Neurology, Rio de Janeiro, Brazil disorders (NMODS) Objective J. Paivaa, L. Paulab, M. Almeidaa, A. Silvaa, V. Portilhoa The chronic form of Devic´s disease with relapsing remitting aClinical's Hospital of the Triangulo Mineiro, Neurology, Uberaba, Brazil course was only recognized in the 90´s . Neuromyelitis optica (NMO) bClincal's Hospital of the Triangulo Mineiro, Neurology, Uberaba, Brazil affect mainly Asian and African women. The aim of this study was to evaluate the long term prognosis and quality of life in NMO patients A 67-year-old, male, presented paraparesis with T12 sensory from Rio de Janeiro, where the majority of the population is Afro level, sphincteric alteration, and abolition of reflexes in limbs. The descendent. spinal cord MRI showed signs of extensive thoracic myelitis to the medullary cone, with normal cervical. MRI of the skull showed Method volumetric reduction of the left optic nerve leading to the suspicion In a cohort of 1444 patients followed in Hospital da Lagoa since NMODS. Oligoclonal bands and antiaquaporin-4 antibody (AQPA4) 1990 (Figure 1) we selected NMO by 2006 criteria and analyzed long were negative. Made pulsetherapy with parcial improvement, term disability, health quality of life by SF-36 and mortality. The fi followed by continuous azathioprine. After one year, worsened with classi cation of the NMOSD syndromes (2015) were applied. paraplegia and T6 sensitive level. MRI of thoracic spine showed intradural serpiginous images compatible with fistula. Made arteri- Results ography in which there was no finding of the nidus, following 122 Recurrent NMO patients, 89.3% women, 70.55 % black, mean – conservative treatment. This is a case of spinal dural fistula disease onset of 31.30 years (3 70 years), 18.9% pediatric forms b mimicking NMOSD which is characterized by severe attacks of optic ( 18 years) were analyzed. 827 acute events were registered [TM neuritis and extensive myelitis. Antibody AQP4 is one of the main (53%), ON (31%), [ON+TM] (9%), brainstem syndrome (4%), markers, but in some patients, it may be negative. The differential encephalopathy (3%), diencephalic syndrome (0.81%)] . Prevention diagnosis includes diseases that produce a similar picture as multiple treatment included azatioprin, micophenolato and rituximabe. At sclerosis and arteriovenous malformation. The medullary dural last assessment after the average of 12.7 years ± 8.98 (1-40), 39 % of fistula is rare, affecting mainly men, around 40 years old, with a the patients had paraplegia and bilateral amaurosis (Devic like- predilection in the inferior thoracic region. The suspicion occurs syndrome), 83.6% severe bilateral dysfunction and 59.8% severe through the presence, in MR, of serpiginous images due to venous motor dysfunction. The SF-36 scores were under 50 in all domains. fi hypertension. The arteriography, considered gold standard, can 46 patients died (38%) died, 9 unknown data. They were classi ed as identify anomalous communication of the vessels, but not always SDNMO (2015) AQP4-Ab positive (40,2%), negative (23,8%) or possible as in our case. Dural fistula causes sequelae by late diagnosis unknown (36,1%). as it may mimic other diseases that cause longitudinally extensive myelitis. Thus, in patients with negative AQP4, the etiology for spinal Conclusion arteriovenous malformation should be remembered. NMO represented 11% of all IIDD patients. Recurrent NMO I have obtained patient approval as necessary. patients developed severe disability, had a high reduction in quality of life and and high mortalily. doi:10.1016/j.jns.2019.10.1359 doi:10.1016/j.jns.2019.10.1360

WCN19-1551 WCN19-1559

R. Alvarengaa, V.C. Nerib, A.M.D.S. Catharinoc, A.R. Airãod,H. M. Bozhenko, N. Bozhenko, T. Nehrych Alvarenga Filhoe, H.H. Siqueiraf, A.B.C.D.G. Pereirag, A.R. Lucidia, M.P. Danylo Halytsky Lviv National Medical University, Neurology Depart- Alvarengah, C. Bentoa, C. Vasconcelosa ment, Lviv, Ukraine aUniversidade Federal do Estado do Rio de Janeiro, Neurology, Rio de Janeiro, Brazil Background and aims bHospital Federal da Lagoa, Universidade de Campos, Neurology, Rio de Patients with MS have a 20-fold increased risk of trigeminal Janeiro, Brazil neuralgia. The frequency of trigeminal neuralgia (TN) in multiple ARTICLE IN PRESS

294 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx sclerosis(MS) patients is about 1.9–6.3%. Last researches showed that Results MS patients have a dual mechanism of TN, which consist of MS A 29-year-old male, born of consanguineous parents presented plaque, and a neurovascular contact. with fatigable ptosis and ophthalmoparesis since birth. Progressively he developed voice and limb muscle fatigue with diurnal variation. Methods He has frequent buckling falls, proximo-distal weakness and unable Analysis of disease history and review of patients with multiple to stand on toes for 2 years. On examination he has bilateral ptosis, sclerosis. opthalmoplegia, mild facial weakness, calf wasting and elbow contractures. Grade 3 power in proximal muscles with selective Results ankle and finger flexion weakness. His elder brother, a 32y-old, During 2017-2019, there were 6 patients with MS and TN in our presented with progressive proximal lower limb weakness, difficulty neurological department. The average age of patients was - 37,8 ± in standing on toes and exertional myalgias for 4 years without 9,2 years and the average duration of disease was 6,7 ± 2,7. 5 ocular symptoms or fatigability. On examination he had calf wasting patients were under the age of 40, and one - 55 y.o. In the case of sex, with selective ankle flexion weakness and grade 4 power in proximal patients were divided equally. The diagnosis of TN was first muscles without cranial muscle involvement. established in three patients. For one - TN was the first manifestation Serum Creatine Kinase levels were 1720 and 4709IU/L. Repetitive of the disease. Second and third branches where damaged in 83,3%, nerve stimulation in proband showed significant decrement in all three branches - in 16,7%. Neuro-vascular conflict according to deltoid(N30%), nasalis and orbicularis oculi (N10%), elder sibling MRI was in 50%, pontine plaque was found in 83,3% patients. TN was had decrement in trapezius and quadriceps (N20%). Muscle biopsy accompanied by sensory disorders (paresthesia, numbness) and showed absent Dysferlin staining in both. abnormal Trigeminal reflex in 83,3 % patients. Mutation analysis done after obtaining consent showed concomitant homozygous DYSF (c.1116CNA) and CHRNE founder Conclusions (c.1327delG) mutation in younger sibling. Elder sibling had identical In people under the age of 40 who do not have MS, TN occurs DYSF mutation but lacked CHRNE mutation. Parents showed rarely, so it is especially important to determine whether this heterozygosity for both mutations. symptom is related to MS. Unlike classical neuralgia, in which there is no sensory impairment, TN in MS is accompanied by sensory Conclusion disorders. Neuralgia of the trigeminal nerve in MS is associated with To our knowledge this is the first report of DYSF and CHRNE plaques localized in the pontine trigeminal root entry zone. mutations in same patient which can occur due to common/founder mutations in communities where endogamy or consanguinity is high. doi:10.1016/j.jns.2019.10.1361 doi:10.1016/j.jns.2019.10.1362

WCN19-1561 WCN19-1568 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Poster shift 03 - Autonomic nervous system disorders/history of demyelinating diseases/neuromuscular disorders/vestibular dis- neurology/motor neuron disease/movement disorders/MS & orders and nutritional disorders or intoxications demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications DYSF and CHRNE ‘double-trouble’ mutations: A rare combination of limb girdle muscular dystrophy with congenital myasthenic Secondary parkinsonian syndromes syndrome in a South-Indian family B. Nadiaa, N. Farhatb, S. Sakkab, O. Hdijib, M. Dammakb, C. Mhirib,T. K. Polavarapua, S. Vengalilb, V. Preethish-Kumara, G. Arunachalc, N.P. Dhakera Mahajanb, S. Nashib, M. Keerthipriyab, C. Pradeep-Chandra-Reddyb,N. aUniversity Habib Bourguiba Hospital, Neurology, Sfax, Tunisia Gayathrid, A. Nalinib bHabib Bourguiba University Hospital, Neurology, Sfax, Tunisia aNational Institute of Mental Health and Neuro Sciences NIMHANS, Neurology, Clinical Neurosciences, Bangalore, India Background b National Institute of Mental Health and Neuro Sciences NIMHANS, Secondary Parkinsonism (SPD) is a differential diagnosis of Neurology, Bangalore, India idiopathic Parkinson's disease (PD).SPD is caused by an identiﬁed c National Institute of Mental Health and Neuro Sciences NIMHANS, structural, toxic, or metabolic mechanism. The pathophysiology is Human genetics, Bangalore, India poorly understood, different mechanisms, seem to produce parkin- d National Institute of Mental Health and Neuro Sciences NIMHANS, sonian symptoms by interfering with normal dopaminergic produc- Neurolopathology, Bangalore, India tion or transmission.

Background Objective Double-trouble mutations are described in several diseases. The purpose of our study is to clarify the clinical, etiological speciﬁcities and prognosis of SPD. Objective To report on co-existent mutations in DYSF and CHRNE genes Methods resulting in a mixed phenotype. We conducted a retrospective study of 75 cases of patients with a diagnosis of parkinsonian syndrome. We collected demographics data ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 295 and ﬁndings of neurological examination. All patients underwent Conclusion Brain imaging. Other exams were asked according to the context. Neuropathy is one of the major causes of disability and dependence. Diabetic PNP is the most common Polyneuropathy, Results further in this group distal symmetrical PNP is most common. We recruited 75 patients, sex ratio equal to 1.3. The average age Neuropathic pain is the most common and disabling feature of was 72.75 years [21-80].The reasons for consultation were: slowness neuropathies. GBS, CIDP and inherited PNP are the major etiologies in the execution of the gestures in 32, 35%, tremor in 27, 94%. of functional dependence. Neurological examination showed:asymmetrical Parkinson's syndrome in 58.82% and predominant to the lower limbs in 26.47%. doi:10.1016/j.jns.2019.10.1364 Cerebellar and pyramidal syndromes were found respectively in 3 and 11 cases. Cognitive disorders found in 22.05%. Cerebral MRI showed leukoaraiosis in 19 patients, and ischemic lacuna in the basal ganglia in 11/75 patients.Thirty-three patients had SPD. The causes WCN19-1580 were vascular in 33.33%, drug toxic in 3 patients and Wilson's disease in 5 patients. The evolution was favorable in patients of toxic origin after therapeutic weaning. For Patients who presented wilson’s Poster shift 03 - Autonomic nervous system disorders/history of disease, evolution was favorable in all patients after being put under neurology/motor neuron disease/movement disorders/MS & Artamin. For patient with vascular etiologies, evolution was demyelinating diseases/neuromuscular disorders/vestibular dis- progressive. orders and nutritional disorders or intoxications

Conclusion Management of depression comorbid with familial spastic Numerous causes may induce parkinsonism, we must look for red paralysis (case report)? ﬂags which should lead to exclude PD. A. Wafaa, E.B. Maryema, E. Ellouzeb, D. Farhata, C. Bessema, M. Abbesa, doi:10.1016/j.jns.2019.10.1363 G. Latifaa aRegional Hospital of Gabes, Psychiatric department, Tunisia, Tunisia bRegional Hospital of Gabes, Department of neurology, Tunisia, Tunisia

WCN19-1570 Introduction Familial spastic paralysis is a rare degenerative progressive Poster shift 03 - Autonomic nervous system disorders/history of disorder involving mainly the pyramidal tracts in the spinal cord. It neurology/motor neuron disease/movement disorders/MS & is characterized clinically by weakness and rigidity of the lower limbs. Depression is a fairly common psychiatric comorbidity. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Aims Our purpose is to discuss, through an illustrative clinical case and Neuropathy; How etiological factors impact on variability of clinical literature data, the choice of antidepressant in patients with familial and demographic parameters of mono and polyneuropathy? spastic paralysis.

a a b a a Y. Malik , U. Jamil , A. Alhawai , H. Alzarooni , A. Almadani Case report a Rashid Hospital, Neurology, Dubai, United Arab Emirates We report the case of Mrs. G aged 25, followed in neurology since b Rashid Hospital, Internal Medicine, Dubai, United Arab Emirates 2 years for family spastic paralysis. She was addressed to the department of psychiatry for a depressed mood. The psychiatric Neuropathy effects almost 8% of the world population and holds interview noted a depressive syndrome. The diagnosis of depression significant role in global burden of disease. Various etiologies have was retained and the patient was put on paroxétine in progressive been identified and these represent a considerable variability in doses. We have observed a good neurological tolerance. clinical parameters. Discussion Materials and methods Clinical experience has shown that the use of tricyclic antidepres- We collected the data of all neuropathy patients from Rashid sants is limited by their frequent anticholinergic effects of urinary hospital, Dubai from 2000-2017 as a retrospective record review retention and dry mouth. According to the studies, it would appear aiming to see variability of clinical, etiological and demographic that the antidepressants of choice would be the selective serotonin parameters in our population. reuptake inhibitors (SSRIs). They have fewer anticholinergic side effects and are generally as effective as tricyclics. But some clinical Results studies have shown that SSRIs can exacerbate spasticity. Nevertheless, Total of 1000 patients were collected with female predominance a gradual increase in dosage limits initial neurological adverse effects. (53%). Amongst age groups elderly age predominated(63%). First If spasticity does increase, discontinuing use of the antidepressant may symptom of disease was pain = 46%, numbness = 41% and weak- be preferable to adding antispasticity medication. ness=8% patients. Data indicated presence of mono-neuropathy in 60% and polyneuropathy(PNP) in 40% patients. Most common etiology of Conclusion polyneuropathy was diabetes whereas most common mononeuropathy The management of depression in patients with spastic paralysis was carpal tunnel syndrome(CTS) which was also caused majorly by represents for the clinician a neuropharmacologic challenge of DM. Clinical features like first symptom was numbness/pain in diabetic balancing antidepressant activity with the effects of agents for PNP and CTS but weakness appeared first in GBS/CIDP. Age of onset was spasticity. younger in GBS and CTS, whereas it was middle to old in radiculopathy, CIDP and diabetic PNP. Critical course of disease and functional disability doi:10.1016/j.jns.2019.10.1365 was more related to CIDP, GBS and inherited PNP. ARTICLE IN PRESS

296 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-1585 Background Fatigue is one of the most common and troublesome symptoms in multiple sclerosis (MS). It is still undervalued because it can’tbe Poster shift 03 - Autonomic nervous system disorders/history of predicted by objective measures. Several factors intervene in its neurology/motor neuron disease/movement disorders/MS & genesis including sleep disturbances (SD) and urinary disorders demyelinating diseases/neuromuscular disorders/vestibular dis- (UD). orders and nutritional disorders or intoxications Methods Role of increased levels of endothelial factors in cognitive We conducted a cross-sectional study including patients with MS, impairment in the Parkinson’s disease and vascular parkinsonism over a 14-year period. We evaluated the severity of fatigue using the "Fatigue Severity Scale" (FSS). Significant fatigue is retained from an D. Akramova, G. Rakhimbaeva FSS score greater than or equal to 4. The Epworth Sleepiness Scale Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan (ESS) was used to detect sleep debt. A score between 9 and 14 indicates a significant sleep deficit. Patients were also questioned about the presence of UD. Relationship between fatigue, UD and SD Objective was studied. We analyze the role of endothelial factors in the pathogenesis cognitive impairment PD and VP. Results Sixty patients participated in our study. The mean age was 37,38. Patients and methods The sex ratio was 0,32. The average age at onset of MS was 29,78 We chose to study the cognitive impairment and Alpha- years. The average FSS score was 4,67. The prevalence of fatigue was fetoprotein concentration to analyze the endothelial factors. A total 65%. Only 12% of patients with fatigue had a significant sleep deficit. of 100 patients were participated in our investigation, 56 patients of The frequency of UD in the tired group was as follows: urgency the mare suffering from VP, whereas 44 patients are with PD. All 28,2%, nycturia 25,64%, incontinence 20,5%, dysuria 7,7%, and urinary patients were tested anamnestic, neurological and neuropsycholog- retention 2,56%. The only significant correlation was noted between ical examination. We used the Mini‐Mental State Examination the presence of urinary incontinence and fatigue. (MMSE) and other cognitive impairment test methods; the Cam- bridge Cognitive Examination (CAMCOG), and the Mental Alterna- Conclusion tion Test moreover we studied of the concentration of Alpha- Our results don’t agree with those reported in the literature. In fetoproteinin blood serum in the morning using immunofluores- fact, our study was cross-sectional and assessed the presence of cence methods. these fleeting symptoms at a precise time of the course of the disease while the other studies covered longer periods of follow-up. Results The Alpha-fetoprotein levels were higher in 58.3% patients with doi:10.1016/j.jns.2019.10.1367 VP, but in 42.7% it was not determined. The Alpha-fetoprotein levels were higher in 29.51% patients with PD.

Conclusions To sum up the mechanisms of VP and PD are different from each WCN19-1593 other’s, which seem to be the result of endothelial changes. Endothelial factors always play an important role in the pathogenesis Poster shift 03 - Autonomic nervous system disorders/history of of Cognitive impairments in patients with VP and PD. The neurology/motor neuron disease/movement disorders/MS & concentration of Alpha-fetoprotein is higher in blood serum in demyelinating diseases/neuromuscular disorders/vestibular dis- patients with PD. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1366 Sub-analysis of real nutrition of patients with jaw fractures

A. Xolmurodova, D. Bobamuratovab a WCN19-1592 Specialized Center of Thoracic Surgery, Congenital Heart Disease, Tashkent, Uzbekistan bTashkent Medical Academy, Maxillofacial Surgery, Tashkent, Poster shift 03 - Autonomic nervous system disorders/history of Uzbekistan neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Objective orders and nutritional disorders or intoxications To evaluate the home real nutrition of patients with the mandible fractures during the treatment. Fatigue in multiple sclerosis: The involvement of sleep disturbances and urinary disorders Materials and methods Was investigate 57 men aged 18–59 years with a fractured ТМА K. Laabidi, H. Derbali, A. Riahi, M. Messelmani, M. Mansour, J. Zaouali, mandible in II clinics of . All patients given appropriate surgery – R. Mrissa and drug treatment. Material collection was carried out 13 20 days Military Hospital of Tunisia, Neurology, Tunis, Tunisia after splinting, through interviews and questionnaires. Results: Intermaxillar ﬁxation blocks chewing process, as they are forced to ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 297 take liquid and semiliquid food. As a result of the survey it was found motor action potentials (CMAP) were decreased, the motor that calorific value of rations patients was within minimal 1080 kcal/ velocities were normal and the distal motor latencies were day, maximal energetic value was 3300 kcal/day(p N 0.01) and an extended (except at the left tibial nerve). Amplitudes of sensory average of 2030 ± 35 kcal / day, had no statistically significant nerve action potentials (SNAPs) and sensory velocity were normal. differences (p N 0.05). The energy value of the daily diet of most F-wave latencies were shortened at the ulnar, peroneal and tibial to respondents (77%) was lower than the average daily energy the right, peroneal and tibial to the left. On needle electromyog- expenditure even of a healthy person. 8%of patients consumed diet raphy, the spontaneous activities were: fibrillations, positive sharp greater than 3000 Kcal/day. The diet was poor patients to proteins, waves and fasciculations. At effort, the pattern was neurogenic in especially animals. The protein intake was on average 63,8 ± 1,3 (p all patients. N 0.05) was the diet of women, 74,4 ± 1,8 of men. Such a diet is reflects at outcome major diseases. Conclusion The ENMG is essential for the diagnosis of ALS. Electro- Conclusions neuromyographic characteristics found in our patients have been The diet of patients with jaw fractures has insufficient nutritional reported in the literature. value, do not correspond on hygiene standards, also low biological inferiority. It is the need to correct the diet of patients to recommend doi:10.1016/j.jns.2019.10.1369 the inclusion in foods rich in complete protein, unsaturated fats, enriched with vitamins, elements. It is necessary to conduct studies of patients to proper nutrition. WCN19-1624 doi:10.1016/j.jns.2019.10.1368

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-1602 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Poster shift 03 - Autonomic nervous system disorders/history of Neuromyelitis optica spectrum disorders (NMO-SD) in a Sub- neurology/motor neuron disease/movement disorders/MS & Saharan Africa Country: A preliminary study of sixteen senegalese demyelinating diseases/neuromuscular disorders/vestibular dis- cases orders and nutritional disorders or intoxications N.M. Gaye, M. Fall, A.M. Diop, M. Ndiaye, A.G. Diop Electroneuromyographic features of amyotrophic lateral sclerosis Fann Teaching Hospital, Neurology, Dakar, Senegal in Senegalese patients: A preliminary study Background a a b D. Gams Massi , M. Njankouo Yacouba , D. Marième Soda ,D. Neuromyelitis optica (NMO) is an autoimmune disease of the b b b b Massar , S. Lala Bouna , N. Moustapha , D. Amadou Gallo central nervous system. In Sub-Saharan Africa, publications are rare a Douala General Hospital, University of Douala, Internal Medicine, and deal with isolated cases. Our goal was to analyze the Douala, Cameroon characteristics of NMO spectrum disorders in a senegalese cohort b Fann National Teaching Hospital, Cheikh Anta Diop University, compiled in Dakar. Neurology, Dakar, Senegal Patients and method Background This was a retrospective descriptive study conducted at the Amyotrophic lateral sclerosis (ALS), also known as Charcot or Lou Neurology department of Fann Teaching Hospital. We included all Gehrig's disease, is a fatal neurodegenerative disease characterized patients with NMO-SD according to the 2014 diagnostic criteria. by the loss of upper and lower motor neurons. Results Methods Sixteen patients were enrolled, 4 men and 12 women with an We conducted a descriptive retrospective study from July 2016 to average age of 30 years. Ten patients (62.5%) presented an acute June 2017. The objective of this study was to determine the myelopathy associated with retrobulbar optic neuritis and 5 electroneuromyographic features in ALS patients. 11 patients were (31.25%) had isolated spinal cord injury. Spinal MRI showed collected in the neurophysiology unit Michel NDIAYE of the abnormal cervical (6 patients), dorsal (4 patients), bulbar-cervical Neuroscience Clinic Ibrahima Pierre NDIAYE of Dakar in Senegal. (3 patients) or cervico-dorsal (2 patients) signal extended (³3 vertebral segments) of the spinal cord. Visual evoked potentials Results (VEP) showed demyelinating optic nerve involvement in 8 patients. The mean age of the cases was 40.8 ± 16.4 years. The male Ten patients were positive to AQP-4 IgG. Systemic corticosteroid gender was in the majority (sex ratio 2.7). The mean age of onset of therapy was the rule in all patients, associated with azathioprine in symptoms was 38.8 ± 16.4 years. 27.3% of the patients had a 10 of them. The clinical course at 3 months was predominantly juvenile form. The mean values of the amplitudes of the compound favourable (10 patients). ARTICLE IN PRESS

298 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Conclusion WCN19-1639 This cohort is the ﬁrst one compiled in Dakar. African multicentric epidemiological studies are needed. Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1370 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-1626 Chorea-acanthocytosis: A Tunisian family

D. Turkia, S. Sakkaa, O. Hdijib, N. Farhata, M. Dammaka, C. Mhiria Poster shift 03 - Autonomic nervous system disorders/history of aCHU Habib Bourguiba, Neurology, Sfax, Tunisia neurology/motor neuron disease/movement disorders/MS & bCHU Habib Bourguiba, Neuroogy, Sfax, Tunisia demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Background The neuroacanthocytosis (NA) syndromes comprise a phenotyp- Reduction of multiple sclerosis burden with specialised multiple ically and genetically heterogeneous group of progressive neurode- sclerosis clinic generative disorders involving the central nervous system with peripheral blood acanthocytes (red blood cell that has a spiked cell J. Eliwaa, F. Husainb, A. Albeloushic, S.F. Ahmedd, J. Al-hasheld,R. membrane). Behbehanie, R. Alroughanif Chorea-acanthocytosis (CA) is a NA syndrome with neurodegen- aMinistry of Health, Alsabah Hospital, Kuwait City, Kuwait eration of the basal ganglia caused by mutations in the VPS13A gene bMinistry of Health, Farwaniya Hospital, Kuwait City, Kuwait that codes for the production of chorein. cMinistry of Health, Mubarak Alkabeer Hospital, Kuwait City, Kuwait dIbn Sina Hospital, Neurology, Kuwait City, Kuwait Patients and methods eAl-Bahr Hospital, Opthalmology, Kuwait City, Kuwait We report here the cases of three patients of the same family fAmiri Hospital, Neurology, Kuwait City, Kuwait from two different generations, They all presented psychiatric symptoms, chorea, orobuccal dystonia, axonal neuropathy, with Introduction elevated CPK. Acanthocytes were identified in the peripheral blood Multiple sclerosis (MS) specialists prescribe the correct disease and the diagnosis of chorea-acanthocytosis was made in all the modifying therapy (DMT), potentially reducing the number of MS patients. MRI was done in two patients showing caudal nucleus relapses, and hence, saving healthcare costs and disability burden. atrophy in one case and striatal hyperintensity in the other Our study aims to identify the impact of the establishment of a specialised MS clinic on the rate of relapses, Expanded Disability Results Status Scale (EDSS), and escalation therapy. Our three patients were treated with haloperidol with good outcome for the arms movements. The psychiatric symptoms Methods remained in all patients, and one patient died by suicide. The oro- This cross-sectional study quantified the rate of admission due to facial dystonia remained in one patient. relapses three years prior and three years after establishing Ibn Sina Hospital's from admission logbooks. Each patient’s sociodemographic Conclusion data, EDSS status, and treatment plan, was attained from the The CA is a rare uncommon disorder, seizures and psychiatric hospital's online filing system. symptoms are the first symptoms to appear and oro-lungual dyskinesia is the most pathognomic symptom and the hardest to Results control. Botulinum toxin injection and surgical management can also A total of 1,246 RMMS patients, mostly females (69.6%), were be proposed. The early diagnosis for an early medication and involved in the study with a mean age at diagnosis 26.54 ± 8.58. psychiatric care is necessary for a better outcome. The mean admission rate due to relapse was significantly reduced from 24.54 ± 7.48 to 2.92 ± 4.26, P b 0.004 after establishing the doi:10.1016/j.jns.2019.10.1372 MS clinic. Most patients (61%) had escalated therapy (mean duration till escalation: 7.4 years). MS specialists prescribed second line DMT for 600 patients (50.1%). Escalation therapy was significantly higher in patients who were seen by an MS specialist WCN19-1645 versus those who were seen by a general neurologist (66.6% vs 33.4%, Pb0.0. Mean EDSS at latest visit to MS clinic was less than 3 in 1039 (83.3%), from 3-4 in 59 (4.7%) and more than 4 in in 148 Poster shift 03 - Autonomic nervous system disorders/history of (12.7%). neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Conclusion orders and nutritional disorders or intoxications Optimal therapy decisions for MS patients via a specialised MS clinic are associated with a significant decrease in relapse rate, Othello syndrome in Parkinson's disease patients: About 4 cases disability, and hence, the economic burden of multiple sclerosis. S. Sabiry, Z. Abdulhakeem, M.A. Rafai, B. El Moutawakil, H. El Otmani doi:10.1016/j.jns.2019.10.1371 Ibn Rochd University Hospital, Neurology-Neurophysiology, Casablanca, Morocco ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 299

Introduction Discussion Othello syndrome (OS), is a type of paranoid delusional jealousy, OS is an uncommon neuropsychiatric disorder seen in Parkinson's characterized by the false absolute certainty of partner inﬁdelity, patients, associating persistent paranoid and sexual delusions. rarely described in patients with Parkinson's disease (PD) using These results suggest that DA usage is a risk factor of OS in dopamine agonists (DA). patients with PD, regardless of dementia’s presence or not.

Objectives Conclusion The aim of our study was to analyse the clinical features and the OS is more common in patients with early disease onset. It should underlying cause behind OS occurrence, being an unusual psychiatric systematically be scanned for, at each consultation, to avoid dramatic disorder with an unknown neuropsychological proﬁle. marital distress and conﬂict.

Materials and methods doi:10.1016/j.jns.2019.10.1373 We identiﬁed 4 patients suffering from OS. Their records were retrospectively analysed and some clinical data were included: sex, age at PD onset, age at OS onset, PD timeline, types and duration of DA consumption WCN19-1655

Results All patients were males. Mean age at OS onset was 50.25 years Poster shift 03 - Autonomic nervous system disorders/history of (39–62). Time from PD until OS onset was of 4 to 9 years. All neurology/motor neuron disease/movement disorders/MS & patients were using dopaminergic agonists, 3 of whom were on demyelinating diseases/neuromuscular disorders/vestibular dis- Levodopa/Piribedil combination and one patient on Pramipexole orders and nutritional disorders or intoxications alone. Two patients exhibited impulse control disorders and the neurological examination in 2 cases revealed a cognitive Cortical neural connectivity analysis on olfactory spatial atten- impairment. Two patients suffered from divorce as a consequence tion: A prime ﬁnding on progressive state of Parkinson’s disease of OS. Patients’ follow up showed total disappearance of OS in one patient after DA tapering and a partial improvement in another H. Chatterjeea, G. Elumalaib, P. Maitic, N. Osakwea, N. Sewrama, G. case. Vinodhanada ARTICLE IN PRESS

300 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx aTeam NeurON, Texila American University, College of Medicine, Georgetown, Guyana bTeam NeurON, Texila American University, Neuroscience, Georgetown, Guyana cSaginaw Valley State University, Neuroscience-Brain Research Labora- tory, Michigan, USA

To study any inconsistency in the neural connections responsible for facilitating spatial recognition of olfactory stimuli as observed in people diagnosed with Parkinson’s Disease (PD) compared to those not affected by PD, and during the progression of the same. Olfactory dysfunctions are observed in approximately 90% people affected by PD. Like in most neurodegenerative disorders, olfactory dysfunction is a clinical marker appearing years before declining motor and cognitive functions in PD. The temporal-parietal junction (TPJ) contributes to spatial recognition, and lesions in this region or disruption of its neural communication has been associated with lack of spatial attention to various stimuli including visual, auditory, and less popularly olfactory. This study applies fiber tractography using Diffusion Tensor Imaging. The neural connection between the olfactory cortex and TPJ have been studied in MRIs from four different groups of people including a control group, and three groups of people diagnosed with PD from widely spaced stages of progression of the disease. Upholding the hypothesis, a significant difference in the number of fibres have been seen between the control and PD affected groups, and further decline has been observed in the progressive stages of PD (Fig. 1-4). This suggests that monitoring the neural connectivity between the olfactory cortex and TPJ or monitoring the neural connections facilitating one’s spatial attention to olfactory stimuli can provide for a way to monitor progression of PD or a mode of staging on the basis of olfactory dysfunction progression. doi:10.1016/j.jns.2019.10.1374

studies that describe the presence of neuroglobin in the nigrostriatal pathway and motor cortex in Parkinson’s disease. WCN19-1677 Objective Identify the presence of Neuroglobin in: prefrontal cortex, basal Poster shift 03 - Autonomic nervous system disorders/history of ganglia and substantia nigra in an experimental murine model. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Material and methods orders and nutritional disorders or intoxications For the induction of Parkinson's disease it was used the Dunnett & Iversen method, 1980. 30 male Wistar rats of 250 g underwent Presence of neuroglobin in cerebral areas of a murine model of stereotactic surgery, treated group n = 15 received an injection of 6- Parkinson's disease hydroxydopamine in substantia nigra pars compacta (Anteroposterior, 3,9; Lateral, 1,8; Ventral, 6,7 mm) and the control group n = 15 received M.G. Enríquez Mejíaa, E. Segura Anayaa, A. Martínez Gómezb,D. a vehicle injection. 14 days later, intracardiac perfusion was performed. Millán Aldacoc, M. Palomero Riveroc, D. Hinojosa Bobadillaa, P.Ó. Then coronal brain sections of 40μ were made according to Paxinos and González Vargasd, P. Vieyra Reyesa Watson atlas. The sections were subjected to the immunohistochemical aFacultad de Medicina, Universidad Autónoma del Estado de México, process using the anti-neuroglobin antibody (Sigma N7162). Neuroﬁsiología de la Conducta, Toluca, Mexico bFacultad de Medicina, Universidad Autónoma del Estado de México, Results and conclusion Biología Molecular, Toluca, Mexico The treated group showed greater presence of neuroglobin in the cInstituto de Fisiología Celular, Universidad Nacional Autónoma de Méx- three areas of interest, this ﬁnding may correspond to the following: ico, Biología Celular, Ciudad de México, Mexico A) response to the damage induced by the loss of dopaminergic dHospital Perinatal Monica Pretelini, Neurología, Toluca, Mexico neurons, not only in the substantia nigra pars compacta, but in areas corresponding to it’s main projections; striated nucleus and motor Background cortex. B) Upregulation not only due to hypoxia, as previous studies Neuroglobin (Ngb) is a protein expressed in nervous system reveal, but there may be several regulatory mechanisms that involve participating in oxygen transport, apoptosis and neuroprotection; pathways related to the pathogenesis of Parkinson's disease. processes involved in neurodegenerative pathologies like Parkinson's doi:10.1016/j.jns.2019.10.1375 disease. However, despite the potential of neuroglobin, there are no ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 301

WCN19-1678 compared to their corresponding data after six months, an increase in the grouping coefﬁcient, modularity, assortativity and strength for different densities was observed. However, these differences were Poster shift 03 - Autonomic nervous system disorders/history of not statistically signiﬁcant. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Conclusions orders and nutritional disorders or intoxications The results are comparable with studies between people with obesity vs. normal weight. The values from the NN show that after Metric alterations of neural networks in patients with morbid gastric bypass, connectivity tends to be similar to normal weight obesity undergoing gastric bypass subjects networks.

doi:10.1016/j.jns.2019.10.1376 A. Delgadillo Ramíreza, P. Flores Ocampoa, L. Pacheco Blasb,N. González Garcíab, D. Hinojosa Bobadillaa, P.Ó. González Vargasc,P. Vieyra Reyesa aFacultad de Medicina, Universidad Autónoma del Estado de México, Neurofisiología de la Conducta, Toluca, Mexico WCN19-1682 bHospital Infantil de México Federico Gómez, Neurociencias, Ciudad de México, Mexico Poster shift 03 - Autonomic nervous system disorders/history of cHospital Perinatal Monica Pretelini, Neurología, Toluca, Mexico neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Background Recent studies have shown that obesity leads to alterations in orders and nutritional disorders or intoxications Neural Networks (NN). Gastric bypass can provide long-term weight loss, however, it´s unknown if it promotes normal NN. Subthalamic deep brain stimulation can improve constipation and other bowel dysfunction in Parkinson’s disease Objective Determine metric alterations of NN in patients with morbid T. Uhciyamaa, T. Yamamotob, Y. Higuchic, K. Suzukid, T. Kadowakid,T. obesity undergoing gastric bypass. Shingoe, S. Kuwabarab, K. Hiratad, H. Muraif aSchool of Medicine, International University of Health and Welfare, Patients/material and methods International University of Health and Welfare Ichikawa Hospital, Functional magnetic resonance scans were performed in resting Neurology, Chiba, Japan state (rs-FMRI) in 27 women with morbid obesity (body mass index bChiba University Graduate School of Medicine, Neurology, Chiba, Japan 40.4095 ± 4.5 Kg/and body weight 104.381 ± 15.06 Kg) using a cChiba University Graduate School of Medicine, Neurosurgery, Chiba, magnetic resonator GE-3.0 Teslas Discovery-MR750®. Data were Japan collected in a longitudinal study one week before undergoing gastric dDokkyo Medical University, Neurology, Tochigi, Japan bypass surgery and six months later. eDokkyo Medical University, Neurosurgery, Tochigi, Japan A brain functional connectivity matrix was constructed with fSchool of Medicine, International University of Health and Welfare, normalized correlation coefficients using wavelet decomposition International University of Health and Welfare Mita Hospital, Neurol- scales between regional signals dependent on blood oxygenation ogy, Chiba, Japan level and its co-registration in the JD-Power atlas of 264 brain regions. The properties of the NN were calculated in connectivity It is established that deep brain stimulation of the subthalamic matrices with a connection density of 10–50%. Permutation tests nucleus (STN-DBS) improves motor dyfunction in advanced PD. were performed to evaluate the group differences (p b 0.05) for each However, it remains unclear as to whether STN-DBS would be network metric and density. effective for bowel dysfunction. Then, we investigate the effects of STN-DBS on owel function in PD. A total of 19 patients with PD who Results underwent bilateral STN-DBS were enrolled. We performed ques- The rs-FMRI data obtained pre-surgery showed an increase in tionnaire of bowel symptom and colonic transit time (CTT) study path length and a decrease in the weighted overall efficiency before and 3 months, 1 year after the STN-DBS surgery. The CTT ARTICLE IN PRESS

302 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx study was performed using the repetitive ingestion method. We cadence, acceleration, and trajectory, suggesting music therapy is counted the number of markers in each segment of the large bowel effective at reducing gait disturbance in PD. and then calculated each CTT. Subjective improvement in bowel frequency was shown in 15 patients (88.2%) at 3 months post doi:10.1016/j.jns.2019.10.1378 surgery and 10 (83.3%; n = 12) at 1 year post surgery. And stop or dose reduction of purgatives in 7 patients (42.2%) at 3 months and 10 (83.3%; n = 12) at 1 year post surgery. Improvement of the total CTT was shown in 15 patients (78.9%) at 3 months post surgery and WCN19-1706 10 (83.4%; n = 12) at 1 year post surgery. The right CTT, rectosigmoid CTT and total CTT were signiﬁcantly decreased, but the left CTT was unchanged at both 3 months and 1 year post surgery Poster shift 03 - Autonomic nervous system disorders / history of (Total CTT: 70.4 hours [pre] to 57.8 [3M], 47.2 [1Y]). STN-DBS can neurology /motor neuron disease / movement disorders / MS & improve bowel dysfunction, resulting in reduction of purgatives. One demyelinating diseases / neuromuscular disorders / vestibular of the causes may be improvement of colon and anorectal motility by disorders and nutritional disorders or intoxications STN-DBS. Normative values of the brief international cognitive assessment doi:10.1016/j.jns.2019.10.1377 for multiple sclerosis (BICAMS) in Arab population: corrected for age, sex and education

A. Alboudia, F. Alshaikhb, H. Aqlehb, A. Hadidb, A. Rais Alib WCN19-1696 aMercy Health Saint Mary, Neurology, Grand Rapids, USA bDubai medical college, Medicine, Dubai, United Arab Emirates Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & Objective Provide BICAMS normatives in Arab population with correction demyelinating diseases / neuromuscular disorders / vestibular for age, gender and education, as well as to assess test-retest disorders and nutritional disorders or intoxications reliability in Arab population, following the international standards for test application. Music therapy on gait disturbance and gait analysis for Par- kinson's disease using a portable gait rhythmogram Background Cognitive impairment is common in multiple sclerosis patients E. Gondo, A. Hayashi, S. Mikawa, Y. Irisawa with 43-70% of patients are affected. This starts early in the disease a Graduate School at Juntendo University, Rehabilitation, Tokyo, Japan course. The international cognitive assessment for multiple sclerosis b Juntendo University, Rehabilitation- Neurology, Urayasu, Japan BICAMS is a new battery for cognitive assessment in multiple c LSI Medience Corporation, Clinical examination business, Tokyo, Japan sclerosis patients. It was suggested in 2012 and being evaluated in different languages and cultures. This the first time to our knowledge Gait disturbance is one of the most frequent and intractable to be evaluated in Arab population. motor symptoms in Parkinson’s disease (PD). The aim of this study is to develop the application of music therapy and a new method of Methods gait analyses in PD. Normal subjects were recruited from different areas in Dubai and The subjects of this study were 20 outpatients with PD with gait included many Arab nationalities. Corrections for demographics were disturbance. We examined whether music therapy is effective as calculated using multi-variable linear regression models. Test-retest rehabilitation for gait disturbance in PD, by using a portable gait reliability was assessed using the Pearson correlation coefficient. rhythmogram. PD gait speed was significantly slow, the steps were small, the cadence was also slower compared as that of normal Results control. By the addition of 3-dimensional acceleration measure- Two hundred eighty-one normal subject were enrolled (174 ment, the strength of PD gait was weak compared as normal women, mean age 30.04±10.31 years, mean education 17.73±3.49 control. The regression line indicates that gait force is an essential years). The baseline and re-test were highly correlated, with r is 0.77, and BVMT-R respectively. We made װ-factor for gait speed. We checked a trajectory of the accelerometer, 0.67 and 0.67 for SDMT, CVLT which indicates that PD patients have a large amplitude of the a regression model equation to help calculate the expected value of mediolateral direction. BICAMS for specific age, gender and years of education. The patients carried out common walking training tasks carrying a portable gait rhythmogram. The walking training eight tasks were Conclusion walking 5 meters in a straight line at subjects’ usual walking speeds, This study is showing that BICAMS is reliable in Arab population. fast walking, walking with hand clapping, walking in step with music It also gives the normatives of the test among Arabs. The study is of 90 BPM (beat per minute), 100 BPM, 110 BPM, and 120 BPM, and showing that the BICAMS can be used in day to day practice and for fast walking without music. longitudinal patient assessment. We recognized an effect of the music therapy by comparing walking before and after accompanied by stimulation with music. doi:10.1016/j.jns.2019.10.1379 Significant improvements were observed in stride, gait speed, ARTICLE IN PRESS

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WCN19-1713 Background Physical exercise (PE) reduces individual's ability to perform physical activity due to acute muscle weakness especially in people Poster shift 03 - Autonomic nervous system disorders / history of with underlying neuromuscular condition such as multiple sclerosis neurology /motor neuron disease / movement disorders / MS & (MS). demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications Objective Objective is to develop accurate and comparable home-monitor- Effects of physical exercise on balange, range of motion (ROM) ing system for MS-patients that also takes in consideration the and angular velocity (AV) measurements differential effect of physical exercise in accurate monitoring.

T. Kaska, A. Kuusika, S. Meerolla Kumara, H. Maamägib, K. Gross-Pajub Patients, Materials and methods aTallinn University of Technology, Thomas Johann Seebeck Department 15 MS patients and 15 healthy patients (individuals with no of Electronics, Tallinn, Estonia underlying neuromuscular disorder diagnosed) with the age range of bWest-Tallinn Central Hospital, Department of Neurology, Tallinn, 20-35 years with equal amount of female and male patients. PE on Estonia group 1 consisted of rapid walk from 2 meter long hallway (4 meters in total). PE on group 2 consisted of running on treadmill for 1 or 1,5 ARTICLE IN PRESS

304 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx km depending on individual's previous physiological status. Range of K. Roberto, A. Espiritu, M. Astejada motion (ROM), angular velocity (AV) and balance (Romberg test) University of the Philippines - Philippine General Hospital, Department tests were carried out pre- and postworkout. Statistical analysis of Neurosciences, Manila, Philippines (ANOVA) measured basic statistics, postural sway (PS) and normalized jerk score (NJS) (smoothness of movement in more depth) to Neuromyelitis optica spectrum disorders (NMOSD) are a group analyze the dynamic movement of patient's self-balance regulation. of autoimmune inﬂammatory process associated with the presence of aquaporin-4 autoantibody (AQP4-IgG), preferentially affecting Results the optic nerves and spinal cord. Mycophenolate mofetil (MMF) is Statistical analysis showed no statistical difference between an immunosuppressant proven to be effective in a number of balance values meaning that physical exercise has no effect on balance immune-mediated disorders. The main objective of this study was and ROM on all individuals (P-value N 0.05). ROM worsened slightly in to conduct a systematic review of case series and cohort studies to MS patients whereas improved in healthy subjects. Interestingly AV evaluate the effectiveness in the reduction of relapse rate and was better for MS-patients. MS-patients with higher expanded disability progression, and tolerability of MMF in patients with disability status scale (EDSS) showed higher physiological changes. NMOSD Articles were searched from Cochrane Central Register for Controlled Trials by The Cochrane Library, MEDLINE by PubMed, Conclusion LILACS, and ClinicalTrials.gov websites from June to December Our study showed the effect of physical with higher impact on 2018. Four retrospective case series, two retrospective cohorts, and the balance of individuals with neuromuscular disorder than on three prospective cohort, which utilized MMF for patients with healthy individuals. Group comparisons didn't reveal statistically NMOSD, were included. Data collected from the included studies signiﬁcant changes. were study design, population characteristics, treatment regimen, annualized relapse rate (ARR), expanded disability status scale doi:10.1016/j.jns.2019.10.1380 (EDSS) scores, and adverse events. Mycophenolate mofetil may be effective in reducing ARR to up to 92% when used as initial, second-line, or adjunct therapy. It may also be effective in reducing disability progression as noted in the improved or WCN19-1722 stabilized median EDSS scores in 83-91% of patients. It is well tolerated in patients with NMOSD, with hair loss (4-6.78%), elevated liver enzymes (0.95-6.67%), and low white blood cell Poster shift 03 - Autonomic nervous system disorders / history of and neutrophil counts (0.92-4%) as the most frequent adverse neurology /motor neuron disease / movement disorders / MS & events reported. There is a need for randomized controlled demyelinating diseases / neuromuscular disorders / vestibular trials to determine the true treatment effect and risks of MMF disorders and nutritional disorders or intoxications therapy.

Effectiveness and tolerability of mycophenolate mofetil in the treatment of neuromyelitis optica spectrum disorders: A system- doi:10.1016/j.jns.2019.10.1381 atic review ARTICLE IN PRESS

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WCN19-1727 (LSES), Beck Depression Inventory and Rosenberg Self-Esteem Scale after being fully informed about the goal and content.

Poster shift 03 - Autonomic nervous system disorders / history of Results neurology /motor neuron disease / movement disorders / MS & Demographics of patients and scores on questionnaires are demyelinating diseases / neuromuscular disorders / vestibular shown in Table 1. In general, enacted and internalized stigma disorders and nutritional disorders or intoxications showed a mild to strong positive correlation with age (r=0.274, p=0.043; r=0.341,p=0.011,respectively), disease duration Internalized and enacted stigma in relapsing-remitting and (r=0.330,p=0.014; r=0.288,p=0.033,respectively), EDSS(r=0.281, secondary-progressive multiple sclerosis p=0.038; r=0.368,p=0.006,respectively), depression (r=0.324, p=0.016;r=0.645,p=0.000; respectively) and loneliness (r=0.356, S. Demircia,C.Yılmazb p=0.008; r=0.467, p=0.000; respectively). Higher was the enacted aSüleyman Demirel University School of Medicine, Neurology, Isparta, and internalized stigma lower was the self-esteem (r=-0.289, Turkey p=0.034; r=-0.565, p=0.000; respectively). In SPMS group EDSS bSüleyman Demirel University faculty of Science and Literature, showed strong correlation with internalized stigma(r=0.711, fi Sociology, Isparta, Turkey p=0.014), self-ef cacy(r=-0.645, p=0.032), self-esteem(r=-0.619, p=0.047) and depression(r=0.720, p=0.012). Either enacted or internalized, stigma caused increased feeling of loneliness (r=0.341, Background p=0.024; r=0.406, p=0.006; respectively) in RRMS group, the same Multiple sclerosis patients may confront with many psychosocial relationship was not observed in SPMS patients. Internalized stigma problems. Enacted or internalized stigma is one of the important correlated negatively with self-efficacy(r=-0.624, p=0.000) in RRMS aspects affecting patients’ psychosocial well being. patients, the same relationship was not significant (r=-0.585, p=0.059) for SPMS patients. Objective To evaluate the experienced internalized and enacted stigma in Conclusion relapsing-remitting(RRMS) and secondary-progressive(SPMS) MS The proven stigma affects mood, self-esteem, self-efficacy and patients. adaptation to disease in both group of patients, however those with longer disease duration and disability may use different adaptation Methods and coping strategies. Fifty-five patients from the outpatient clinic agreeing to partici- pate the study were asked to fill in the Stigma Scale for Chronic doi:10.1016/j.jns.2019.10.1382 Disease (SSCD), UCLA-Loneliness Scale, Liverpool Self-Efficacy Scale ARTICLE IN PRESS

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WCN19-1728 WCN19-1730

Poster shift 03 - Autonomic nervous system disorders / history of Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications disorders and nutritional disorders or intoxications

Upregulation of programmed cell death ligand 1 expression in the Multifocal motor neuropathy presenting without conduction skeletal muscle of patients with myasthenia gravis block and with fulminant respiratory failure: A case report

K. Iwasaa, H. Yoshikawab, Y. Furukawac, M. Yamadaa M. Bharath, P.S. Kumar, A.B. Netto, R. Patil aKanazawa University Graduate School of Medical Science, Department Bangalore medical college and research institute, Neurology, Bangalore, of Neurology and Neurobiology of Aging, Kanazawa, Japan India bKanazawa University, Health Service Center, Kanazawa, Japan c National Hospital Organization Ishikawa Hospital, Department of Mr.P,a 38 year old gentleman presented with progressive Neurology, Kaga, Japan weakness of left upper limb for 8 months,progressive weakness of right upper limb for 6 months,progressive thinning of both hands Object for 6 months and breathlessness on lying down for 4 months.There We investigated the expression patterns of programmed cell was no history of craniobulbar,sensory or autonomic symptoms. death-ligand 1 (PD-L1)in the skeletal muscle of patients with Family history was negative.On motor system examination,there myasthenia gravis (MG). was asymmetric distal predominant weakness of both upper limbs with wasting of intrinsic hand muscles bilaterally.Higher mental Methods functions,cranial nerves and sensory system examination was We examined muscle biopsies from 15 patients with MG. In normal.Routine hematological investigations,ANA profile,par- addition, biopsy specimens were obtained from 5 patients with aneoplastic profile&protein electrophoresis were unrewarding. inflammatory myopathy and 6 patients without myopathy. The ECG,2D ECHO,HRCT-Chest done i/v/o orthopnoea were normal. sections were stained with rabbit monoclonal PD-L1 antibody (clone: Ultrasound chest&abdomen showed sluggish movements of dia- 28-8). qRT-PCR was performed using TaqMan® Gene Expression phragm bilaterally.Anti-MUSK antibody,RNS,Anti GM1 antibody Assaysfor the target genes (PD-L1, Hs01125301_m1) and an internal were negative.Nerve conduction study showed asymmetric motor control gene (GAPDH, Hs99999905_g1). Gene expression levels were axonal neuropathy involving both upper limbs with no evidence of normalized to that of GAPDH; the fold change was calculated with conduction block.Needle EMG was suggestive of acute and chronic the 2-ΔΔCtmethod. To analyze the relationship between PD-L1 mRNA denervation involving both upper limbs.MRI cervical spine with expression and the quantitative MG(QMG) score, we relied on an dynamic flexion was normal.A trial of steroids was considered and approach using a generalized additive model (GAM). patient significantly worsened with severe respiratory distress, single-breath-count dropping from 25 to 3.A repeat bedside Results ultrasound showed complete paralysis of the right hemidiaphragm. In 11 of the MG patients, immunohistochemistry confirmed the IVIG was started at 2g/kg over 5 days following which patient's localization of PD-L1 in the muscle cell membrane with cytoplasmic dyspnoea significantly improved to Single-Breath-Count of 30 over integrity. PD-L1 mRNA expression levels were higher in the muscles one week.I/V/O asymmetric pure motor neuropathy affecting both of MG patients as compared to those of non-myogenic patients (p b upper limbs,significantly worsening with steroids and promptly 0.05). Moreover, unimodal relationships between PD-L1 mRNA levels improving with IVIG,a diagnosis of Multifocal motor neuropathy and QMG scores for disease severitywere identified by GAM. We was considered and patient was discharged to follow-up for estimated that QMG score influenced PD-L1 mRNA expression (the monthly IVIG-pulses.Concluding,this case highlights the fact that deviance explained is 60.2% in the GAM). multifocal motor neuropathy can present as an axonal variant without conduction blocks and respiratory failure can be one of the Conclusion presenting complaints. We demonstrated the up-regulation of PD-L1 expression in NOTE:Patient/IRB approval taken muscle cells in MG. The severity of MG symptoms likely affects PD- L1 expression in muscle cells, and PD-L1 expression in muscle cells might influence the autoimmune reactivity and immune tolerance of MG. doi:10.1016/j.jns.2019.10.1383 doi:10.1016/j.jns.2019.10.1384 ARTICLE IN PRESS

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WCN19-1732 T. Lataa, D.K. Khatrib aRajiv Gandhi College of Arts Commerce and Science, Biology, Mumbai, India Poster shift 03 - Autonomic nervous system disorders / history of bInstitute of Chemical Technology, Pharmacology, Mumbai, India neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Objective disorders and nutritional disorders or intoxications There is a great degree of interest in the use of mindfulness- related involvements in the management of Parkinson Disease (PD) Constipation as a predictor of Parkinson's disease in persons of like personal care, stress-management and other lifestyle factors, Uzbek nationality such as diet and exercise, assist in reducing disability and improving quality of life in people with PD. The aim of this study is to explore O. Naimov, E. Abdukodirov, R. Matmurodov, K. Khalimova the experiences of self-care management among people living with Tashkent medical academy, Neurology, Tashkent, Uzbekistan PD.

Constipation may be a complication of Parkinson's disease (PD), Methods but in some cases it may appear earlier than the motor symptoms of In the present study we have selected 1000 patients of age the disease. between 45-65 years with PD. We have conducted face to face interview on the selected PD patient. During the interview we asked Objective different questions like 1) Smoking habbit? 2) What are the eating To study the Constipation of patients with PD in persons of Uzbek habits? 3) Do they follow prescription and taking medicines on ﬁ nationality. time? 4) What is the pattern of Exercise? 5) What are the dif culties in understanding the symptoms? 6) Do they require more education Material and methods on PD care? 7) Alcohol consumption. We examined 36 patients with PD at the age of 33 to 65 years (mean age 46.7±8.1 years), 18 men and 18 women. The control Results ﬁ group consists of 10 patients, without parkinsonism. All patients We identi ed that consumption of alcohol and cigarette smoking underwent clinical, neurological, neuropsychological studies. is main key factor in worsening the symptoms. Also, we found that the self management is not enough unless they are educated on Results regularly basis. It has also been observed that the patients cannot 12 patients out of 36 had constipation after treatment with control on their diet and lifestyle management as well due to levodopa, who were treated for a long time and large doses. In 24 constraint in living status. patients with PD. Constipation of patients were analyzed before and after the manifestation of the main motor symptoms, such as Conclusion tremor, hypokinesia and rigidity. In 18 patients (75%), The It highly recommended that a multifaceted, healthy lifestyle and constipation as the intrusiveness began a few years (10-15 years) mindfulness programme need to be designed to improve quality of earlier than clinic motor symptoms. At the same time these life for PD patients. Self-management program by a dedicated patients were observed violations of smell and sleep. At the same medical professionals and social workers are required to provide time these patients were observed violations of smell and sleep. In the better care and management of PD. 6 (25%) patients, other non-motor symptoms were observed, doi:10.1016/j.jns.2019.10.1386 which aggravate the course of vomiting progression. Of the 24 patients who had constipation after 6-7 years, observed sided tremor. These patients did not suffer from diseases of the gastrointestinal tracts. WCN19-1742 Сonclusion Thus, constipation is the main vegetative disorder of PD, but some Poster shift 03 - Autonomic nervous system disorders / history of cases may be a predictor of the disease together with other non- neurology /motor neuron disease / movement disorders / MS & motor manifestations, such as disturbances of smell and sleep, as well as changes in character. demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1385 Initial manifestations of Parkinson's disease in Uzbek nationality

R. Juraev, E. Abdukodirov, R. Matmurodov, K. Khalimova WCN19-1738 Tashkent medical academy, Neurology, Tashkent, Uzbekistan

Non-motor symptoms may be predictors of Parkinson's disease Poster shift 03 - Autonomic nervous system disorders / history of (PD). It depends on the damage not only of the dopaminergic neurology /motor neuron disease / movement disorders / MS & systems, but also on the cholinergic, serotoninergic and glutamater- demyelinating diseases / neuromuscular disorders / vestibular gic systems. disorders and nutritional disorders or intoxications Aim Diet and lifestyle management in Parkinson disease: A population to study the predictors of early diagnosis of PD in persons of based study in India Uzbek nationality and create a prediction algorithm. ARTICLE IN PRESS

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Material and methods Implicit Model of Illness Questionnaire (IMIQ) and the Anxiety and 84 patients with PD, aged from 33 to 65 years (mean age 46.7 Depression Scale Hospital (HADS) were administered. ±8.1 years), 42 men and 42 women were examined. The control group consisted of 12 patients with vascular parkinsonism (VP) Results which coincided by age and sex. All patients underwent clinical, Patients refer to a more specific etiology at the origin of the disease neurological, neuropsychological and neurophysiological studies. and perceive MS as less disabling than controls. The males obtained higher scores in "localization" (p = 0.015) and "chance" (F (1, 24) = Results 5.26) ; p = 0.031) so they perceive a more specific location and a greater In 84 patients with PD, disturbances of touch, cognitive, affective influence of chance on the evolution of the disease. The youngest disorders, that is, depression and anxiety, autonomic, sensory patients perceive a lower influence of personal habits. In general, the disorders, in particular pain syndrome and sleep disorders, were highest scores correspond to the factors "disability", "mutability" and analyzed. In 38 patients (74.6%) of 51 patients with PD, the main "cause" and the lowest ones with "identity","cure" and responsibility". symptoms of the disease began several years ahead than tremor, hypokinesia and rigidity. Sleep disturbances in the form of insomnia, Conclusions hypersomnia and parasomnias are relatively common in patients MS is perceived as chronic, with serious consequences and with with PD (80.4%), and only insomnia was observed in VP in 25.0% of little perception of personal control and tretament. patients. Affective disorders in the form of depression and anxiety in PD were found in 84.3% of cases, and in VP - in 37.6%. Sensory doi:10.1016/j.jns.2019.10.1388 impairment in the form of pain in PD was recorded in 76.5% of patients. Various autonomic disorders were relatively more pronounced in patients with PD (82.3%). WCN19-1755 Conclusions Sleep and olfactory disorders, vegetative and psycho-emotional disorders can be predictors of PD in people of Uzbek nationality. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1387 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Vitamin D and ocxidative stress in multiple sclerosis WCN19-1748 N. Beridze, M. Beridze, T. Losaberidze, E. Devidze, G. Chakhava, O. Poster shift 03 - Autonomic nervous system disorders / history of Samushia, N. Gogokhia, N. Kvirkvelia Tbilisi State Medical University, Neurology, Tbilisi, Georgia neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Vitamin D receptors found in most immune cells and may play disorders and nutritional disorders or intoxications role in consequences of inﬂammation related with multiple sclerosis (MS). Study aimed at deﬁning the role of vitamin D in secondary Perception of the disease in patients with multiple sclerosis progression of MS.

a b b M.Y. Pérez Martín , M. González-Platas , P.T. María Pilar Methods a Hospital Universitario de Canarias, Depatment of Neurology, La Totally 45 MS patients, 30 -with relapse remitting MS (RRMS), Laguna, Spain 15- with secondary progressive MS (SPMS) aged 20 to 45years b Hospital Universitario de Canarias, Department of Neurology, La investigated. Control comprised 12 healthy age matched individuals. Laguna, Spain Patients evaluated by EDSS. Brain visualized by MRI (1.5 T). Blood vitamin D assessed by electrocemiluminescence (ECL) method. Blood Background free toxic radicals detected by Electron Paramagnetic Resonance The image that one has about a certain disease can have method (EPR). EPR signals measured in arbitrary units. Statistics important implications in areas such as the search for sanitary help, performed by SPSS- 20. compliance with medical recommendations and adherence to treatment or the reactions of the population to the people who Results suffer from it. Blood vitamin D level (nmol/l) slightly differed in control against RRMS and was signiﬁcantly higher compared to SPMS (35± 4.4 Objective versus 29± 6.8 versus 22±6.2; pb0.05 and pb0.01 respectively). To study the perception that patients with multiple sclerosis (MS) Blood EPR specters of Lypoperoxiradical (LOO-) and reactive oxygen - have of their disease as well as its relationship with different (02) increased in SPMS patients compared to RRMS and control (11.2 variables (gender, age, age of onset, degree of neurological disability, ±0.6 versus 6.4 ±0.5 versus 2.8 ±0.3; pb0.05) respectively (8.4 ± degree of of anxiety and degree of depression). 0.6 versus 4.2 ± 0.4 versus 1.8 ± 0.2; pb0.05). Negative correlation found between blood vitamin D and EDSS (r= -0.51 pb0.05) and - - Methods with blood LOO EPR specters and 02 (r= - 0.72 and r= - 0.64 25 patients (19 women) with a mean age of 40.12 years, an age of respectively, pb0.05). Multiple logistic regression showed signiﬁ- onset of 29.84 and a score on the EDSS 0 to 6.5 and 31 healthy cance of blood vitamin D in conjunction with blood LOO- toward the participants as control subjects. The Spanish adaptation of the brain damage volume (pb0.05). ARTICLE IN PRESS

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Conclusion So population screening using INSIT could be used for early Present study indicated that vitamin D low content might assist detection of IPD ﬂ free radical production during chronic in ammation, thus leading to doi:10.1016/j.jns.2019.10.1390 secondary progression of MS. doi:10.1016/j.jns.2019.10.1389

WCN19-1773

WCN19-1757 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Poster shift 03 - Autonomic nervous system disorders/history of demyelinating diseases/neuromuscular disorders/vestibular dis- neurology/motor neuron disease/movement disorders/MS & orders and nutritional disorders or intoxications demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Clinical and radiological progression of RRMS after a brief period of B-interferon discontinuation in neurosciences hospital Application of Indian Smell Identiﬁcation Test (INSIT)as screening test to study unawareness of Hyposmia and effect of cognition on Z.N.H. Al Uqla, Z.A. Jihanker it inpatient of idiopathic Parkinson’s disease Neurosciences hospital, Neurology, Baghdad, Iraq

U. Patel, C. Sankhla Objective P.D.Hinduja Hospital, Neurology, Mumbai, India The aim of the study is to explore the out come of patients who obligated to stop his ( interferons treatment). Background Olfactory bulb involvement may be the earliest site of pathology Methods in IPD. A cross sectional-retrospective study was made on 50 patients This study examines the frequency of unawareness of hyposmia with MS on interferon treatment who stopped their DMT in in study cohorts with and without IPD and its correlation with Neurological unit, in neurosciences Teaching hospital from February cognitive impairment. 2016 to August 2016. patient with RRMS who stopped his/her DMT for 6 months was assessed by EDSS and sent for a new 3 T MRI with Objectives DWI, FLAIR and Gadolinium contrast in axial and sagittal view . In this study, olfactory function has been assessed using an Indian Smell Identification test(INSIT) smell test to evaluate the unaware- Results and discussion ness of hyposmia in the IPD . A total of 50 patients 38 females and 12 males with relapsing Their cognitive status has been assessed using Mini Mental State remitting multiple sclerosis included in this study meeting the Examination(MMSE) to study effect of cognitive status on unaware- McDonald criteria with the most common affected age group was ness of hyposmia in IPD cases. (30-39) 24/50 cases (48%), and most common in female 38/50 cases (76%), with the most of them received DMT for less than 1 year 22/50 Materials and methods cases (44%) and most of them received avonex 22/50 cases (44%) , the Single Centre cross sectional prospective study study didn’t show significant association between age group and 30 cases of IPD & age and sex matched 30 healthy controls gender, age group and type of treatment, gender and duration of 3 months treatment, gender and type of treatment, cerebellar lesion and DMT, The cutoff used for hyposmia was an INSIT score ≤4. brainstem lesion and DMT, spinal cord lesion and DMT, while there Unawareness was defined as reporting normal sense of smell in was significant relation between age group and duration of treatment, the setting of a low INSIT. mean number of relapses, mean number of EDSS, duration of treatment and relapses, DMT and hemispheric lesion, optic nerve Result lesion and DMT, complicated lesions and DMT, new T2 lesions and Out of the 30 patients in each group, 18 patients were unaware of DMT, Gadolinium enhancing lesions and DMT, brain atrophy and DMT. hyposmia in the IPD group as compared to 4 patients in the control doi:10.1016/j.jns.2019.10.1391 group Pvalue of b 0.001 The mean smell identification score using INSIT was 4.13 ± 2.42 in IPD patients and 6.86 ± 2.3 in the control group Pvalue of b0.0001 Mean MMSE score who were unaware of hyposmia was WCN19-1776 27.89 who were aware of smell mean MMSE was 29.25 Pvalue of 0.1087. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Conclusion demyelinating diseases/neuromuscular disorders/vestibular dis- Unawareness of hyposmia in IPD is high compare to elderly orders and nutritional disorders or intoxications without Parkinsonism. Under reporting of hyposmia seen in patients with IPD. Early onset but long survival in Chinese sporadic amyotrophic There is no increase in unawareness of hyposmia in IPD with dementia. lateral sclerosis ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 313

T. Liua, L. Mingshengb, L. Cuib said, clinicopathological co-mobidity of the two diseases has aPeking University health science center, Medical English, Beijing, China remained unproven. bPeking Union Medical College Hospital, Neurology, Beijing, China Methods Objectives We investigated neuropathological characteristics of two patients To explore the cause of long survival but early onset and other who clinically presented both PSP and ALS. prognostic factors among Chinese sporadic amyotrophic lateral sclerosis (ALS) patients. Results The patients, without family history of neurological disorders, Methods first presented with atypical parkinsonism consistent with PSP with Patients with ALS were recruited and followed up from Jan 2013 Richardson’s syndrome or progressive gait freezing. In a few years, to Jan 2017. Phenotype and survival were compared among different they developed muscle weakness with or without pseudobulbar age-at-onset groups. Candidate prognostic factors were analyzed by symptoms, which was compatible with ALS. Disease duration was 5- Kaplan-Meier method, Cox regression and Royston Parmar (RP) 8 years after the onset of PSP and 1-2 years after the onset of ALS. model dealing with breaches of proportional hazard assumption. Neuropathological findings demonstrated coexistence of PSP and ALS in both cases. Immunohistochemical examination in a recent case Results confirmed 4-repeat tauopathy including globose type neurofibrillary In the cohort of 530 patients, mean age-at-onset was 53.68 years tangles, tufted astrocytes, and oligodendroglial coiled bodies, as well (SD:10.85) and overall estimated median survival time was 59 as TAR DNA-binding protein 43 kDa pathology in association with months (95% CI: 48.29-69.71). Pairwise comparison showed that upper and lower motor neuron degeneration. In this case, immuno- patients above 65 years at onset were more frequently bulbar onset blotting showed hyperphosphorylated full-length 4-repeat tau bands (adjusted residual: 3.0), less frequently lumbosacralonset (adjusted with 64 and 68 kDa and C-terminal fragments with 33 kDa, residual: -3.0), and had shorter survival compared with other age supporting the diagnosis of PSP and excluding other parkinsonian groups (p=0.002).Cox and RP model demonstrated independent disorders such as corticobasal degeneration. prognostic variables including age at onset, bulbar onset, diagnostic delay, MRC-score at first diagnosis and region of residence. Conclusions Our cases highlight clinicopathological comorbidity of PSP and Conclusions ALS in sporadic cases. The possibility of multiple proteinopathies This clinic-based study suggested that Chinese sporadic ALS should be considered when distinct symptoms developed in the patients had relatively long survival probably due to younger ageand disease course. less bulbar onset cases. Short diagnostic delay, low MRC-scoreand northern residence were also predicative of short survival. doi:10.1016/j.jns.2019.10.1393 doi:10.1016/j.jns.2019.10.1392

WCN19-1806

WCN19-1784 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Poster shift 03 - Autonomic nervous system disorders / history of demyelinating diseases/neuromuscular disorders/vestibular dis- neurology /motor neuron disease / movement disorders / MS & orders and nutritional disorders or intoxications demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications A cognizance analysis on amyotrophic lateral sclerosis in Middle East countries Clinicopathological co-morbidity of progressive supranuclear palsy and amyotrophic lateral sclerosis S. Elayarajaa, G. Elumalaib, V. Jangida, A. Bhonslea aYoung Scientiﬁc Researcher, Neuroscience, Georgetown, Guyana b Y. Izumia, K. Fujitab, T. Matsubarac, F. Udakad, R. Miyamotoe,T. Team NeurON - College of Medicine - Texila American University, Kawaraia, S. Murayamac, R. Kajia Neuroscience, Georgetown, Guyana aTokushima University Hospital, Department of Neurology, Tokushima, Japan Introduction bTokushima University Hospital, Deaprtment of Neurology, Tokushima, ALS, or amyotrophic lateral sclerosis, is a progressive neurode- Japan generative disease, affects nerve cells in the brain and spinal cord [1]. cTokyo Metropolitan Geriatric Hospital & Institute of Gerontology, Ever since, the repeat expansions in the C9ORF72 gene were Department of Neurology and Neuropathology, Tokyo, Japan identiﬁed as a major cause of ALS and frontotemporal dementia, dSumitomo Hospital, Department of Neurology, Osaka, Japan researchers have been debating whether the disease is caused by loss eTokushima University Hospital, Department of Neurology, Tokushima, of normal protein(s) or accumulation of toxic aggregates [2]. Japan Demographic details Background Amyotrophic lateral sclerosis (ALS) epidemiology has rapidly Coexistence of distinct neurodegenerative diseases in single cases developed in the Middle East, was spread after the gulf war [3]. In recently gathers greater attention. In this line, phenotypic co- 2015, there were 45,810 men and 34,352 women diagnosed with occurrence of progressive supranuclear palsy (PSP) and amyotrophic ALS in the developed countries. The projected number ALS is lateral sclerosis (ALS) has been documented in several cases. That expected to grow from 80,162 in 2015 to 105,693 in 2040, represents ARTICLE IN PRESS

314 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

an increase of N31%. In Middle East (like, Iran) there is high WCN19-1809 prevalence in ALS, 112% (Fig-1), due to insignificant awareness on ALS [4]. Poster shift 03 - Autonomic nervous system disorders/history of Recent advancements neurology/motor neuron disease/movement disorders/MS & The recent discovery of pathological TDP-43 in both ALS and demyelinating diseases/neuromuscular disorders/vestibular dis- FTLD-U confirms a new biochemical class neurodegenerative disease, orders and nutritional disorders or intoxications called TDP-43 proteinopathies [5]. Recently, researchers developed an antibody that reduces the number of TDP-43 protein aggregates in Epilepsy and multiple sclerosis the mice brains with ALS, resulting in significant improvements in their cognitive and motor performance (Fig-2). Preliminary results J. Djelilovic-Vranic, E. Dozic, M. Tiric-Campara, A. Nakicevic suggest that injecting TDP-43 antibodies directly into the cerebro- Clinical Center of the University of Sarajevo, Neurology clinic, Sarajevo, fl spinal uid could effectively reduce protein aggregates in nerve cells Bosnia and Herzegovina [6]. Introduction Conclusion Multiple sclerosis (MS) is an autoimmune disease that affects the The current cognizance analysis focus on the mechanism of nervous system.Epilepsy is a clinical syndrome of primary cerebral single-chain (scFv) antibodies against the RNA recognition motif 1 origin in which there is a disorder of consciousness, feelings and (RRM1) of TDP-43 protein aggregates in the ALS pathology. behavior. It is approximately equally represented in male and female Keywords and can occur at any age. ALS Middle East Aim TDP-43 To determine the occurrence of epileptic manifestations in Single-chain (scFv) antibodies patients with MS. doi:10.1016/j.jns.2019.10.1394 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 315

Patients and methods of MuSK myasthenia gravis. There was improvement with addition of In this paper, we analyzed all patients who were treated with MS pyridostigmine and she was later discharged on Pyridostigmine, at the Neurological Clinic in Sarajevo for period of three years Prednisolone and Azathioprine. On follow up, the patient is showing (January 2016-December 2018) and who had epileptic seizures. All sustained improvement. patients had conﬁrmed diagnosis of MS in accordance with McDonald's criteria. Conclusions Autoimmune disorders are being increasingly reported with HIV Results infection since the advent of HAART. MuSK myasthenia gravis with In this period there were a total of 517 patients with MS, 377 HIV has been reported in very few case reports. The use of were female and 140 were male, age between 18-75 years. 221 immunosuppressants in the treatment of these conditions should female and 61 male were treated for the ﬁrst time, while others had be carefully evaluated. repeated hospitalization, Relapse remitting form of MS had 334 of I have obtained patient and/or Institutional Review Board patients, secondary progressive had 56 and progressively relapse MS approval, as necessary. had 24 of patients Epileptic seizure had 31 of patients. . Only in one An Institutional Review Board and /or Animal Use Committee case there was a primary generalized form, while the other had have waived the requirement for their formal approval of the study. partial attacks with or without secondary generalization, In 2 cases there were partial complex attacks. Most of the epileptic manifesta- doi:10.1016/j.jns.2019.10.1396 tions were in the RR form MS with high EDSS (total 19), and 12 with SP form MS.

Conclusion WCN19-1848 Epilepsy is not so rare in patients with multiple sclerosis, and in our study was present at 6.5% of respondents. Poster shift 03 - Autonomic nervous system disorders / history of doi:10.1016/j.jns.2019.10.1395 neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications

WCN19-1833 Paroxysmal kinesigenic dystonia mimics focal seizure

a b a c a Poster shift 03 - Autonomic nervous system disorders/history of H. Elhasin , M. Szolics , A. Hassan , F. Salih , N. Soliman aTawam Hospital, Neurology, Al ain, United Arab Emirates neurology/motor neuron disease/movement disorders/MS & bTawam Hopsital, Neurology, Al Ain, United Arab Emirates demyelinating diseases/neuromuscular disorders/vestibular dis- cTawam Hospital, Internal Medicine, Al Ain, United Arab Emirates orders and nutritional disorders or intoxications Background A rare case of musk myasthenia gravis in a HIV patient Dystonia refers to a syndrome of involuntary sustained or spasmodic muscle contractions involving co-contraction of the a b b b a b V. Aglave , P. Ojha , H. Faldu , R. Ansari , P. Barvalia , A. Ansari agonist and the antagonist. Paroxysmal kinesigenic dyskinesia is a Senior Resident- Grant government Medical college and Sir JJ Hospital, one of the rare episodic hyperkinetic movement disorders, charac- Neurology, Mumbai, India terized by brief attacks of abnormal involuntary movements b Grant government Medical college and Sir JJ Hospital, Neurology, comprising of dystonia, chorea, athetosis, hemiballismus or a Mumbai, India combination, usually precipitated by sudden movements.

Background Case report Autoimmune disorders have been frequently described in HIV A 16 years old male previously healthy presented with 1 year patients but there has been little research on the association history of involuntary movement descried as a sudden stiffness of the between HIV and myasthenia gravis. MuSK myasthenia gravis left arm, sometimes involving the left side of the body after sudden coexisting with HIV is an even rarer entity. movement of the trunk or while standing up after prolonged physical rest. The spells usually lasted up to 30 to 60 seconds with frequency 3- Case report 4/week and were painless. The patient never had a loss of conscious- 50 years old lady with HIV and baseline CD4 count of 170, on ness during spells. He had a normal physical and neurological antiretroviral therapy from 1.5 years, presented with complaints of examination. Brain MRI, routine EEG, laboratory tests including liver difficulty swallowing, change in voice and intermittent double vision functions testes, serum ceruplasmin and copper and 24 hours urine since 10 months. There was a history of fluctuations. On examination copper were all normal. Simple partial seizure disorder was suspected. she had bilateral mild lateral gaze palsy with mild asymmetric As part of workup the patient was admitted in epilepsy bifacial weakness. Her gag reflex was weak on both sides. Her CD4 monitoring unite and had a long term video EEG. Recoded spells count done 6 months back had increased to 477 and her MRI brain were consistent with a focal dystonic posturing in the left side of the was normal. Myasthenia gravis was strongly suspected. Repetitive body precipitated by sudden active movement with a normal EEG Nerve Stimulation showed a decremental response in bilateral background, without any epileptiform discharges . Carbamazepine median innervated muscles. Her MuSK antibody was positive and 200 mg /day was initiated as a treatment of kinesigenic dystonia Ach receptor antibody was negative, which confirmed the diagnosis with good clinical response. ARTICLE IN PRESS

316 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Conclusion WCN19-1855 Paroxysmal kinesigenic dystonia can be frequently misdiagnosed with other medical conditions and required careful clinical assessment and work up. Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & doi:10.1016/j.jns.2019.10.1397 demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications

Beneﬁcial and protective effects of Terminalia chebula in a WCN19-1853 murine model of parkinson's disease

Poster shift 03 - Autonomic nervous system disorders / history of P. Kashyap, R. Khanna neurology /motor neuron disease / movement disorders / MS & Vivekananda Global University, Department of Basic and Applied demyelinating diseases / neuromuscular disorders / vestibular Sciences, Jaipur, India disorders and nutritional disorders or intoxications Present study was designed to investigate whether Terminalia The magnetic resonance imaging (MRI) of brain and cervical chebula (TC) extract would prevent 1-methyl-4-phenyl-1,2,3,6- spine findings, at time of diagnosis of neuromyelitis optica tetrahydropyridine (MPTP) induced neurotoxicity in male Wistar spectrum disorder patients in Isfahan city rats. MPTP was stereotaxically injected into the substantia nigra pars compacta of male Wistar rats. Male Wistar rats were divided into R. Mehdipour Dastjerdia, F. Ashtarib, V. Shaygannejadb following five groups: Group I received vehicle (saline (10 ml/kg for aNeurologist - Neuroscience PhD Candidate, Isfahan Neuroscience 21 days) orally); Group II received TC extract (200 mg/kg for 21 Research Center, Isfahan University of Medical Science -Iran, Neurology, days) orally; Group III was treated with 20 mg/kg MPTP i.p. for 21 Isfahan, Iran days; Group IV received 20 mg/kg MPTP, i.p. along with 200 mg/kg bIsfahan University of Medical Science, Neurology, Isfahan, Iran TC orally for 21 days. Group V received 20 mg/kg MPTP i.p. along with 400 mg/kg TC orally for 21 days. Introduction The mice treated with MPTP developed motor dysfunction and Neuromyelitis optica spectrum disease is a demyelinating disease behavioral alterations similar to Parkinson disease (PD), which was which mainly involves optic nerve and spinal cord. Most of patients tested with rotarod test, and hang test which revealed the impaired have abnormal brain MRI.The point is involvement of regions with performance and impaired neuromuscular strength respectively. high expression of aquaporin 4 channels. Long extending transverse Group III rats elicited significant increase in lipid peroxidation along myelitis (LETM) in cervical region is one of the main signs for with reduction in level of antioxidant enzymes and total antioxi- NMOSD diagnosis. dants. Histological data revealed that MPTP treated rats shown pathological changes like cellular inflammation and vascular degen- Method eration in brain tissue. Treatment with TC extract reversed the motor This is a retrograde study on 105 patients with neuromyelitis dysfunction, antixodant enzymes, lipid peroxidation significantly in a optica spectrum disease diagnosis, referred to Isfahan Kashani dose dependent manner. The Bcl-2/Bax gene expression ratio and Hospital MS clinic. protein expression of brain derived neurophic factor (BDNF) were Patient's age, age of onset, presenting symptom ,primary brain significantly increased in treated groups. fi and cervical MRI ndings and Expanded Disability Status Scale TC by its antioxidant property has a neuroprotective activity both (EDSS) recorded. Anti-AQP4 antibody was checked with cell based in motor activity and behaviourally in the MPTP induced PD in mice. culturing method in a unique laboratory. doi:10.1016/j.jns.2019.10.1399 Results 105 cases of NMOSD including 80(76.19%) females and 25 (23.81%) males with mean age of onset of 28±12 were recorded. 32(30.47 %) patients were.LETM and segmental spinal lesions were found in 48(45.71%) and 28(26.66%) of all. Diagnostic brain lesions WCN19-1872 (Including Area postreoma ,brain stem ,thalamus ,hypothalamus, periepandymal area of 3rd and 4th ventricle)and posterior per- Poster shift 03 - Autonomic nervous system disorders / history of iventricular lesions were seen in 34(32.38%) and 44(41.90%) of them. neurology /motor neuron disease / movement disorders / MS & Also posterior periventricular lesions were present in 16 seropositive demyelinating diseases / neuromuscular disorders / vestibular and segmental spine lesion were found in 9 (28.12%) of them.49.52% disorders and nutritional disorders or intoxications of all patients presented with optic neuritis at the first time and 39.04% referred with myelitis. Influence of dyslipidemia on multiple sclerosis disease activity Conclusion We found lesions in posterior periventricular region and seg- G. Kaur, S. Saravana, P. Banerjee, M. Kumar, D. Khurana mental type myelitis.These lesions did not mentioned in the NMOSD Postgraduate Institute of Medical Education and Research PGIMER, diagnosis criteriayet. Another point is occurrence of vertigo and Neurology, Chandigarh, India ataxia in 11 patients as first presentations of the disease which show lesions in regions with high expression of aquaporin 4 channels. Background Multiple Sclerosis (MS) is a demyelinating disorder in young doi:10.1016/j.jns.2019.10.1398 adults and may lead to significant disability. Dyslipidemia is a ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 317 recognized risk factor for cardiovascular diseases. Its role in iMcGovern Medical School, The University of Texas Health Science neurodegeneration is being increasingly recognized. However, its Center at Houston UTHealth, Department of Neurology, Houston, TX, role in disease activity and progression in MS has not been USA investigated. Background Objective The efficacy and safety of ocrelizumab in relapsing multiple To determine the prevalence of dyslipidemia and to assess the sclerosis were demonstrated in the double-blind control period of influence of dyslipidemia on Multiple Sclerosis disease activity. the Phase III OPERA I/II trials (NCT01247324/NCT01412333). Results for the 3-year follow-up of the pooled OPERA open-label extension Methods (OLE) period have previously been reported (Hauser SL, et al. Data was collected from our in-hospital MS registry. Inclusion ECTRIMS 2018;Abstract P590). criteria: Adult MS patients of any gender, Relapsing and Secondary progressive subtypes, on any disease modifying agent (DMA) and Objective patients providing informed written consent. For each the following To assess the efficacy of switching to or earlier initiation of parameters were assessed: Current EDSS, Annualized Relapse Rate ocrelizumab therapy on disease progression after 4 years’ follow-up (ARR), CSF OCB (Cerebrospinal Fluid Oligoclonal Bands) status, Lipid in the OLE period of OPERA I/II. profile (Total Cholesterol, Triglycerides, High Density and Low Density Lipoproteins in mg/dL). Methods At OLE commencement, patients continued ocrelizumab (OCR- Results OCR) or switched from interferon (IFN) β-1a to OCR (IFN-OCR). 31 MS patients with mean age 36.39±9.95, male: female Adjusted annualised relapse rate (ARR) and time to onset of 24-week ratio=1:1.6 were assessed. 27 (87.1%) had RR (Relapse Remitting) confirmed disability progression (CDP24) were analysed. and 4 (12.9%) were SP (Secondary Progressive). The mean age of disease onset was 31.45±9.48. ARR was 0.69±0.53 (Range 0-.CSF Results OCB was positive in 8 (25.8%) patients. Median EDSS was 3 (IQR:2- Among IFN-OCR patients, ARR decreased from 0.20 in the year 4). 12 (38.7%) patients had dyslipidemia; 2 groups were created on pre-switch to 0.10, 0.08, 0.07 and 0.04 at Years 1, 2, 3 and 4 post- basis of disease activity i.e., ARRb 1 and ARR N1. No correlation was switch. OCR-OCR continuers maintained low ARRs through the year found between dyslipidemia and disease activity (PN0.05). pre-OLE and the 4 years of OLE (0.13, 0.10, 0.08, 0.07 and 0.05). CDP24 was less frequent in OCR-OCR continuers versus IFN-OCR Conclusion switchers in the year pre-switch and OLE Years 1, 2, 3 and 4 (7.7%/ Prevalence of Dyslipidemia is high in MS, although no direct 12.0%, 10.1%/15.6%, 13.9%/18.1%, 16.2%/21.3%, 19.2%/23.7%; pb0.05, impact on disease activity in Relapsing and Secondary progressive all comparisons). The safety profile observed in the OLE was subtypes was observed; an interaction of the dyslipidemia with other generally consistent with the double-blind period. disease markers needs to be investigated. Conclusions doi:10.1016/j.jns.2019.10.1400 Switching from IFN to ocrelizumab at the start of the OLE provided rapid reductions in ARR, maintained throughout the 4-year follow-up. After 6 years’ follow-up, patients who initiated ocrelizumab 2 years earlier accrued significant, sustained reductions WCN19-1874 in disability progression compared with patients switching from IFN.

doi:10.1016/j.jns.2019.10.1401 Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications WCN19-1875

Long-term reduction of relapse rate and confirmed disability Poster shift 03 - Autonomic nervous system disorders / history of progression after 6 years of ocrelizumab treatment in patients with relapsing multiple sclerosis neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular G. Giovannonia, L. Kapposb, S.L. Hauserc, X. Montalbande,H. disorders and nutritional disorders or intoxications Koendgenf, K. Prajapatig, M. Manfrinif, L. Mehtah, J. Wolinskyi aQueen Mary University of London, Department of Neurology, London, Effects of liraglutide in the treatment of severe obesity in a young United Kingdom patient with parkinson’s disease bUniversity Hospital Basel- University of Basel, Department of Neurol- ogy, Basel, Switzerland M. Barichella, E. Cassani, V. Ferri, D. Vallauri, G. Pinelli, C. Pusani, R. c University of California, Department of Neurology, San Francisco, CA, Cilia, G. Pezzoli USA Parkinson Institute, ASST G. Pini - CTO, Milano, Italy dUniversity of Toronto, Division of Neurology, Toronto, ON, Canada e ’ Vall d Hebron University Hospital, Department of Neurology, Barcelona, Purpose Spain The Glucagon-like peptide-1 (GLP-1) analog exert disease-modi- f F. Hoffmann-La Roche Ltd, n/a, Basel, Switzerland; fying effects in patients with Parkinson’s disease (PD). Nonetheless, g GCE Solutions Inc., n/a, Amsterdam, The Netherlands the significant improvement in motor performance was paralleled by h Genentech Inc., n/a, South San Francisco, CA, USA significant reduction in body weight in exenatide-treated PD, so that ARTICLE IN PRESS

318 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx it could argued that levodopa pharmacokinetics could have been neurologic events are being increasing described in PAF patients, improved by either body weight loss or by other GLP-1-related. and some of these episodes don´t have a clear etiology althought TTR deposits seems do be the main precipitant factor; in some cases Methods there is EEG dysfunction and in others epiletiform activity. In this We describe a 42-year-old female Italian PD patient with a 2-year case, there where clinic an electrographic criterium for status history of PD, who presented a few month after the onset (October epileticus, which is why one more possible diagnosis to add patients 2017) with III class obesity (BMI 47 kg/m2). To assess the effects of with FAP and paroxysmal neurological changes, and from the body weight loss both on efﬁcacy of pharmacological treatment and authors' knowledge a rare diagnosis of these patients. on PD symptoms, the GLP-1 analog Liraglutide was prescribed for obesity (according to Italian guidelines) in adjunct to pramipexole doi:10.1016/j.jns.2019.10.1403 PR 1 mg/day and levodopa/benserazide 400mg/day. Plasma Levo- dopa levels were investigated.

Results WCN19-1883 The overall body weight loss of 29 kg (-21% , from 138 kg to 129 kg) was associated to a significant improvement in the motor disability was paralleled by a 2-fold increase in the area-under-the Poster shift 03 - Autonomic nervous system disorders / history of curve (AUC from 119 to 235 (ug/ml)*min and the Cmax (from 1.45 neurology /motor neuron disease / movement disorders / MS & to 2.98 ug/mL). demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications Conclusions The use GLP-1 analog Liraglutide is able to improve PD motor Efficacy and safety of ocrelizumab in patients with relapsing- disability in the short-term, likely because of optimized levodopa remitting multiple sclerosis with a suboptimal response to pharmacokinetics. It remains to be elucidated whether this is a previous disease-modifying therapies (1‑year interim results) consequence of either body weight loss or greater levodopa intestinal absorption or both. P. Vermerscha, J.P. Eralinnab, R. Nicholasc, C. Oreja-Guevarad, A. Sivae, B. Van Wijmeerschf, H. Wiendlg, R. Buffelsh, R. Kuheljh,W.Weih,G. doi:10.1016/j.jns.2019.10.1402 Comii aUniversity of Lille, Department of Neurology, Lille, France bNEO Research, Department of Neurology, Turku, Finland cImperial College Healthcare NHS Trust and Imperial College London, WCN19-1882 Department of Neurology, London, United Kingdom dHospital Clinico San Carlos, Department of Neurology, Madrid, Spain e Poster shift 03 - Autonomic nervous system disorders / history of Istanbul University, Cerrahpasa Faculty of Medicine, Cerrahpasa, Turkey neurology /motor neuron disease / movement disorders / MS & fUniversity Hasselt and Rehabilitation & MS-Centre, Department of demyelinating diseases / neuromuscular disorders / vestibular Neurology, Overpelt, Belgium disorders and nutritional disorders or intoxications gUniversity of Münster, Department of Neurology, Münster, Germany hF. Hoffmann-La Roche Ltd, na, Basel, Switzerland Paroxysmal neurological phenomena in patients with familial iVita-Salute San Raffaele University, Department of Neurology, Milan, amyloidotic polyneuropathy - Is there a need to broaden the Italy classification? Background S. França, C. Cruto The aim of the prospective, multicentre, single-arm Phase IIIb Unidade Local de Saúde de Matosinhos-Hospital de Pedro Hispano, CASTING study (NCT02861014) is to assess the efficacy/safety of Neurology, Matosinhos, Portugal ocrelizumab in patients with relapsing-remitting multiple sclerosis (RRMS) with a prior suboptimal response to disease-modifying In patients with familial amyloid polyneuropathy there is therapy (DMT). accumulation of transtirretin (TTR) in heart and nerve, but in the post- hepatic transplantation era, the TTR produced by the choroid Objective plexus plays an increasingly important in the etiology of the To report 1-year interim analyses of no evidence of disease symptoms of this patients. They ere described has presenting short activity (NEDA) in CASTING. transitory neurological events, including migraine, seizures like episodes, TIAs and “amyloid spells”. According to the one of Methods the largest series most of them seem to occur on average Patients (Expanded Disability Status Scale [EDSS] score ≤4.0; 14.6 years after disease onset and have usually minuts to hours disease duration ≤10 years; discontinued prior DMT of ≥6 months’ of duration. duration due to suboptimal disease control) received intravenous A 42 years-old male patient diagnosed with PAF, submitted to ocrelizumab 600 mg/24 weeks for 96 weeks. NEDA (absence of: liver transplantation presented with a motor aphasia and right protocol-defined relapses [PDR], 24-week confirmed disability hemiparesis. Brain CT was normal. In the day after developed a progression [24W-CDP], T1 gadolinium-enhancing or new/enlarging generalized tonic clonic seizure without recovery of the previous T2 lesions [rebaselined at Week 8]) was assessed every 24 weeks in level of consciousness. EEG revealed continuous focal epiletiform the modified intent-to-treat population (mITT; patients receiving activity. The diagnosis of status epilepticus was made and patient any dose who discontinued early without a protocol-defined event started on antiepileptic drug (AED) and 2 days after was seizure free were imputed as having an event if treatment discontinuation was and latter on discharged asymptomatic on AEDs.Transitory for lack of efficacy/death; others were excluded). ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 319

Results found a direct association between 25(OH) D and MMSE score A total of 680 patients (female, 64%; mean [SD] baseline EDSS (p=0.03) and an inverse association between 25(OH) D and age score, 2.1 [1.1]; disease duration, 5.0 [2.7] years; pretreated with one (pb0.01) and the Hoehn & Yahr stage (p=0.01). Plasma 25(OH) D DMT, n=411 [60.5%]; pretreated with two DMTs, n=265 [39.5%]) levels positively correlated with solar exposure (p = 0.04). were included in the interim mITT population. At Week 48, most patients had NEDA (n/N=586/674 [86.9%]). Rates of 24W-CDP Conclusion (4.8%), PDR (4.2%), T1 gadolinium-enhancing (2.8%) and new/ In a large PD cohort, low 25(OH) D levels correlated with older enlarging T2 lesions (6.2%) were low. The adjusted annualised age and with worse global cognitive function and disease severity. relapse rate was 0.048. Safety outcomes will be reported. Daily time of sun exposure greatly increased 25(OH) D levels. We strongly recommend screening PD patients for 25(OH) D plasma Conclusions levels and -in case of deﬁciency- ensuring oral supplementation to Most patients in CASTING had NEDA. Future CASTING data will reduce the risk of cognitive dysfunction and motor disability. describe additional efﬁcacy/safety results of ocrelizumab treatment in patients who had ongoing disease activity while receiving another doi:10.1016/j.jns.2019.10.1405 DMT. doi:10.1016/j.jns.2019.10.1404 WCN19-1897

Poster shift 03 - Autonomic nervous system disorders / history of WCN19-1884 neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Poster shift 03 - Autonomic nervous system disorders / history of disorders and nutritional disorders or intoxications neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Acute onset ocular myasthenia gravis disorders and nutritional disorders or intoxications G. Akdag, F. Akkoyun Arıkan, A. Bardakci, M. Cetiner, S. Canbaz Kabay Vitamin 25(OH) D and cognitive function in parkinson’s disease Kutahya University of Health Sciences, Neurology, Kutahya, Turkey

a a b a a a M. Barichella , C. Bolliri , G. Pinelli , L. Iorio , V. Ferri , E. Cassani ,A. Introduction a a a a a a Giana , S. Caronni , C. Pusani , F. Del Sorbo , R. Cilia , E. Quacci ,G. Involvement of the eye muscles in myasthenia gravis (MG) is not c c d a Riboldazzi , S. Lanfranchi , E. Cereda , G. Pezzoli uncommon and is characterized by a fluctuating involvement that a Parkinson Institute- ASST G.Pini-CTO, ASST G.Pini-CTO, Milan, Italy does not conform to cranial nerve involvement. bDipartimento Riabilitazione Malattia di Parkinson e Disturbi del Movimento- Ospedale Classificato Moriggia Pelascini- Gravedona, Case Milan, Italy A 54-year-old male patient was admitted to the emergency c U.S. Riabilitazione Parkinson- Fondazione Gaetano e Piera Borghi di department with a sudden onset of double vision. In his neurological Brebbia, Fondazione Gaetano e Piera Borghi, Brebbia, Italy examination; he was conscious, cooperative, oriented, pupils were d Clinical Nutrition and Dietetics Unit- Fondazione IRCCS Policlinico San isochoric and ptosis was present in the right eye. The inward, up- Matteo- Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy down gaze of the right eye was fully restricted, and the lateral view was minimally restricted. The other examination findings were Objective normal. Her past medical history revealed diabetes mellitus and he The role of vitamin D is gaining attention in neurodegenerative was taking oral antidiabetic medication. Neuroimaging(Contrast) diseases, as low serum concentrations have been consistently found and venography, repetetive nerve stimulation (RNS) tests were in both Alzheimer’s and Parkinson’s disease (PD). This study aims to performed. In the RNS test, a decrement response was detected. correlate 25(OH) D plasma levels with global cognitive function and Pridostigmine and IVIG(0.4 gr kg/day) were started with the severity of motor function in PD. diagnosis of MG. Thoracic imaging and acetylcholine receptor antibody examination were requested. Oral steroid was added to Methods the treatment with dense blood glucose monitoring. The patient is 350 patients with idiopathic PD (66% male, mean age 70.5 yr followed up with a complete recovery in his clinic. (range 60-87), mean disease duration 9.9±5.7 yr) were consecutively recruited at a tertiary referral clinic during an 8-month period. Conclusion We correlated plasma 25(OH) D levels with demographic and MG, diabetic cranial neuropathy, cavernous sinus diseases, clinical data, including PD severity (Hoehn & Yahr stage) and global aneurysm, brain stem lesions can be presented with unilateral cognitive function (Mini Mental State Examination, MMSE) . In ophthalmoparesis. In the differential diagnosis, initial findings, addition, Plasma 25(OH) D levels were correlated with sun exposure pupillary involvement, presence of pain and fluctuations are (hours/day). clinically important clues. In spite of this, it is important not to neglect the diagnostic tests in terms of differential diagnosis. Results Overall, PD patients showed 25(OH) D deficiency (mean level doi:10.1016/j.jns.2019.10.1406 17.1 ± 8.34 ng/ml). Using simple linear correlation analysis, we ARTICLE IN PRESS

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WCN19-1899 WCN19-1903

Poster shift 03 - Autonomic nervous system disorders / history of Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications disorders and nutritional disorders or intoxications

Using functional status to aid interpretation of the composite Pregnancy outcomes in patients treated with ocrelizumab unified huntington’s disease rating scale (cUHDRS) scores in patients with huntington’s disease (HD) C. Oreja-Guevaraa, S. Wrayb, R. Buffelsc, D. Zecevicc, S. Vukusicd aHospital Clinico San Carlos, Department of Neurology, Madrid, Spain D. Trundella, G. Palermob, J.D. Longc, B.R. Leavittd, S.A. Schobele, S.J. bSibyl Wray MD Neurology PC, n/a, Knoxville- TN, USA Tabrizif cF. Hoffmann-La Roche Ltd, n/a, Basel, Switzerland aRoche Products Ltd., Outcomes Research, Welwyn Garden City, United dService de Neurologie et Sclérose en Plaques- Fondation EDMUS pour la Kingdom Sclérose en Plaques- Hôpital Neurologique Pierre Wertheimer, Depart- bF. Hoffmann-La Roche Ltd, Pharmaceutical Division, Basel, Switzerland ment of Neurology, Lyon, France cUniversity of Iowa, Department of Psychiatry, Iowa City, USA d University of British Columbia, Centre for Molecular Background Medicine and Therapeutics, Department of Medical Genetics, Vancouver, Ocrelizumab, an approved treatment for relapsing and primary Canada progressive forms of multiple sclerosis (MS), was also studied in e F. Hoffmann-La Roche Ltd, Product Development Neuroscience, Basel, rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Switzerland As many patients with MS are women of reproductive age, f University College London, UCL Huntington’s Disease Centre, UCL Queen pregnancy outcomes in ocrelizumab-exposed patients are important. Square Institute of Neurology, London, United Kingdom Objective The cUHDRS is a novel scoring algorithm that provides a To report updated pregnancy outcomes in women receiving relatively holistic and sensitive outcome assessment of cognitive, ocrelizumab in clinical trials/post-marketing setting. motor and functional impairment in early to moderate manifest HD. It combines the Total Functional Capacity (TFC), Total Motor Score, Methods Symbol Digit Modalities Test and Stroop Word Reading measures Analyses use clinical trial/post-marketing sources to 31/03/2019. into a single metric. We aimed to enhance understanding of cUHDRS Contraception throughout and up to 12 months after the last scoring by linking to established measures of meaningful daily ocrelizumab infusion is recommended in the EU; trials required use function and independence. of two contraceptive methods until 48 weeks after the last Data from Enroll-HD were evaluated. For patients meeting the ocrelizumab infusion/until B-cell repletion (whichever longer). reference population for the cUHDRS (manifest HD, TFC 5–13, ≥20 Foetal in utero ocrelizumab exposure was defined as an infusion years; N=3,490), the cUHDRS score range was calculated. Patients within 3 months of contraception or during pregnancy or if the date were divided into groups around each integer score (e.g. for was unknown. cUHDRS=11: 10.5≤cUHDRS scoreb11.5). For each grouping, the mean HD stage, Independence Scale (IS), mean Functional Assess- Results ment (FA) and number of FA items that ≥50% of individuals can A total of 362 ocrelizumab-exposed pregnancies (MS, N=267; RA achieve were calculated. or SLE, N=33; no reported indication, N=62) have been reported up A figure was created to aid interpretation of cUHDRS scores by to 31/03/2019. Of these, 267 were patients with MS (trials, n=78; linking to measures of functional ability in patients with HD. In post-marketing, n=189); 118 were considered to have foetal the early to moderate manifest HD population, cUHDRS exposure (no foetal exposure, n=47; foetal exposure unknown, score groupings ranged 3–18 (N=3,484; six individuals with n=102). Preliminary outcomes of the 267 pregnancies in women cUHDRS=2 were excluded from subsequent analyses). As cUHDRS with MS exposed to ocrelizumab at cut-off include 62 live births, 86 scores declined, the number of FA items achievable and the level ongoing pregnancies, 25 elective abortions, 10 spontaneous abor- of independence declined. For patients in the cUHDRS=3 tions, 1 stillbirth, 3 ectopic pregnancies, 22 lost to follow-up and 58 group, only 12 FA items were achievable by ≥50% and the mean unknown/outcome not reported. IS=65. cUHDRS scores reflect the differing levels of function in Conclusions individuals with early to moderate manifest HD and can better Reviewed cases are not indicative of an ocrelizumab-related differentiate between individuals with Stage I HD than commonly increased risk of adverse pregnancy outcomes, including spontane- used measures of function. ous abortions or malformations. Data remain consistent with prior reports and continue to be collected/assessed. doi:10.1016/j.jns.2019.10.1407 doi:10.1016/j.jns.2019.10.1408 ARTICLE IN PRESS

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WCN19-1911 K.S. Moalla, S. Sakka, N. Farhat, O. Hdiji, S. Daoud, H. Haj Kacem, M. Damak, C. Mhiri Habib Bourguiba Hospital, Neurology, Sfax, Tunisia Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & Introduction demyelinating diseases / neuromuscular disorders / vestibular Osteomalacia is an uncommon cause of muscle weakness. The disorders and nutritional disorders or intoxications course and manifestations of this myopathy are variable. Vitamin D deficiency is an important treatable cause of osteomalacic myopathy. The Roche HD natural history study – An external comparator by design Methods We conducted a search in the database of our department to G. Hoopera, G. Palermob, F. Hlavacc, C. Finnegand, E. Fricke,L. identify patients with osteomalacia and myopathy. Their medical BoakfThe Natural History Study Investigators records were reviewed retrospectively. , R. Doodyg, S. Schobelf, B. Leavitth aRoche Products Ltd, Neuroscience-Huntington's Disease, Welwyn Results Garden City, United Kingdom Two women aged of 43 and 22 years, with a past history of bF. Hoffmann-La Roche Ltd., Product Development, Basel, Switzerland thyroid surgery for 6 years and fracture in the humerus respectively, c fi F. Hoffmann-La Roche Ltd, Product Development Global, Basel, Switzerland were identi ed. They developed respectively since one and three d fi Almac Group, Almac Clinical Technologies LLC, Souderton, USA years lower limbs heaviness with dif culties to walk and climb stairs. e fi F. Hoffmann-La Roche Ltd., PD Clinical Operations, Basel, Switzerland; Physical examination disclosed proximal muscle de cit. Deep tendon f fl F. Hoffmann-La Roche Ltd., Product Development Neuroscience, Basel, re exes, plantar responses Switzerland and sensory system were normal. Serum muscle enzymes and gF. Hoffmann-La Roche Ltd and Genentech Inc., Neurodegeneration, South electroneuromyogram were normal. Complete blood tests showed a San Francisco- USA, and Basel, Switzerland hypocalcaemia with hypophosphatemia. The FT4 and TSH rate were hCentre for Molecular Medicine and Therapeutics, Department of Medical normal. Parathyroid hormone assay showed high levels with low Genetics, University of British Columbia, Vancouver, BC, Canada serum vitamin D in both cases. X-ray of the spine and the pelvis showed respectively patchy osteolytic areas and small fractures of The Roche Huntington’s disease (HD) Natural History Study the ischio-pubic branches. Cervical ultrasound was normal. There was (NHS) (NCT03664804) is a prospective, longitudinal cohort study a clear amelioration of muscle strength under calcium and vitamin D designed to provide further clinical validation of mutant huntingtin supplementation. protein (mHTT) as a prognostic biomarker of disease outcomes in early stages of manifest HD. The NHS will also be used as an external Conclusion fi comparator group for the ongoing HD open-label extension (OLE) of Vitamin D de ciency is a cause of osteomalacia in adults. the Phase I/IIa study (NCT03342053). The study designs are Myopathy are uncommon and in rare cases, it reveals the disease. comparable in terms of clinical, digital clinical, fluid biomarker and Women are more often affected than men. Screening for vitamin D fi imaging outcomes. Length of follow-up and assessment schedule for de ciency in patients with acquired myopathy is needed to identify fi measures common to both studies are identical. this treatable disorder. Vitamin D supplementation allows signi cant Up to 100 Stage I or II HD individuals are being enrolled and improvement in the physical symptoms. matched on parameters of disease Stage (I or II), age (+/- 5 years), sex and CAG repeat length (+/- 2), and followed prospectively in the doi:10.1016/j.jns.2019.10.1410 15-month study, with key outcomes assessed at baseline, 3, 9 and 15 months. A matching algorithm has been developed using the Almac IxRS system to facilitate enrolment of "matched" patients by constantly updating the list of unmatched OLE patients. The comparability between the two cohorts, and baseline WCN19-1929 symptom severity on key outcomes of the Unified HD Rating Scale (UHDRS) and the composite UHDRS, will be presented. Poster shift 03 - Autonomic nervous system disorders / history of This study will provide important longitudinal data on the relation- neurology /motor neuron disease / movement disorders / MS & ship between mHTT and measures of HD disease progression. A demyelinating diseases / neuromuscular disorders / vestibular comparison of outcome data between the NHS and OLE studies will allow consideration of RG6042 effects versus natural disease progression. disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1409 Clinical, electrophysiological and MRI profile of Hirayama disease: A case series

R. Paudela, J. Khan Ahmadb, B. Bijukaccheb, P. Niraulaa a WCN19-1914 Grande International Hospital, Neurology, Kathmandu, Nepal bGrande International Hospital, Orthopedics and Spinal Surgery, Kathmandu, Nepal Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & Introduction demyelinating diseases / neuromuscular disorders / vestibular Hirayama disease (HD) is a focal amyotrophy of the young adults disorders and nutritional disorders or intoxications involving the distal upper limbs. It is often misdiagnosed as motor neuron disease and writer’s cramp. This often leads to delay in the Osteomalacia and myopathy diagnosis of the disease . ARTICLE IN PRESS

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Materials and methods Patients presenting with insidious onset of hand wasting from January 2014 to February 2017 were evaluated clinically and electro physiologically. Cervical dynamic MRI in neutral position and in ﬂexion was done.

Results There were a total of 17 male patients.with mean age of 22 years (range 18–31 years). Duration of illness was three months to seven years. All patients presented progressive weakness and lower motor neuron type of wasting of hands. Nine (52.9%) patients had right sided involvement, six (35.3%) patients had left sided involvement and two (11.7%) patients presented with bilateral involvement.. In MRI, localized lower cervical cord atrophy was seen in 14 (82.3%) cases and asymmetric cord flattening was in 14 (82.3%) cases. Loss of dural attachment was noted in 14 (82.3%) cases, anterior displace- ment of dorsal dura on flexion in 15 (88.2%) cases and epidural flow voids in 15 (88.2%) cases. Enhancing epidural crescent in flexion was seen in 12 (70.5%) cases. Intramedullary hyper intensity was the least common finding and was seen in only 2 (11.7%) cases.

Conclusion Clinical features of HD corroborated well with electrophysiolog- Radiographs of the cervical spine showed bony erosion of the ical diagnosis of anterior horn cell disease of lower cervical cord. right lateral part of C4 vertebral body and foramina. Since dynamic contrast MRI is characteristic, it should be ordered in CT of the lumbar s/o soft-tissue mass in the right C4 paravertebral all suspected cases.. region, extended through the intervertebral foramen, resulting in the bony erosion and obliteration of the epidural fat. doi:10.1016/j.jns.2019.10.1411 DSA s/o AVM Patient underwent embolization and there was complete resolution of symptoms.

WCN19-1940 Discussion It is very important to diagnose this potentially treatable condition Poster shift 03 - Autonomic nervous system disorders / history of I have obtained patient and/or Institutional Review Board (IRB) neurology /motor neuron disease / movement disorders / MS & approval, as necessary. demyelinating diseases / neuromuscular disorders / vestibular An Institutional Review Board (IRB) and/or Animal Use Commit- disorders and nutritional disorders or intoxications tee have waived the requirement for their formal approval of the study. A rare case of extradural arterio-venous malformation presenting as cervical radiculopathy doi:10.1016/j.jns.2019.10.1412

N. Kadam, S. Khadilkar, P. Ojha, G. Soni, B. Patel Grant Medical College and Sir JJ Group of Hospitals, Neurology, Mumbai, India WCN19-1943

Arteriovenous malformations (AVMs) are the most common Poster shift 03 - Autonomic nervous system disorders / history of spinal vascular anomaly majorly being intradural . Their presentation neurology /motor neuron disease / movement disorders / MS & in the extradural location and at the cervical cord region is rare. An demyelinating diseases / neuromuscular disorders / vestibular accurate diagnosis is important as these lesions are reversible cause disorders and nutritional disorders or intoxications of neurological dysfunction. We report an unusual AVM that was fed by the cervical arteries and was located totally in the extradural space, resulting in right cervical radiculopathy/ brachial plexopathy. Case-control study to determine the feasibility of clinical study for diagnosis of dysautonomia Case a a a A 58-year-old man experienced acute onset painful paraesthesia J.A. Nader Kawachi , Y.C. Pino Peña , M.D.L.L. Andrade Magdaleno ,J. b b on the right side of the neck radiating to right upper limb with right Lopez Estrada , P. Manrique Mirón a upper limb proximal weakness. He was unable to abduct his right Medica Sur Hospital, Neurology, Mexico City, Mexico b arm above the shoulder. Institute of Mathematics, National Autonomous University of México, There was marked wasting in right deltoid with pronounced Mathematics, Cuernavaca, Morelos, Mexico weakness in right deltoid, trapezius, infraspinatus, teres minor & mild weakness in right pectoral, Biceps & brachioradialis. Power in We propose a new test to evaluate dysautonomia, Multimodal forearm and hand muscles were normal on the same side. Monitoring for Diagnosis of Dysautonomia (MMDD), which consists Generalized hyporeﬂexia. Sensory: Normal.On auscultation: bruit in of recording by digital monitoring 11 variables of the cardio- right cervical region. cerebrovascular system during a free mobility protocol. ARTICLE IN PRESS

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The subject performs nine two-minute stages:1,3,5,7,9, laying Methods down and 2,4,6,8, sitting horizontal feet, sitting feet hanging, In this retrospective cohort, patients with MG referred to the standing and 15 genuflections, respectively. MCA velocity, ECG, neuromuscular clinic of Shariati Hospital were enrolled.HLA DRB1, Systolic-velocity(Peak), Mean-velocity(Mean), End-Diastolic-Veloc- DQA1 and DQB1 typing was performed by polymerase chain reaction ity(EDV), Pulsatile-Index(PI), Resistance-Index(RI), Diastolic/ based on sequence-specific primers. Recommendations of the MGFA Systolic-index(S/D), Systolic-blood-pressure(Tas), Diastolic-blood- task force were used to determine disease severity and response to pressure(Tad) and Heart-Rate(HR) are always recorded. treatment. We select 21 patients suffering syncope and 21 volunteers tested with MMDD. The presence of syncope defined the groups. Results To understand the significance of change between each stage, we In 146 patients with MG, 15 different alleles of DRB1, 7 alleles of used logistic regression (certain subgroup of variables) and bootstrap. DQA1, 9 alleles of DQB1 and 19 haplotypes were identified. Crude p- The sign value of the coefficients of regression and their p-value were values were significant for HLA DRB1 07* DQA1 *0201 and their recorded. When at least 80% of the results coincide sign and p-value haplotype 07/0201/0201 in relationship to maximum disease severity. below 0.1, they are considered. The sign determines the change of DRB1 *1101*0301*1601, DQA1 *0501*0102 and their haplotypes 16/ cardio-cerebrovascular variables, which might characterize syncope. 0102/0501 and 11/0501/0301 needed less intense treatment options. DQA1 *0501 and the associated haplotype 11/0501/0301 needed less Results immunosuppressive. DRB1*1501 DQA1*0103 *0104, DQB1 *0601 and 21 patients having syncope(16F/6M, mean age 43.5) and 21 15/0103/0601 haplotype were related to poorer disease control. control subjects (15F/7M, mean age 45.1). Adjusted p-values did not show any significant association between The main changes indicating syncope:(Tad, Tas) both increase these loci, disease severity and treatment options. between stages 7-8. (HR,Tas,RI) HR decreases, Tas increases at 5-6. (Peak,Mean,EDV) Peak increases, Mean decreases, 4-5. (PAM,Mean, Conclusion SD) PAM increases at 1-2, decreases at 2-3, increases at 3-4, While the small sample size might have imposed a limitation, decreases at 4-5. (Tas,Peak,PI) Tas increases at 1-2, decreases at larger studies are needed to establish the relationship in certain 2-3, increases at 3-4, decreases at 4-5. (Tas,Peak,PI) PI decreases subgroups of patients particularly those receiving biologics. at 5-6, increases at 6-7. (HR,Tas,RI) HR decreases at 5-6, increases at 5-6. doi:10.1016/j.jns.2019.10.1414 MMDD can detect subtle changes in the autonomic nervous system and distinguish people who suffered a syncope. suffered a syncope. WCN19-1945 doi:10.1016/j.jns.2019.10.1413

Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & WCN19-1944 demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications

Poster shift 03 - Autonomic nervous system disorders / history of Disease modifying therapy of multiple sclerosis with neurology /motor neuron disease / movement disorders / MS & natalizumab: A nine years experience of MS clinic in the Eastern demyelinating diseases / neuromuscular disorders / vestibular region of Abu Dhabi Emirate, UAE disorders and nutritional disorders or intoxications A. Hassana, H. Elhasina, N. Solimana, B. Beiramb, M. Szolicsa HLA class II polymorphism contribution to disease severity and aTawam Hopsital, Neurology, Al Ain, United Arab Emirates response to treatment in myasthenia gravis bTawam Hopsital, Pharmacy, Al Ain, United Arab Emirates

a b b c b F. Sinaei , S. Nafissi , F. Fatehi , S. Oveis Gharan , S. Ehsan ,K. Background d e Kamali , M. Mahmoudi The prevalence of multiple sclerosis (MS) in the Gulf countries a Shariati Hospital- Tehran University of Medical Sciences, Neurology, and particularly in UAE clearly had been increased. Natalizumab is a Tehran, Iran; powerful, second line disease modifying treatment (DMT) of MS due b Tehran University of Medical Sciences, Neurology, Tehran, Iran; to high relapse rate reduction. cRush University Medical Center, Alzheimer Research Center, Tehran, Iran; Aim d Zanjan University of Medical Sciences, Public Health, Zanjan, Iran; To assess the Natalizumab efficacy and safety in cohort of MS e Tehran University of Medical Sciences, Rheumatology Research Center, patients treated in Eastern region of Abu Dhabi Emirate. Tehran, Iran Methods Background Retrospective charts review of MS patients at Tawam hospital As many autoimmune disorders, myasthenia gravis (MG) is the from January 2010 till May 2019. result of genetic and environmental interactions. MHC class II polymorphism has well-known predisposing effects. It is also related Results to disease severity and response to treatment in many autoimmune They were 34 females and 20 males, mean age 33 years, received disorders. Previous studies have shown the role of MHC class II alleles Natalizumab in Tawam hospital. Natalizumab was a first DMT for in the pathogenesis of MG. However, data on the relationship of these twelve patients, while others used from 1 to 4 DMT before HLA alleles to disease severity and treatment responses are lacking. Natalizumab. Six patients had positive JCV antibodies before starting ARTICLE IN PRESS

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Natalizumab. The number of infusions ranged from 1 to 104 (an WCN19-1953 average 34). Annualized relapse rate before starting the drug was 1.12 (0-4) which decreased to 0.032 on Natalizumab. Brain MRI follow up revealed six patients (11.1%) who had new or active Poster shift 03 - Autonomic nervous system disorders / history of lesions after ﬁrst 6 months of treatment. The average EDSS remain neurology /motor neuron disease / movement disorders / MS & the same (2.66 before and after DMT), but in eleven patients (20.4%) demyelinating diseases / neuromuscular disorders / vestibular EDSS improvement ranged from 0.5 to 3.5 points. Five patients disorders and nutritional disorders or intoxications (9.3%) had a clinical MS relapse on Natalizumab and nine (16.7%) had a progression in EDSS (from 1 to 6 points), most of them Relationship between the working capacity level and the results progressed to SPMS (average EDSS before DMT was 4.3 and after of the brief ICF core set for multiple sclerosis 6.3). D. Valadkeviciene, R. Kizlaitiene, D. Jatuzis Conclusion Vilnius University, Department of Neurology and Neurosurgery, Institute ﬁ DMT with Natalizumab in our MS cohort revealed a high ef cacy of Clinical Medicine, Faculty of Medicine, Vilnius, Lithuania in patients who were carefully selected for treatment. Introduction doi:10.1016/j.jns.2019.10.1415 Multiple Sclerosis (MS) impairs levels of working capacity and causes substantial impact on personal quality of life as well as on the welfare of person’s family.

WCN19-1952 Purpose The purpose of the article is to analyse a hypothesis that patients fi Poster shift 03 - Autonomic nervous system disorders / history of with different working capacities faced different dif culties and barriers. neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Method disorders and nutritional disorders or intoxications 184 persons with MS were divided into groups by various levels of working capacity and were assessed at one centre by using Brief Magnetic resonance imaging findings of hemifasial spasm ICF Core Set for Multiple Sclerosis. The results of assessment were compared with said groups of working capacity levels. S. Canbaz Kabaya, S. Tok Umayb, G. Akdaga, O. Temela,F.Arıkana,M. Cetinera Results aKutahya University of Health Sciences, Neurology, Kutahya, Turkey The analysis of the dynamics of different categories during the bKutahya University of Health Sciences, Radiology, Kutahya, Turkey worsening of working capacity demonstrates that people in the lowest 0–25% category could be in a lower position by more than 1 Introduction row score comparing to those with higher working capacity. Those Hemifacial Spasm (HFS) is irregular, painless, clonic withdrawal with a working capacity of 0–25% indicated that d450 – Walking of one half of the face. In addition to medical treatment, surgical function was a severe difficulty, while those with the working treatment is applied according to the underlying cause. magnetic capacity of 30–40% indicated that it was a moderate difficulty (M = resonance imaging (MRI) is essential to show the offending vessel at 3.2 and M = 1.9 respectively). The same tendency is seen with b620 the origin of HFS and the absence of other causes of nerve – Urination functions (M =2.9 in the 0–25% working capacity group compression. and M = 1.8 in the 30–40% working capacity group) or with d850 – Remunerative employment (M = 2.8 and M = 1.6, respectively). Methods Twenty-five patients with HFS (17 female, 8 male) were Conclusions evaluated in our clinic. The higher the working capacity level of the individual, the fewer the impairment specified by patients in the assessment by ICF Results categories. The mean age of the patients was 56.4 years (32-78). While MRI of 17 patients was unremarkable, 4 patients had basilar artery doi:10.1016/j.jns.2019.10.1417 dolichoectasia, 2 patients had basilar artery indentation, 1 patient had cerebellopontin tumour and 1 patient had cerebellopontine arachnoid cyst. The patient with cerebellopontin mass lesion had undergone operation and 12 patients were treated with botulinum WCN19-1978 toxin.

Conclusion Poster shift 03 - Autonomic nervous system disorders/history of Imaging has first to exclude a secondary hemifacial spasm and neurology/motor neuron disease/movement disorders/MS & secondly to search for and characterize the responsible neuro- demyelinating diseases/neuromuscular disorders/vestibular dis- vascular conflict, including arterial dolichoectasia or mass lesions. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1416 A scrutinized reflection on multiple sclerosis in Middle East countries ARTICLE IN PRESS

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V. Jangida, G. Elumalaib, S. Elayarajac, A. Bhonslec Introduction aYoung Scientific Researcher, Neuroscience, Georgetown, Guyana Myasthenia gravis is an autoimmune disease caused by specific bTeam NeurON, College of Medicine, Texila American University, autoantibodies that induce dysfunction of neuromuscular transmis- Neuroscience, Georgetown, Guyana sion. The diagnostic procedure is the same for all patients and at any cCollege of Medicine, Texila American University, Neuroscience, George- age. The management starts with the diagnostic, which is often town, Guyana unduly delayed. Our study aims to assess this delay and the associated reasons. Introduction MS or Multiple Sclerosis is a chronic - progressive disease Methodology involving nerve cells sheath of the brain and spinal cord [1]. Recent This was a retrospective, cross-sectional and descriptive study studies observe the connotation between socioeconomic status (SES) over a four years' period from 2014–2017. Data were directly and disability outcomes and progression in MS. The association collected from myasthenic patients all coming from the in and between SES at MS onset sustained and confirmed Expanded outpatient data books of the Fann National University Hospital Disability Status Scale (EDSS) 6.0 and 4.0 and onset of secondary Center and Department of Pikine National Hospital. A hetero- progression of MS [2]. administered questionnaire was administrated.

Demographic details Results According to WHO, MS is rapidly increased in the Middle East The study included 18 myasthenic, of who 13 women with a ratio and North African countries. In 2015, it was observed that the of 2.6. With a mean age up to 32 years [14–60 years]. The average prevalence is 115.94 per 10 thousand population and the women time from onset to diagnosis was 24 months. The patients had were most affected. A mean age of MS onset is identified as 28.54 consulted an average of four times before the diagnosis of yrs. The most common pretentious type of MS in the Middle East myasthenia gravis. The dedicated hospital was attended by 78% of were relapsing remitting multiple sclerosis (RRMS) (87.8% in Iran) patients and the ophthalmology department was the first consulted [3]. in 13 (72%) patients. The mean age of onset was 28 years old. Ptosis was the first symptom in 56% of cases. Recent advancement According to the recent (2018) evidence, FDA accepted to re- Conclusion evaluate the Cladribine – an immunosuppressant drug for treatment Myasthenia gravis is an unknown affection by the common public of RRMS [4]. Oral fingolimod is identified as advanced disease and most of the practitioners. The ophthalmologist is often the first modifying therapy against MS, even for pediatric patients [6]. A resort. The medical practitioner at in and outpatient consultation current (2019) prospective study sought to investigate autologous must be aware of the main symptoms of myasthenia gravis. hematopoietic stem cell transplantation (AHSCT) as a therapeutic intervention in MS. Keywords: Myasthenia gravis, Therapeutic route, Dakar

Conclusion AHSCT is done to replace the body's immune system so that it no longer attacks the myelin or cause inﬂammation in a person's brain doi:10.1016/j.jns.2019.10.1419 and spinal cord. Our current study is mainly focus on the quantitative and qualitative analysis of AHSC therapy against MS.

Keywords: Multiple Sclerosis, RRMS, AHSCT WCN19-1989

Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1418 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-1983 Stroke medicine in two ancient tablets

Poster shift 03 - Autonomic nervous system disorders/history of S. Karim Azadi teaching hospital, Neurology and stroke, Duhok, Iraq neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Introduction orders and nutritional disorders or intoxications More than 6000 years ago, the cradle of civilization, Mesopotamia, grew-up in what is known today as Iraq. The history of cerebrovascular Therapeutic itinerary of the patients followed for myasthenia in diseases in Mesopotamia is insufﬁcient to supply scholar needs. Dakar Therefore, the goal of this review is to highlight some remarkable points in the history of stroke during the ancient Mesopotamian eras Y. Kabaa, D. Atsaa, C.C. Affognona, M. Bugemea, S. Mourabitb and to explore the knowledge and expertise of ancient healers.The aClinique neurosciences Ibrahima Pierre Ndiaye – CHNU Fann, neo-Sumerian period (2112–2004 BCE) documented, through clay medecine, Dakar, Senegal tablets, many medical records about two kinds of medical specialists; bCentre Hospitalier National de Pikine, medecine, Dakar, Senegal the āšipu (exorcists) and the ašu (physician-priests). ARTICLE IN PRESS

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Methods and findings evidence of neuronal injury, apoptosis and microglial activity. We The information herein was gathered through literature review used hematoxylin and eosin(H&E), glial fibrillary acidic protein using online resources, such as NCBI, Google Scholar, PubMed, UCLA, (GFAP), PCNA and Caspase III staining. and HİNARİ.Initially, most of the knowledge we have got was acquired mainly from two well-known transliterated cuneiform Results texts. Both tablets had clearly addressed stroke. One tablet, part of Using open field maze for testing locomotion, behavioral, anxiety the“diagnostic” series is currently in the Louvre Museum in Paris, and drug-induced neuromuscular disorders, the intake of bGH while the other one is in the British Museum in London and is part of significantly decreased walking distance, increased anxiety-like the “therapeutic” series. The ašu and the āšipu demonstrated an symptoms, increased freezing time and the number of defecation observational knowledge of anatomy and but no knowledge of the boils. nervous system, the concept of pathology, or physiology as we call Regarding the histological assessment, the bGH group showed them today. Physical treatment was mentioned to the patients. neuronal damage, more neuronal apoptosis (Caspase III staining), more astrocyte activation (more GFAP staining) and less glial Conclusion proliferation (PCNA). This descriptive review tells us that the history of stroke in the medical practice was well-encountered in the first half of the second Conclusion millennium BCE and that physicians were keen observers to describe The use of bodybuilding drugs especially bovine growth hormone stroke presentation and prognosis. has neurodegenerative changes both clinically and histologically.

Keywords: Mesopotamia, stroke, cerebrovascular disease, history of doi:10.1016/j.jns.2019.10.1421 medicine

doi:10.1016/j.jns.2019.10.1420 WCN19-1994

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-1992 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Neuro expressionism in Avicenna contributions demyelinating diseases/neuromuscular disorders/vestibular dis- A.I. Fumagalli orders and nutritional disorders or intoxications Sanatorio Parque, Neurology, Rosario, Argentina

The neurodegenerative effect of anabolic drugs (Bovine growth Avicenna (Abu-Ali al Husayn ibn Abdalla Ibn Sina, 979–1037 A.D.) hormone); Case report and animal study was among the leading figures during medieval ages and influenced the upcoming periods in the Eastern and Western hemispheres for H. Gabra, N.A. Kishkb, A.M. ShamsEldeenc, A. Sabbahb, H. Rizkb, A.M. a a long time. Ibn Sina wrote more than 450 treatises on medicine and Hamaad , N.G. Metwally fi a his Canon of Medicine became a main reference work. The rst Faculty of Medicine, Cairo University, Clinical pathology, Cairo, Egypt medical study of facial paralysis is attributed to him. He was the first bFaculty of Medicine, Cairo University, Neurology, Cairo, Egypt c to record differences between central and peripheral facial paralysis. Faculty of Medicine, Cairo University, Physiology, Cairo, Egypt Avicenna's neuro-expressionism contributions were epilepsy in children and adults, stroke, vertigo, wry mouth, tremor, meningitis, Background amnesia and dementia, head injuries and traumas, hysteria and We still have little knowledge about what triggers the immune conversion disorder, fainting and stupor, nervous tic, love sickness, changes leading to the development of multiple sclerosis. A 21-year delusion and hallucination, insomnia, nightmare, mania and psycho- old body-building male developed multiple sclerosis after regular sis, melancholia, paranoia, asthenia, hydrocephalus, sciatica and consumption of bovine growth hormone. pharmacological approach to memory enhancement. Many later scholars applied the scientific method of Avicenna in their work. Aim Medicine, despite its penchant for specialism, has always been To investigate the role of the bovine growth hormone (bGH) in responsive to polymathy. Avicenna, the Persian physician from the developing MS-like syndrome. we made an experimental model to 11th-century Islamic golden age, wrote on astronomy, geography, understand the mechanistic insight into functional and histological mathematics, physics, and philosophy, as well as works of poetry. His effect of bGH on the nervous system. legacy shows expressionism, like in art and serve as inspiration for modern colleagues in the field of neuroscience. The Arabic-Islamic Methods world added much to earlier achievements in neurology. I saw Twelve adult male rats were included. They were randomly convex equatorial deserts and each one of their grains of sand, The subdivided into two groups. Group 1; growth hormone (GH) group: Aleph, Jorge Luis Borges rats were supplied with GH at a dose of 0.54 mg. Group 2; the control group. Rats were assessed regarding motor power, coordinated gate, locomotion, anxiety and body weight at baseline and 6 weeks after doi:10.1016/j.jns.2019.10.1422 intake of bGH. After that, rat brains were histologically examined for ARTICLE IN PRESS

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WCN19-1998 Dysgeusia, as a non-motor symptom of PD, has not been established as strongly as olfactory dysfunctions have. Multiple studies done in the past state that dysgeusia has been noted Poster shift 03 - Autonomic nervous system disorders/history of uniformly in PD patients, but certain studies also state that taste neurology/motor neuron disease/movement disorders/MS & acuity is seen to be enhanced in these patients. Such patients demyelinating diseases/neuromuscular disorders/vestibular dis- experienced a more intense response to gustatory stimuli of low orders and nutritional disorders or intoxications concentration as compared to control groups. Three studies have presented statistically significant results to support that taste Gustatory neural structural connectivity in Parkinson’s disease impairments and disease progression are associated. However, its progression consistency and clinical utility remains under scrutiny. This encouraged us to conduct a comparative study of neural connections of the H. Chatterjeea,G.Elumalaib,P.Maitic,N.Osakwea,N.Sewrama, PGC and regions associated with conveying and processing of G. Vinodhanada gustatory stimuli between a control group and PD affected group aTeam NeurON - Texila American University, College of Medicine, and between the progressive stages of PD. fi Georgetown, Guyana Using bre tractography on structural MRIs, we observed the fi bTeam NeurON, Texila American University, Neuroscience, George- number, length and volume of bres connecting PGC with other town, Guyana regions. Comparisons were made among 4 groups, each group cSaginaw Valley State University, Brain Research Laboratory, containing 40 MRIs obtained from different individuals, one control Neuroscience, Michigan, USA group and three PD affected groups of spaced stages of progression. From the results observed, we may derive that while there is significant decrease in the fibres in PD affected group and we may The objective of this study is to analyze the consistency of neural conclude that PD does affect gustatory functions, there is very little connections of the primary gustatory cortex (PGC), ranging from decline in fibres from initial stages to advanced stages. control group to progressive stages of Parkinson’s Disease (PD). ARTICLE IN PRESS

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doi:10.1016/j.jns.2019.10.1423 for DM1.The endocrine function was assessed by measuring the concentration of hormones and metabolites in blood.

Results WCN19-2000 16 patients(9 female, 7 male). The mean age was 37 years. All this patients have DM1 genetically conﬁrmed. The endocrine system disorders were found in 50%. Abnormal thyroid-stimulating hormone Poster shift 03 - Autonomic nervous system disorders/history of values were found in 18.75%. Hyperprolactinemia was found in neurology/motor neuron disease/movement disorders/MS & 6.25%. The gonadotropic axis was affected in 25% with demyelinating diseases/neuromuscular disorders/vestibular dis- increased luteinizing hormone levels in 12.5%, sexuel impotence in orders and nutritional disorders or intoxications 6.25% and testicular atrophy in 6.25% with normal testosterone levels. Diabetes was found in 31.25% and abnormal lipid proﬁl was Myotonic dystrophy type 1 and endocrine system involvement found in 6.25%.

Conclusion E. Kacem, S. Sakka, N. Farhat, O. Hdiji, H. Hajkacem, M. Dammak, C. Patients with DM1 have an increased incidence of endocrine Mhiri dysfunctions. Several studies showed correlation between CTG(n) Habib bourguiba hospital, Neurology, Sfax, Tunisia expansion size and the endocrine system involvement.These abnormalities increase furthermore over time and affect the course Introduction of the disease as well as the quality of life of this young Myotonic dystrophy type 1 (DM1) is a multisystem disorder that population. Thus, hormonal concentration should be monitored affects muscles, eyes, heart and endocrine system. The objective of periodically in order to minimize the complications both organic this study is to describe the endocrine disorders. and psychological.

Methods We conducted a descriptive study, including patients followed-up doi:10.1016/j.jns.2019.10.1424 in the department of Neurology of habib bourguiba hospital in Sfax ARTICLE IN PRESS

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WCN19-2003 cNational Hospital Organization Kanazawa Medical Center, Pathology and Laboratory Medicine, Kanazawa, Japan

Poster shift 03 - Autonomic nervous system disorders/history of Objective neurology/motor neuron disease/movement disorders/MS & We performed retrospective hospital-based post-mortem study demyelinating diseases/neuromuscular disorders/vestibular dis- to clarify the clinical features and pulmonary pathology of amyotro- orders and nutritional disorders or intoxications phic lateral sclerosis (ALS) patients with non-invasive ventilation (NIV) support. Efficacy of Natalizumab in multiple sclerosis patients with high disease activity: Casablanca neurology department's experience Patients and methods To elucidate the clinical features and lung pathology of ALS with R. Amzil, V. Fotso, S. Sabiry, A. Bennis, Y. Habtany, F. Mouni, Z. Attar, NIV support (ALS-NIV), we retrospectively reviewed medical records S. Bellakhdar, H. El Otmani, B. El Moutawakil, M.A. Rafai of 96 consecutive autopsied patients between 2008 and 2016. Ibn Rochd University Hospital, Neurology - Neurophysiology, Casa- Pathological diagnosis was confirmed when the following two blanca, Morocco histopathological features were observed. First of them was systematic both upper and lower motor neuronal degeneration, and second Natalizumab (NTZ) as a high-cost long-term disease-modifying was the existence of TDP-43 inclusion body pathology and/or Bunina treatment indicated as a basic mono-therapy in patients with active bodies. relapsing-remitting multiple sclerosis (RRMS) has provided clinical efficiency. We evaluated clinical and radiological features and Results fi outcome of our NTZ MS patients. All RRMS patients defined by the From 96 autopsied cases, we identi ed 8 ALS-NIV patients. 2010 McDonald criteria who received Natalizumab (NTZ) treatment Average age of death was 66.6 y.o. and mean survival time was any time since 2013. Variables analysed included age, gender, disease 57.4 months. According to initial symptom, 4 cases were upper limb duration, MRI, Expanded Disability Status Scale (EDSS), previous onset, 3 cases were bulbar onset, and a case was lower limb onset. treatment, relapse rate before and after receiving NTZ, adverse Age of onset ranged from 50 to 77 years old, and age of death ranged events, complications, and reasons of discontinuation. Of 16 patients from 52 to 80 years old. Right lung weight ranged from 270 g to 935 in our study, the female-to-male ratio was 2:1. The mean age was 33 g, and left lung weight were from 300 g to 870 g. Seven out of 8 ALS- years, mean disease duration was 12 years with a range of (2–25). At NIV patients had bronchopneumonia, and 5 of these 7 patients had the time of this analysis, 14 patients had received 1 year of NTZ. severe bronchopneumonia. Lung abscess were found in 1 case. Approximately 80% of these patients were relapse-free after 1 year. The relapse rate after treatment was significantly lower (0,13 Vs 4,5). Conclusion Fifteen patients haveN9 T2 lesions at the beginning of treatment with From this institute-based retrospective study, NIV support could fi only 7 patients that had Gd-enhancing lesions on MRI, 87% had improve prognosis of ALS patients. However, it would be dif cult to achieved radiological NEDA (No evidence disease activity). We didn't avoid bronchopneumonia in the end stage of ALS-NIV. Pulmonary notice any opportunistic infections such as progressive multifocal pathology which we showed by this post-mortem study could be fi leukoencephalopathy (PML), or any adverse events. However, useful ndings to improve respiratory management of ALS-NIV. treatment was discontinued in 2 cases because of pregnancy in 1 doi:10.1016/j.jns.2019.10.1426 patient and high positive JC virus serology with a high risk of PML in the other. NTZ significantly reduces progression of disability, occurrence of clinical relapse and formation of new lesions at MRI in our MS patients. However, PML increasing risk require a longer- term follow-up. WCN19-2008 doi:10.1016/j.jns.2019.10.1425 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- WCN19-2006 orders and nutritional disorders or intoxications

Guillain Barré syndrome with dysautonomia and dysglycemia as a Poster shift 03 - Autonomic nervous system disorders/history of manifestation of the Chikungunya virus infection neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- K. Lebeisa, R. Brederb, C. Bittar Braunec, M. Beiral Hämmerleb, T. Lins orders and nutritional disorders or intoxications de Mirandab, M. Sinclair Haynes de Menezesa, F. Ramon Canale Ferreirab a Pulmonary pathology of amyotrophic lateral sclerosis with non- Federal University of the State of Rio de Janeiro, Internal Medicine, Rio invasive ventilation de Janeiro, Brazil bFederal University of the State of Rio de Janeiro, Neurology, Rio de K. Komaia, C. Ishidaa, K. Takahashia, A. Tagamib, Y. Motozakia,A. Janeiro, Brazil c Kawashimac Fluminense Federal University, Neurology, Rio de Janeiro, Brazil aNational Hospital Organization Iou National Hospital, Neurology, Kanazawa, Japan Chikungunya infection is caused by the Chikungunya virus and bNational Hospital Organization Iou National Hospital, Respiratory transmitted by the Aeds aegypti mosquito. It happens with an acute Medicine, Kanazawa, Japan onset of fever, and arthralgia, usually self-limited. Serious cases of ARTICLE IN PRESS

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WCN19-2012

Knowledge of Parkinson's disease among medical students of Kyrgyz State Medical Academy

E. Zhunusovaa, C. Shambetovab aI. K. Akhunbaev Kyrgyz State Medical Academy, Faculty of General Medicine, Bishkek, Kyrgyzstan bClinical Hospital of the Administrative Department of Presidential and Government Affairs of the Kyrgyz Republic, Neurology Department, Bishkek, Kyrgyzstan

Objective Aim of the study was to determine the level of knowledge on Parkinson's disease (PD) among students of Kyrgyz State Medical Academy (KSMA).

Methods An anonymous survey was conducted by using (Knowledge of Parkinson's Disease Questionnaire) KPDQ1 (Tan AH), which was previously translated into local languages for another study1. the disease were reported with neurological manifestations, respiratory, renal and endocrinal dysfunctions. Results 180 students took part in the survey. Students had different levels Case report of knowledge in neurology: 17.22% did not study neurology yet (1st 42 years old male with a history of fever and arthralgia, followed group), 31.67% had an introduction course to neurology (2nd group), by lower extremity paresthesia. A week later, it quickly evolved to 51.11% - 4th, 5th, and 6th-year students had ﬁnished full course of upward paresis, dysphagia, dysphonia, and respiratory failure with neurology (3rd and 4th groups). Overall the most recognized motor the need for mechanical ventilation. In addition, he presented symptom (MS) was tremor (91.7%). Only 8.9% of respondents dysautonomia, with arterial hypertension of difﬁcult control, acute recognized constipation as a symptom of PD. Full data on MS and dialytic renal failure, and relevant dysglycemia.

Physical examination flaccid tetraparesis worst distally, deep tendon reflexes were absent in the four limbs. Indifferent plantar cutaneous reflex. Joint position sense was absen in the four limbs, with sensitive ataxia. There was no sensory level. Bilateral facial nerve palsy.

Complementary exams CSF examination with protein-cytological dissociation, 15 cells/ mm3, 100% mononuclear, 175 mg/dL of proteinorrhachia, 60 mg/dL of glucose. Electroneuromyography of the limbs is not available in the public health network. Positive serology in the blood and in the CSF for Chikungunya.

Discussion and comments The increase in Chikungunya infections in tropical regions serves as an alert for its rare neurological manifestations, such as the Guillain Barré syndrome. The severe form of the disease can be followed by respiratory failure, dysglycemia, and acute dysautonomia. The difﬁculty to perform the electroneuromyography should not delay the diagnosis and the initiation of immunomodulating therapy. Our patient showed signiﬁcant improvements after the treatment with intravenous immunoglobulin. doi:10.1016/j.jns.2019.10.1427 ARTICLE IN PRESS

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NMS was written in Fig. 1. Table 1 and Fig. 2 indicate data on true/ Population of Kyrgyz Republic [abstract], Mov. Disord. 33 (suppl false statements, depending on the students' year of studying. 2) (2018). Overall revealed tendency shows an increase of knowledge on PD by 3rd-4th years when students take neurology course, yet that knowledge decrease further on. doi:10.1016/j.jns.2019.10.1428 Conclusion Despite some understanding of PD among medical students, the level of knowledge remains signiﬁcantly low and has a tendency to decrease towards graduation. The study shows that the system of educations WCN19-2035 needs to be adjusted and improved to improve knowledge of PD. Poster shift 03 - Autonomic nervous system disorders/history of Uncited reference neurology/motor neuron disease/movement disorders/MS & [1] demyelinating diseases/neuromuscular disorders/vestibular dis- References orders and nutritional disorders or intoxications

[1] C. Shambetova, A. Arykova, I. Nisheglotova, B. Abdildaev, S.Y. Transcutaneous vagus nerve stimulation for the treatment and Lim, A.H. Tan, Knowledge of Parkinson's Disease in Urban prevention of non-epileptic seizure disorder: A case study ARTICLE IN PRESS

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N. Buckalew Personal information form which is developed by the researcher Integrative Ability Medicine, Living Oak Integrative Medicine and by reviewing the literature on this subject, (Appendix 1), Beck Rehabilitation, Medical Director Medical and Research Program Direc- Depression Inventory Life Satisfaction Scale^, Visual Similarness Scale tor, Hauser, USA for Fatigue. Stress Tendency Scale and EDSS scores of neurology records evaluated by physicians were used in collecting the data. The Introduction analysis of the data in this study was evaluated with IBM SPSS.22 Non-epileptic seizures (NES) are paroxysmal events resembling package program. In the evaluation of the data; Descriptive statistics, epileptic seizure but are not accompanied by electroencephalo- t^-test, Mann-Whitney U test, Kruskal-Wallis H test were used. graphic changes. Like epileptic seizure, NES can be associated with Explanatory variables which had significant effect on Suicidal Idea aura. NES is a frank disability where 1/3 of patients undergo a Scale were performed by multiple regression analysis (stepwise). chronic intractable course resulting in significant disability and lack The mean age of the patients was 41.3 ± 11.44. Of these, %68.2 were of community integration. female and %31.8 were male. It is known that %38 of the patients have primary, %33 have high school education, %23.5 have under- Objective graduate education and %5.6 do not have education. Suicidal ideation To describe the application of transcutaneous vagus nerve was significantly affected by depression, fatigue, stress predisposi- stimulation (TVNS) for the treatment and prevention of NES. tion and life satisfaction of participators (p b .05). Research results in line, depression and fatigue in MS patients Clinical features affect suicidal ideation. At the same time, decreasing quality of life A 34 y/o female patient presented for chronic fibromyalgia and and increasing susceptibility to stress influence suicidal ideation. In migraine headache (MHA) management with a 15-year history of the light of the outcomes of the investigation, a better understanding intractable NES unresponsive to psychotherapy or medical manage- of this effect will enable the improvement of more strategic ment. Aura for her MHA and NES overlapped. Patient had multiple treatments for MS patients. medication sensitivities and allergies with a high disability index. doi:10.1016/j.jns.2019.10.1430 Intervention and outcomes TVNS was prescribed for the MHA. In using the TVNS at times of aura to prevent MHA, patient experienced a significant reduction in episodes of NES (2–4/month to 1/month) and severity of NES WCN19-2040 symptoms notably intensity of motor symptoms (left-sided body convulsions) and episode duration (from an average of 10–15 min to Poster shift 03 - Autonomic nervous system disorders/history of 2–5 min.) The patient commented that she “finally felt she had the power to do something” about her NES and felt more confident going neurology/motor neuron disease/movement disorders/MS & into public places. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Conclusions This case study demonstrates a significant reduction in episodes Effect of kinesiophobia on functional level and quality of life in and symptoms of a female patient with NES disorder with aura after multiple sclerosis application of TVNS. Given this, we are further piloting TVNS for the prevention and treatment of NES (with or without aura) with a M. Terzi, P. Erim prescriptive daily and as needed dosing. Ondokuz Mayis University, Neurology, Samsun, Turkey doi:10.1016/j.jns.2019.10.1429 This study was planned to investigate the relationship between kinesiophobia and functional quality and quality of life in MS patients. The investigation was carried out in Samsun Ondokuz Mayıs WCN19-2039 University Research Hospital between September 30 and December 5, 2018. The participants of the study was composed of MS patients Poster shift 03 - Autonomic nervous system disorders/history of who were registered in Neurology Polyclinic and healthy voluntary people. neurology/motor neuron disease/movement disorders/MS & The personal data form that developed by the researcher demyelinating diseases/neuromuscular disorders/vestibular dis- reviewing the literature on this subject^, BBS^, IPAQ^, FSS^, FIS, SF-36, orders and nutritional disorders or intoxications VAS, McGill, EDSS scores and performance based 6MWT. Expert opinion was obtained for the scope validity of the data collection The relationship between clinical and demographic characteris- tool. The analysis of the data in this study wasevaluated with IBM tics and suicidal ideation in patients with multiple sclerosis SPSS.22 package program. The mean age of 74 patients was 37.81 ± 11.62 years, the mean age M. Terzi, N. Allahguluzad of 11 healthy individuals was 35,90 ± 12,17 years. 68.9% of the Ondokuz Mayis University, Neurology, Samsun, Turkey patients were female and 31.1% were male. 41.9% of the patients were primary school graduates, 17.6% were high school graduates and 40.5% Study was planned to investigate the link between suicidal were university graduates. Pain and fatigue in patients and healthy thinking and clinical and demographic characteristics in patients individuals were found to be related to kinesiophobia. It was found with multiple sclerosis (MS). The participants of the study was that kinesiophobia affected many parameters of quality of life and the composed of MS patients who were registered in Neurology quality of life affected the physical function of the patients. Balance and Polyclinic. fall were found to be associated with a low level of kinesiophobia. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 333

In MS patients, functional status, especially pain and fatigue, is WCN19-2048 related to kinesiophobia. At the same time, increased kinesiophobia affects the quality of life. Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1431 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-2047 Sjogren-Larsson syndrome: Another differential diagnosis of multiple sclerosis in imaging of white matter diseases

Poster shift 03 - Autonomic nervous system disorders/history of E. Pourakbar, S. Zamanian neurology/motor neuron disease/movement disorders/MS & Iran.mashhad university of medical sciences mashhad, Neurology, demyelinating diseases/neuromuscular disorders/vestibular dis- Mashhad, Iran orders and nutritional disorders or intoxications Sjogren-Larsson syndrome(SLS) is an inherited autosomal reces- The comparison of cognitive function between normal and sive neurocutaneous disorder with congenital ichthyosis, spastic abnormal FDG-PET in non-demented Parkinson's disease diplegia or quadriplegia and mental retardation. fi ^MRI nding such as seen in Multiple sclerosis(MS).So this rare S.J. Kim syndrome can be another differential diagnosis of MS. Busan Paik Hospital, Inje University College of Medicine, Department of Neurology, Busan, Republic of Korea Case presentation Index patient was 34 year-old female who was admitted because Objective of weakness and inability to walk. She had globaldevelopmental The aim is to compare the cognitive function between normal and delay.Stiffness in lower limbs started in 4 years before,with progres- abnormal FDG-PET in non-demented Parkinson's disease (PD). sive increase up to the time of presentation.Physical examination showed generalized dryness of skin most prominent on lower limbs Methods with severe pruritus, that is icthyotic lesion.The nail^,plams and soles Twenty seven PD patients performed the FDG-PET. Nine patients were affected.She had short stature.In neurological examination she showed normal FDG-PET. Eighteen patients showed abnormal revealed mental retardation,spasticity in both lower limbs;brisk deep hypometabolism. Cognitive function of PD were evaluated by using tendon reflexes and symmetric bilateral extensor plantar respones. three brief screening tools such as mini-mental state examination Hoffman sign in upper limbs were detected.She had photophobia. (MMSE), Montreal Cognitive Assessment (MoCA) and Frontal EEG showed mild slowing over both hemispheres.MRI of the brian assessment battery (FAB). showed diffuse and no symmetrical plaques with high signal intensity on T2 weighted sequence in bilateral deep periventicular Results white matter and corpus callosum.Some of these lesion were also Hypometabolism was revealed in the parietooccipitotemporal plumb to ventricular. lobes, caudate nucleus and medial frontal lobes, and thalamus. PD patients with hypometabolism showed statistically significant lower Conclusion scores in MMSE (p = .02), MoCA (p = .0245) and FAB (p = .0427) The hallmark of SLS is demyelination of cerebral white matter and compared to those of PD with normal FDG-PET. In subitems analysis, of the corticospinal and vesibulospinal tracts.MRI reveals abnormal a language of MMSE (p = .0201) and MoCA (p = .0252), an high signal intensity on T2 weight and FLAIR sequences especially in orientation of MMSE (p = .0426), and a memory of MoCA (p = periventicular frontal,parietal lobes,corpus callosum and corona .0371) showed significant differences between PD with normal and radiata.Typically,subcortical white matter U fibers are spared. abnormal metabolism. doi:10.1016/j.jns.2019.10.1433 Conclusions Like previously established reports, PD patients with hypometabolism showed more severe cognitive impairment than PD with normal metabolism. Language and memory including WCN19-2057 orientation domains in both MMSE and MoCA showed significantly low score. Contrast to MoCA and MMSE, any subitems of FAB did not showed significant differences between 2 groups. This point showed Poster shift 03 - Autonomic nervous system disorders/history of the limitation of the FAB as a screening assessment in the cognitive neurology/motor neuron disease/movement disorders/MS & function of PD. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1432 Atypical multiple sclerosis with antibody to MOG ARTICLE IN PRESS

334 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

D. Dave, F. Khan, S. Rohatgi, S. Nirhale, P. Rao, P. Naphade WCN19-2062 Dr. D.Y. Patil Medical College- Pune, Neurology, Pune, India

Poster shift 03 - Autonomic nervous system disorders/history of Case presentation We report a case of young female, who had MRI Brain lesions typical neurology/motor neuron disease/movement disorders/MS & of Multiple Sclerosis with CSF Oligoclonal bands and had Definite demyelinating diseases/neuromuscular disorders/vestibular dis- Multiple Sclerosis based on revised McDonald criteria, but she also had orders and nutritional disorders or intoxications atypical features of mild pleocytosis, brainstem and cerebellar peduncle involvement apart from optico-spinal involvement. She turned out to be MTHFR mutation- A rare potentially treatable cause of adult- positive for Anti-MOG antibody as well, hence diagnosed to have onset complicated hereditary spastic paraplegia Atypical Multiple Sclerosis. This case highlights when to suspect Atypical Multiple Sclerosis and their management approach. F.M.A. Khan, D. Dave, S. Rohatgi, S. Nirhale, P. Rao, P. Naphade Dr. D. Y. Patil Medical College- Hospital And Research Center, Neurology, Discussion^ Pune, India Typical MS cases are largely Anti MOG-negative. In a study of 50 Japanese cases with AQP4-IgG-negative opticospinal MS, just two Hereditary spastic paraplegia (HSP) is a group of clinically and were MOG-IgG-positive, but they had some features atypical for MS, genetically diverse disorders that share a primary feature, which is such as bilateral optic neuritis, longitudinally extensive transverse the causation of progressive and generally severe lower extremity myelitis or moderate pleocytosis. weakness and spasticity. No treatment is available for these In another study Antibodies to MOG were found in about 5% (5/ disorders. We present case of 19 year old male with severe 104) of preselected adult patients with MS. Patients with MS with methylenetetrahydrofolate reductase (MTHFR) deficiency manifest- antibodies to MOG showed typical MS lesions on brain MRI with ing as a complicated form of adult-onset hereditary spastic concomitant severe brainstem and spinal cord involvement and had paraparesis. Although juvenile- or adult-onset forms of severe 5,10- a severe disease course with high relapse rates. methylenetetrahydrofolate reductase (MTHFR) deficiency manifesting as complicated hereditary spastic paraplegia have rarely been Conclusion^ described, it is a potentially reversible cause of complex hereditary In conclusion, any patient showing typical MS leisons on brain spastic paraplegia. MRI with OCB present in CSF but has atypical features like mild pleocytosis with brainstem, cerebellar or optico-spinal involvement doi:10.1016/j.jns.2019.10.1436 should also be tested for autoantibodies to MOG and if positive then requires aggressive treatment approach in the form of Plasma Exchange, if resistant to pulse steroid therapy followed by either Rituximab or Natalizumab rather than trying other DMT's. WCN19-2068 doi:10.1016/j.jns.2019.10.1434 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- WCN19-2061 orders and nutritional disorders or intoxications

Dopamine receptor D1 agonism induces DRP-1 inhibition to Poster shift 03 - Autonomic nervous system disorders/history of improve mitochondrial biogenesis and dopaminergic neuro- neurology/motor neuron disease/movement disorders/MS & genesis in rat model of Parkinson's disease demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications A. Mishra, S. Singh, S. Shukla CSIR-Central Drug Research Institute, Divison of neuroscience and aging Study of spectrum of inflammatory demyelinating disease of biology, Lucknow, India central nervous system Dopamine (DA) neurotransmitter acts on dopamine receptors F.M.A. Khan, D. Dave, S. Rohatgi, S. Nirhale, P. Rao, P. Naphade (D1-D5) to regulate motor functions, reward, addiction and cognitive Dr. D. Y. Patil Medical College- Hospital And Research Center, Neurology, behaviour. The depletion of DA in midbrain due to degeneration of Pune, India nigral dopaminergic (DAergic) neurons lead to Parkinson's disease (PD). DA agonist and L-DOPA are the only therapies used for the We report the observations of twenty-five patients from Pune symptomatic relief in PD. However, the role of DA receptors in PD diagnosed with inflammatory demyelinating diseases of CNS over a pathogenesis and how they associated with mitochondrial functions period of 1 year. The aim is to highlight recent observations in and DAergic neurogenesis is not known. In the present study, we inflammatory demyelinating diseases in the central nervous system. investigate the mechanistic aspect of DA receptor D1 mediated We emphasize the discriminative nature of new clinical, imaging, control of motor behaviour, mitochondrial functions and DAergic immunopathologic data, the rising incidence of anti-MOG antibody neurogenesis in 6-hydroxydopamine (6-OHDA) induced rat model of associated central nervous system demyelination and atypical MS PD. The pharmacological activation of D1 receptor markedly due to easy and cost effective availability of anti-MOG antibody test. improves motor deficits and increased mitochondrial biogenesis, In the light of new findings, typical multiple sclerosis may represent ATP levels, mitochondrial membrane potential and protects nigral a small entity in spectrum of demyelinating diseases. DAergic neurons against 6-OHDA induced neurotoxicity in adult rats. However, these D1 agonist mediated effects were abolished follow- doi:10.1016/j.jns.2019.10.1435 ing D1 receptor antagonist treatment in 6-OHDA lesioned rats. Interestingly, pharmacological inhibition of Drp-1 by Mdivi-1 in D1 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 335 antagonist treated PD rats, significantly restored behavioural deficits, found indicates a need for training of movement disorder specialist mitochondrial functions, mitochondrial biogenesis and enhances the in Nigeria and provision of basic drugs for managing affected number of newborn DAergic neurons. The Drp-1 inhibition mediated patients. neuroprotective effects in PD rats were associated with increased level of cell survival cues (Akt and ERK). Taken together, our data doi:10.1016/j.jns.2019.10.1438 suggests that dopamine D1 receptor mediated reduced mitochondrial fission and enhanced DAergic neuron formation involve Drp-1 inhibition that result in improved behavioural recovery in PD rats. doi:10.1016/j.jns.2019.10.1437 WCN19-2091

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-2088 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Effect of gender and sex factors on MS course in women demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications M. Missaoui, S. Naija, A. Hassine, M. Ben Halima, R. Douma, A. Nasr, S. Ben Amor Frequency and pattern of movement disorders in a neurologic Sahloul university hospital, Department of neurology, Sousse, Tunisia clinic in North Central Nigeria: A 3- year review Introduction E. Iwuozoa, O. Obiakob Multiple sclerosis (MS) is a multifactorial disease that affects aFederal Medical Centre, Internal Medicine Department, Makurdi, women during their period of genital activity. The potential role of sex hormones on the immune system raises the question of the Nigeria^ bAhmadu Bello University, Internal Medicine Department, Makurdi, interaction between different phases of genital life and the activity of Nigeria MS.

Aims Background Establish a correlation between the evolutionary proﬁle of MS Movement disorders are neurological disorders found worldwide (EDSS score and progression index) and hormonal factors. with varying forms. They are generally thought to be uncommon in Sub-Saharan Africa (SSA) because of the relatively large young Material and methods population in SSA. There is paucity of data about the disease in Sub- This is an observational analytic study that includes patients Saharan Africa including Nigeria. with MS diagnosed for at least two years and are regularly monitored in our department. The participants were assessed using Objective a simple and brief survey: menarche’s age, gravidity and parity, This study evaluated the frequency and pattern of movement menstrual cycle régularity, EDSS score, disease’s duration and disorders in a neurology clinic in a tertiary hospital in North Central progression index. Nigeria over a 3-year period.

Results Methods Our study included 54 patients, with an average age of 34 years. The records of all patients attending Neurology Clinic in Federal The population is predominantly composed of patients with Medical Centre, Makurdi North Central Nigeria were reviewed relapsing-remitting MS (90%), with an average duration of evolu- retrospectively from March 2016 to February 2019, and diagnoses tion of 4 years. The average age of menarche is 11.5 years. There is as made by the Neurologist were obtained. Data was analyzed using no significant difference between the age of menarche and the the statistical package for social sciences (SPSS) software (version progression of the disease. Half of our patients had children after 20; SPSS, Chicago, IL, USA). the diagnosis of the disease and there is a significant difference between the EDSS score during pregnancy and post partum. It was Results found that 64% of patients had a regular menstrual cycle. The Four hundred patients with neurological diseases were seen over progression index was significantly higher in women with an the time period out of which 49 of them had diagnosis of movement irregular menstrual cycle than in those with a regular cycle (p disorders made; with a frequency of 12.25%. The mean age of b0.05). patients with movement disorders was 55 (SD = 13.49) years, with a range of 25–76 years; 35 (71.4%) were males and 14 (28.6%) were Conclusion females. Twenty eight (57.2%) had Parkinsonism, 6 (12.2%) had There seems to be a relationship between sex hormones and MS. dystonia, 6 (12.2%) had essential tremors, 4 (8.2%) had Tics, 3 (6.1%) Therefore, new therapeutic strategies could be envisaged. had chorea and 2 (4.2%) had myoclonus.

Conclusion Parkinsonism is the most common movement disorder seen in doi:10.1016/j.jns.2019.10.1439 our neurology clinic. The high frequency of movement disorders ARTICLE IN PRESS

336 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-2094 Endoplasmic reticulum (ER) present in the cell provides exclusive oxidizing environment for the proper protein folding before its transfer to the other organelle. Many neurological disorders occur Poster shift 03 - Autonomic nervous system disorders/history of due to the improper protein folding. Oxidative stress and ER stress neurology/motor neuron disease/movement disorders/MS & are very closely related events but the molecular mechanism lying demyelinating diseases/neuromuscular disorders/vestibular dis- beneath the processes are still elusive. Parkinson's disease (PD) is a orders and nutritional disorders or intoxications neurological disorder that is behaviourally characterized by the improper movement and pathologically characterized by the accu- Brachysyndactyly due to Poland syndrome mulation of lewy bodies. Improper folding of proteins is one of the reasons for PD incidence that increases the burden on cellular O. Hernandez-Fustesa, C. Arteaga Rodriguezb, O.J. Hernandez Fustesc physiology. Dopamine is required for the proper movement and its aComplexo Hospital de Clínicas-UFPR, Neurology, Curitiba, Brazil scarcity leads to the PD. Rotenone is a well-known pesticide that bUniversidade positivo, Neurology, Curitiba, Brazil mimics PD like symptoms in rats both by behaviourally and cClinica Neurológica das Américas, Neurophysiology, Curitiba, Brazil pathologically. Thus we have used rotenone in the present study to find out how oxidative stress generated in the system leads to the ER stress and ultimately causes Parkinsonism in rats. Current researches A 49-year-old woman, was referred for electroneuromyography are concerned about the understanding the underlying mechanism examination for paresthesias, pain and thoracic outlet syndrome of neuroprotection provided by the antioxidants. Till date it is not hypothesis. The motor and sensory nerve conduction and electro- clear through which mechanism quercetin provides protection myography was normal. At the physical examination we observed against rotenone induced ER stress, oxidative stress and neurotox- brachysyndactyly (Fig. 1) and atrophy of the pectoralis major icity. In the present study we found that quercetin significantly confirming the diagnosis of Poland syndrome (PS). lowered the level of oxidative stress and ER stress in rotenone The first description is attributed to Alfred Poland, of Guy's treated rats. Thus the study could offer a novel utility of quercetin as Hospital, London, based on the autopsy of a 27-year-old male in a promising therapeutic agent against PD after further examinations. which he described syndactyly of the hand with ipsilateral absence of the left sternocostal head of the pectoralis major. The condition doi:10.1016/j.jns.2019.10.1441 was not named eponymously until 1962, when Dr. Clarkson, described a series of 3 cases of syndactyly with accompanying ipsilateral breast hypoplasia. PS is a congenital syndrome characterized by deficiency of the pectoralis major muscle,presents a wide phenotype variability, WCN19-2102 including partial agenesis or deformity of the cartilage rib; hypoplasia or aplasia of the breast and nipple-areola complex, axillary fold, Poster shift 03 - Autonomic nervous system disorders/history of and subcutaneous tissue; sternal deformities. neurology/motor neuron disease/movement disorders/MS & The association of PS with the deformity of Sprengel, reported by demyelinating diseases/neuromuscular disorders/vestibular dis- 15.9%, may be one of the causes of referral for the performance of neurophysiological studies, since in the physical examination the orders and nutritional disorders or intoxications carriers present elevation of the scapula. Treatment of PS is determined by the severity of the defect and Gender association of impulse control disorders (ICD-RBS) in the resulting anatomical dysfunction. In conclusion, the early patients with Parkinson's disease and its impact on quality of life detection in clinical practice is important because it can be associated with muscle pain, joint pain and functional limitations, requiring an B. Paula, G. Paulb, G. Singhc early multidisciplinary approach. aMBBS MD DM Neurology, Neurology, Ludhiana, India bDMC and Hospital, Critical Care, Ludhiana, India doi:10.1016/j.jns.2019.10.1440 cDMC and Hospital, Neurology, Ludhiana, India

ICD-RBs are under-recognized in clinical practice as patients do not spontaneously offer information, either because of shame or they WCN19-2097 do not understand its correlation to PD and its treatment. Certain ICD relevant in the Indian scenario apart from the criteria set in QUIP-RS have not been studied yet were explored. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Aim demyelinating diseases/neuromuscular disorders/vestibular dis- To study the gender difference and risk factors in the prevalence orders and nutritional disorders or intoxications of ICD-RBs in Indian PD patients and its impact on Quality of life (QOL). Does quercetin protects against oxidative stress and endoplasmic stress interplay in rotenone-induced nigrostriatal dopaminergic Methods neurodegeneration in rats? Hospital based observational cross-sectional study of PD patients on treatment with dopamine replacement therapy for more than six P. Tripathi, S. Shukla months visiting movement disorder clinic. CSIR-CDRI- Lucknow U.P.- India, Department of Neuroscience and Aging After informed consent, patients or informant completed the Biology, Lucknow, India QUIP-RS questionnaire, in the language of choice (English/Punjabi/ ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 337

Hindi) based on behavior that occurred anytime during PD and Methods lasted N4 consecutive weeks. In addition other ICDs relevant in the A literature review was undertaken in PubMed, Global Science. Indian population as body-focused repetitive behaviors were also Org and Google scholar. In addition, data from 213 children including explored as a new part of the QUIP-RS questionnaire. 124 cases and 89 controls from the NIH-DRC konzo project were The frequency and impact of ICD on QOL was studied using used in the retrospective study. Sociodemographic factors, somatic, validated PDQ-39 Questionnaire. neurological and neuropsychological assessment data as well as biomarkers of malnutrition and cyanide intoxication were the Results variables of interest.

Results 1. Total 80 patients (46 men,34 women) were included in 6 months ^ The age of the children was higher in the severe form and lower period. in the mild form. Konzo disease severity was related to socio- 2. Trends show ICD-RBs are more frequent in women (41.8%) ^ economic distress, malnutrition and micronutrient deficiency (sele- compared to men(32%) with punding being the most frequent nium, zinc and copper). Patellar and ankle exaggerated reflexes were (Fig. 1). more common in mild form thus ankle clonus, speech articulation 3. Ât least one ICD-RB was present in 31(38.74%) patients Table 1. ‘ICD not classified elsewhere’ as trichotillomania was present in 3(3.75%) disorders and Lhermitte sign were more frequent in moderate form. 4. ^ patients. Socio-emotional, visual disorders and ankylosis had high frequency in severe form. Moderate form had shorter enzymatic detoxification 5. Significant risk factors for ICD-RB are younger age, longer disease, ^ rate thus higher urinary thiocyanate levels as well as some more years of treatment and higher dose of dopamine. neurocognitive performances. Heart rate increased significantly with 6. QOL was much worse in those with any ICD-RB than those without ^ Konzo severity regardless of age. Frequently, severe form evolved any ICD-RB(Table 2). towards mild form and vice versa. doi:10.1016/j.jns.2019.10.1442 Conclusion Characteristics of three konzo clinical patterns within children suggest different models of adaptation to cyanide intoxication that require in-depth studies. WCN19-2109 Keywords: Socioeconomic distress, heart rate, cyanide intoxication, Poster shift 03 - Autonomic nervous system disorders/history of malnutrition, reflex abnormalities neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1443

Clinical and biological characterization of konzo forms in children in Kahemba/Democratic Republic of Congo WCN19-2113 J. Mbusa Kombia, D.L. Mukeba Kahembaa, J.P. Banea Mayambub,M. Boivinc, J. Muyembe Tamfumd, D. Mumba Ngoyd, D. Tshala Poster shift 03 - Autonomic nervous system disorders/history of Katumbaye, D. Okitundu Luwa E. -Andjafonoa aUniversité de Kinshasa, Neurologie, Kinshasa, Democratic Republic of neurology/motor neuron disease/movement disorders/MS & the Congo demyelinating diseases/neuromuscular disorders/vestibular dis- bInstitut Supérieur des Techniques Médicales, Programme National de orders and nutritional disorders or intoxications Nutrition, Kinshasa, Democratic Republic of the Congo c Michigan State University, Psychiatry- Neurology and Ophtalmology, Reduction of risk of secondary progressive multiple sclerosis Lansing, USA within two years of treatment with cladribine tablets: An analysis d Université de Kinshasa & Institut National de Recherche Biomédicale of the clarity study RDC, Département de Médecine Tropicale, Kinshasa, Democratic Republic of the Congo P. Vermerscha, G. Giovannonib, P. Soelberg-Sorensenc, K. Ram- e Université de Kinshasa- République Démocratique du CongoRDC et mohand, S. Cooke, B. Kellerf, S. Royg Oregon Health & Science University, Département de Neurologie et aUniv. Lille, INSERM U995- CHU Lille- FHU Imminent, Lille, France Department of Neurology and Center for Research on Occupational and bQueen Mary University of London, Blizard Institute- Barts and The Environmental Toxicology, Portland, OR, USA London School of Medicine and Dentistry, London, United Kingdom cUniversity of Copenhagen- Rigshospitalet, Danish MS Center- Depart- Background ment of Neurology, Copenhagen, Denmark Clinical and biological characteristics of three forms of konzo dUniversity of Miami School of Medicine, MS Research Center, Miami, severity are still poorly documented. USA eRutgers- The State University of New Jersey, New Jersey Medical School, Objective Newark, USA Identify clinical as well biological and evolutive characteristics of fMerck Healthcare KGaA, n/a, Darmstadt, Germany three clinical patterns of konzo and associated disorders of other gMerck, a division of Merck Healthcare KGaA- Darmstadt- Germany, organs. Aubonne, Switzerland ARTICLE IN PRESS

338 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Introduction Case report Cladribine tablets 10 mg, cumulative dose 3.5 mg/kg (CT3.5) over 13-year-old Emirati male from a consanguineous family (1st 2 years, showed efficacy versus placebo in patients with relapsing cousins), presented with sudden onset of generalized muscle multiple sclerosis (MS) (CLARITY). This post hoc analysis explored cramping, especially in the abdomen, which led to difficulty the relationship between baseline expanded disability status scale breathing. He was found to have cola-colored urine which improved (EDSS) and risk of progression to secondary progressive MS (SPMS) over few days and high serum CK level (spiked at around 200,000 or to EDSS≥6.0 in CLARITY. Units/l) which plateaued after 2 weeks he had previous similar episodes. Methods A proxy composite definition of SPMS was developed for this Discussion analysis. Patients progressing to EDSS≥6.0 were defined as ≥1 post- His LGMD panel showed a variant which is unlikely to be baseline EDSS≥6.0 with 3- or 6-month confirmed disability progres- pathogenic and was paternally inherited on further testing with trio sion (CDP). exome. The exome did reveal a homozygous, predicted to be truncating, variant in the MLIP gene, which encodes for the muscular Results LMNA interacting protein (MLIP), highly expressed in skeletal and Proxy SPMS progression was seen in 6.7% versus 13.5% (CT3.5 smooth muscles and may contribute to the mesenchymal pheno- versus placebo: OR 0.46 [95%CI: 0.28, 0.76]; p = .0024). In the types of laminopathies. baseline EDSS≤3.0 subgroup proxy SPMS progression was 3.5% This might explain the rhabdomyolysis seen in our patient. versus 7.7% (CT3.5 versus placebo: OR 0.44 [95%CI: 0.19, 0.99]; p = .0471). For baseline EDSS≥3.5, proxy SPMS progression was 12.2% Conclusion versus 22.4% (CT3.5 versus placebo; OR 0.48 [95%CI: 0.26, 0.9]; p = We would like to report this case due to the rarity of this .0212). combination and its exceptional nature as LGMD in this patient is Proportions of patients with 3-month CDP with EDSS≥6.0 were caused by a homozygous MLIP gene variant. This gene is related to 3.5% vs 8.0% (CT3.5 versus placebo; OR 0.42 [95%CI: 0.22, 0.82]; p = the lamin complex but, has only recently been linked to dilated .0114). Patients with 6-month CDP with EDSS≥6.0, were 2.8% versus cardiomyopathy in humans thus far, while animal studies have 5.8% (CT3.5 versus placebo; OR 0.48 [95%CI: 0.22, 1.02]; p = .0566). shown a link to cardiac dysfunction. Patients with baseline EDSS≤3.0 that had ≥1 EDSS≥6.0 were 0.8% vs 4.3% (CT3.5 versus placebo; OR 0.18 [95%CI: 0.04, 0.81]; p b .0262). doi:10.1016/j.jns.2019.10.1445 Those with baseline EDSS≥3.5 and ≥ 1 EDSS≥6.0 were 16.2% versus 29.9% (CT3.5 versus placebo; OR 0.45 [95%CI: 0.26, 0.79]; p = .0051).

Conclusions WCN19-2119 The risk of progressing to proxy SPMS within 2 years of treatment or experiencing EDSS≥6.0 were signiﬁcantly reduced with CT3.5 versus placebo, regardless of baseline EDSS. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Clarity demyelinating diseases/neuromuscular disorders/vestibular dis- NCT00213135 orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1444 An unusual myopathy with dual pathology: Case report

S. Esmail Norfolk and Norwich University Hospital- NHS Foundation Trust, WCN19-2118 Neurology, Norwich, United Kingdom

Poster shift 03 - Autonomic nervous system disorders/history of Abstract Immune-mediated necrotising myopathy (IMNM) and inclusion neurology/motor neuron disease/movement disorders/MS & body myositis (IBM) belong to a spectrum of conditions called the demyelinating diseases/neuromuscular disorders/vestibular dis- idiopathic inflammatory myopathies. However, they both represent orders and nutritional disorders or intoxications distinct conditions, which differ in their clinical presentation, immuno-pathogenesis and in the available treatment options. Novel limb-girdle muscular dystrophy with rhabdomyolysis and In this poster presentation, I report the case of a 69-year old persistently elevated creatine kinase level gentleman who had presented with a gradually progressive, symmetric, proximal myopathy with preservation of distal muscle S.S. Ezadiniaa, K. Kokab Irfanb, K. Aujala Irfanb strength including the deep finger flexors. There was no rash, bulbar aEmergency department, Ras al kaimah, United Arab Emirates disturbance or other focal neurological deficits. A serum creatine bInternal medicine, Ras al khaimah, United Arab Emirates kinase was significantly elevated (4935 IU/L) and serological testing revealed strongly positive anti-HMGCR antibodies. Although these Introduction clinical and biochemical features robustly support a diagnosis of Rhabdomyolysis is a serious clinical emergency in which skeletal IMNM, skeletal muscle biopsy showed evidence of dual pathology muscle breaks down rapidly. Underlying disorders e.g. LGMD(Limb- with histopathological features consistent with a combination of girdle muscular dystrophy) can cause rhabdomyolysis but are often IMNM and IBM. Computed tomography (CT) of the chest, abdomen difficult to diagnose due to their marked diversity and rarity. We and pelvis showed no underlying occult malignancy. The patient report a case of a novel LGMD with rhabdomyolysis as this is the first subsequently demonstrated an excellent clinical and biochemical of its kind to be found in UAE. response to high dose oral corticosteroid treatment, with improved ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 339 muscle strength and mobility, and was simultaneously commenced WCN19-2131 on azathioprine immunotherapy. This poster illustrates an unusual case of biopsy-proven dual IMNM and IBM pathology in skeletal muscle. Herein, I discuss Poster shift 03 - Autonomic nervous system disorders/history of valuable learning points derived from this case, including the neurology/motor neuron disease/movement disorders/MS & importance of interpreting muscle biopsy-ﬁndings in the context of demyelinating diseases/neuromuscular disorders/vestibular dis- the relevant clinical and biochemical proﬁle when making a orders and nutritional disorders or intoxications diagnosis, and constructing treatment strategies, in the myopathic patient. Different diagnoses of patients referred to multiple sclerosis outpatient clinic

F. Balcı doi:10.1016/j.jns.2019.10.1446 University of Health Sciences Haseki Training and Reseach Hospital, Neurology, Istanbul, Turkey

Background and aims The aim of this study is to investigate the frequency and type of WCN19-2124 MS mimics in the patients who were diagnosed with MS in other centers and applied to our MS center in the last 3 years. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Methods demyelinating diseases/neuromuscular disorders/vestibular dis- We included all patients who applied to our MS clinic between January 1, 2016 and December 31, 2018 for the first time in our orders and nutritional disorders or intoxications retrospective study. The definitive diagnosis was recorded and demographic, clinical, laboratory, electrophysiological and radiolog- Preclinical pilot study results of 24-h apomorphine subcutaneous ical variables were collected. infusion delivered via the h-Patch wearable device Results J. Barnes, J. Saldanha, E. Lucera 296 patients were included in the study. Of 235 patients referred from Valeritas- Inc, Corporate Development, Bridgewater, NJ, USA other centers, 166 (70.6%) confirmed the diagnosis of definitive MS, while 69 (29.4%) had an alternative diagnosis. While 52.5% of the patients Treatment of advanced Parkinson's disease (PD) remains referred with clinical suspicion were diagnosed with MS, 8.1% of those challenging, with fluctuations in motor status often resulting in who were referred with radiological suspicion were diagnosed with MS. patients becoming severely handicapped. The short-acting dopa- The most frequently diagnosed diagnoses were: non-specificMRIwhite mine D1/D2 receptor agonist, Apomorphine (Apo), was the first matter lesions (17.9%), migraine (11.5%), psychogenic (8.9%), systemic dopamine receptor agonist used to treat PD. The magnitude and autoimmune diseases (6.4%), NMO (4, 7%), and genetic diseases (1,7%). pattern of the motor response to single dose of subcutaneously Factors that assisted in the correct diagnosis were; non-specific administered Apo is qualitatively comparable to that of oral MRI findings, normal neurological examination, normal evoked levodopa, however side effects (nausea, vomiting, etc.) can be potentials, isolated MRI findings, MRI findings not fulfilling 2010 problematic. Close to a dozen clinical studies have shown McDonald criteria, absence of oligoclonal bands, cognitive presenting subcutaneous Apo infusions are successful in aborting ‘off’ periods, symptoms, family story. reducing dyskinesias and improving PD motor scores with the added benefit of a substantial levodopa-sparing effect. However, Conclusion bulky infusion pumps requiring delivery of relatively large volumes Our study shows that 29,4% of patients had an alternative remain a barrier to development of therapeutic products that are diagnosis. The most common mimickers of MS in our center is not patient (and caregiver) friendly. We investigated the pharmacoki- different from what has been described in other countries. Neuro- netics (PK) of Apo delivered over a single 24-h period using the logical signs and symptoms, age, family story, DNA analysis, wearable h-Patch™ subcutaneous infusion device in dogs. Apo (10 laboratory and radiological findings can help with the true diagnosis. mg) was delivered with PK evaluated at time points to 48 h from the start of infusion. Apo levels were detectable in blood detected doi:10.1016/j.jns.2019.10.1448 within two hours of the beginning of infusion and gradually dropped off after completion of h-Patch™ infusion (24 h). With additional work on a concentrated Apo solution and modest expansion of the h-Patch reservoir, the wearable h-Patch represents WCN19-2132 a patient friendly subcutaneous delivery mechanism for Apo providing the benefits of a full 24 h infusion of low dose Apo to eliminate ‘off’ periods and improve motor status without tolerabil- Poster shift 03 - Autonomic nervous system disorders/history of ity issues seen with subcutaneous injections. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1447 RIS away or on-way to MS ARTICLE IN PRESS

340 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

A. Alskaf Neurological resident, Neurology department, Homs, Syria

Abstract Risk factors groups and percentage to MS conversion still recommended.Patients who start DMT earlier do better than those delayed.The opportunity to intervene earlier is our goal to prevent early disability and death.(BY:Dr–ASMA ALSKAF)

Introduction Radiologically Isolated Syndrome “RIS” describe incidental MRI ﬁndings strongly suggestive of MS in patients with normal neurologic examination.Healthy patients have MRI procedure mainly(up to 60%) ﬁ because of Headaches, cognitive de cit^,increased anxiety and depression.Two-thirds of RIS patients show radiological progression and one- third develop clinical symptoms in 5 years. What the prognosis of RIS is?and should DMT be started at earliest possible stage?.

Methods A study-report of 70 RIS patients undergo paraclinical studies (blood,CSF,and VEP)and MRI follow-up every(3–6)months.

Results 33% of patients had clinical conversion:6 optic neuritis,6 myelitis,5 brainstem symptoms,4 sensitive symptoms,1 cerebellar and 1 cognitive deterioration. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 341

VEP,gadolinium enhancement and infratentorial lesions were pericardial effusion suggestive of associated pericarditis. She had a significant for clinical conversion to MS.IgG and OCB in CSF were raised troponin level. Her relevant lab investigations to reveal a possible significant when associated with 9 T2 lesions on first MRI.Decreased cause of myocarditis and pericarditis such as infectious and vasculitis brain volume and thalamic atrophy presented in early stages of workup all came back negative. Patient was treated in the cardiac critical disease. unit (CCU) and her condition stabilized after 10 days with bed rest, anti- coagulation, anti-platelets, diuretics and ACE inhibitor. Discussion A repeated ECG upon discharge was normal, and a follow up Risk factors for both Radiologic and Clinical progression: echocardiogram 3 weeks later showed marked improvement with normal left ventricular ejection fraction (EF 45%). * More than 9 T2 lesions. This serious adverse effect has already been reported in cases * Gadolinium enhancing lesions. other than Multiple Sclerosis which were treated with the same * Risk factors of Clinical progression: medication. But only few cases reported in MS patients which * Cervical spine lesions. mandates strict monitoring at baseline and on regular basis. * Infratentorial lesions. * Pathologic VEP. doi:10.1016/j.jns.2019.10.1450 * High number of lesions. * Younger age. * O.C.B and/or IgG index with 9 T2 lesions. WCN19-2138 DMT shows efficacy in brain atrophy patients.Thoracic spine lesions are advised to have DMT.Some suggest 6-months MRI follow- Poster shift 03 - Autonomic nervous system disorders/history of up;if dissemination in time proved Radiologically;DMT should be initiated at those of predictive findings in CSF(newly defined neurology/motor neuron disease/movement disorders/MS & including IL-8 and chitinase 3-like-1 protein). demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1449

Transorbital ultrasound in acute optic neuritis

Y.H. Kim WCN19-2141 Hangang Sacred Heart Hospital, Department of neurology, Seoul, Republic of Korea Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Introduction demyelinating diseases/neuromuscular disorders/vestibular dis- Visual evoked potentials (VEP), optical coherence tomography orders and nutritional disorders or intoxications (OCT), and the magnetic resonance imaging have been used to evaluate functional and morphologic status of the afferent visual Alemtuzumab induced myocarditis and myocardial dysfunction pathway, however these utilities have some limitation to evaluate in a patient with multiple sclerosis: A case report optic nerve pathology in early stage. Recently transorbital ultrasound (TOUS) has been introduced as a promising tool to evaluate optic bulb and nerve. B. Mohameda, I. Alsharoqib, R. Matarb, F. Almosawic aSalmanya Medical complex, Neuroscienence department, Manama, Objective Bahrain To assess the practical diagnostic value of TOUS in patients with bSalmanya Medical complex, Neuroscience department, Manama, ON along with other diagnostic tools such as VEP, and OCT. Bahrain cSalmanya medical complex, Family Medicine department, Manama, Methods Bahrain TOUS, OCT, and VEP were performed in both eyes of 8 patients with acute ON. The optic nerve were scanned bilaterally in fi Two months after administering the rst cycle of Alemtuzumab transorbital view to obtain axial image. The optic nerve diameter IV infusion at a dose of 12 mg\d for 5 days for MS treatment, a 28- was measured at point 3 mm posterior to eye globe with and year-old female presented with 1-day history of sudden severe chest without nerve sheath. All measured parameters were compared discomfort, dyspnea, orthopnea and palpitation. On physical exam- between affected and non-affected side. ination, the patient was in respiratory distress associated with tachycardia of 115/min, BP was 130\90, afebrile. Cardiac examination Results showed normal S1 an S2 heart sounds, with no murmurs, no JVP Eight patients (4 men, median age 43.5 years) were recruited. The fi distention noted. However, the respiratory examination revealed ne median diameter of the optic nerve without sheath on the affected basal lung crepitation bilaterally as well as bilateral lower edema in side (median 3.28 mm) was significantly larger compare to the non- keeping with pulmonary edema. Neurological exam was stable. affected side (median 3.08 mm; p = .003). The diameter with sheath ECG showed sinus tachycardia and elevated ST segment elevation in also showed significant difference (5.36 mm in affected side vs. 5.22 all leads. Her echocardiogram was highly suggestive of acute myocar- mm in non-affected side; p = .018). The diameter of optic nerve did ditis with reduced left ventricular ejection fraction (EF 30%) and not show correlation with VEP and OCT on the same side. ARTICLE IN PRESS

342 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Conclusion Keywords: Multiple sclerosis, Brain MRI, Cognitive impairment, Optic nerve diameter in TOUS was signiﬁcantly increased in acute MoCA test ON. TOUS could be considered in patients with ON in early stage as supportive tools to diagnose. doi:10.1016/j.jns.2019.10.1451 doi:10.1016/j.jns.2019.10.1452

WCN19-2147 WCN19-2149

Correlation between brain magnetic resonance imaging, cognitive Unrecognised and undiagnosed parkinsonism in rehabilitation dysfunction and physical disability in multiple sclerosis facilities

ab b b A. Abouelmaatya, M. Flifel Elsaydb, C. Zarad Alic A. Aggarwal , F. Sarmast , M. Hendriks a aLecturer of neurology, Neurology department Helwan University, RPAH, Neurology, Sydney, Australia b Helwan, Egypt MetroRehab, Rehabilitation Medicine, Sydney, Australia bLecturer of neurology, Neurology department Mansours University, Elmansora, Egypt Introduction cLecturer of diagnostic radiology, Diagnostic radiology department port Parkinsonism refers to a range of clinical presentations and said University, Port said, Egypt constellation of symptoms, both motor and non-motor, which are dopamine responsive. The cardinal signs of Parkinsonism are Background bradykinesia with rigidity, resting tremor or postural instability. It Montreal Cognitive Assessment test (MoCA) is a brief, sensitive is due to degeneration of the nigrostriatal dopaminergic system. test that has been recommended as a reliable tool to detect mild cognitive impairment. Associations between brain imaging measures Aim and cognitive functioning have been observed in patients with The aim of this study was to identify patients with undiagnosed multiple sclerosis (MS). and unrecognised Parkinsonism and assess the efficacy of dopamine replacement therapy on their functional mobility. Objectives To evaluate cognitive dysfunction and physical disability in MS Method patients by making correlation between Magnetic Resonance Imag- 20 patients who were admitted to MetroRehab Hospital for ing (MRI), MoCA test and expanded disability status scale (EDSS). various reasons other than Parkinson’s Disease, from November 2017 till July 2019, with slow progress, history of frequent falls or Methods impaired gait pattern were identified and re-examined. Fifty MS Patients and twenty-five controls underwent clinical A trial of Levodopa was considered for patients with signs of evaluation and assessment of cognitive functions using the MoCA Parkinsonism. Madopar or Sinemet was started at a small dose of half test. In addition, all MS patients underwent conventional MRI brain a 100/25 mg tablet twice a day and it was gradually increased to and Expanded disability status scale (EDSS). 200/50 mg tablet three times a day, depending on patients’ response and tolerance. 10MWT was selected as an outcome measure for Results efficacy of Levodopa. The scores for trail making test, memory, attention, serial seven subtractions, fluency, naming and orientation in MS patients were Results significantly different from control (p b 0.05). There was significant Results of this study will be presented. inverse correlation between number of MS plaques in the temporal lobe and abstraction (p b 0.001, r = -0.52). Less inverse correlation Conclusion was found between total number of MRI plaques and concentration, Parkinsonism is a common neurodegenerative disorder that is total number of MRI plaques and abstraction, infratentorial lesions unrecognised in rehabilitation settings. Patients with parkinsonism and clock drawing test. No correlations were found between number are often admitted to rehabilitation facilities with a variable range of of MS plaques in frontal, parietal, occipital, corpus callosum and diagnosis; they show slow progress which might be misinterpreted neuropsychological tests. as deconditioning or just part of the “aging” process. A trial of Levodopa should be considered for patients with signs of Parkinson- Conclusion ism starting at a small dose, which is generally very well tolerated. Although conventional MRI techniques are crucial in the MS diagnostic workup, their accuracy in evaluating and predicting cognitive dysfunction is less relevant. The MoCA test would provide doi:10.1016/j.jns.2019.10.1453 a brief screen for cognitive dysfunction in MS. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 343

WCN19-2150 Materials and methods 30 patients with definite, probable or possible ALS(revised El Escorial Criteria) and 60 healthy controls were included in this study. Poster shift 03 - Autonomic nervous system disorders/history of After obtaining informed consent, they were subjected to oral NMS neurology/motor neuron disease/movement disorders/MS & questionnaire, Beck’s Depression Inventory, and Epworth Sleepiness demyelinating diseases/neuromuscular disorders/vestibular dis- scale. Statistical Analysis was done using the SPSS software. orders and nutritional disorders or intoxications Results Repetitive transcranial magnetic stimulation can improve anxiety ALS patients reported significant higher nonmotor scores in in Parkinson's disease: A randomized, double-blind and con- comparison to controls. There was statistically significant (p b 0.05) trolled trial increased prevalence of daytime drooling, altered taste/smell, dysphagia, nausea, constipation, weight loss, loss of interest, fi A. Juhasz, A. Makkos, M. Kovacs, M. Harmat, D. Pinter, N. Kovacs dif culty concentrating, anxiousness, falling, insomnia, nightmares, University of Pécs, Department of Neurology, Pécs, Hungary leg swelling and unpleasant sensations in leg in ALS patients as compared to controls. There was also statistically significant (p b 0.05) increase in suicidal ideation, pseudobulbar affect and day time High frequency (HF) repetitive transcranial magnetic stimulation sleepiness in ALS patients. Correlation study showed increased NMS (rTMS) over the left dorsolateral prefrontal cortex (DLPFC) can scores with disease progression. improve depression in Parkinson's disease (PD). In literature only a few clinical data are available on efficacy of HF rTMS treatment Conclusion regarding anxiety. Methods: Thirty patients with PD were randomly Nonmotor symptoms were significantly more prevalent in ALS assigned to active (n = 15) and sham (n = 15) rTMS. High patients. They seemed to increase with disease progression. There- frequency rTMS was applied over the left DLPFC for 10 days. An fore these symptoms should be actively sought for and treated to investigator blinded to the treatment performed three video-taped improve the quality of life of ALS patients. examinations on each patient: before stimulation (baseline), 1 day (short term), and 30 days after treatment session ended (long-term Limitations effect). Primary endpoint was change in anxiety measured by the Smaller sample size. Hamilton Anxiety Scale (HAM-A). Results: Two patients (one in the active and another in the sham-treated group) did not complete the doi:10.1016/j.jns.2019.10.1455 protocol; therefore; their data were excluded from the analysis. In the actively-treated group the severity of anxiety improved from 14.9 ± 4.8 points to 8.3 ± 5.2 points (p = .039) while in the sham treated group it remained unchanged (from 14.5 ± 5.7 points to 14.1 ± 5.2 points, p = .879). Conclusions: Our results demonstrate WCN19-2168 the beneficial effects of high frequency left DLPF rTMS on anxiety in PD. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1454 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Juvenile onset parkinsonism-dystonia syndrome: A novel muta- WCN19-2162 tion in the SLC6A3 gene

a a b Poster shift 03 - Autonomic nervous system disorders/history of A. Abusrair , S. Bohlega , S. Wakil aKing Faisal Specialist Hospital & Research Center, Neurosciences, neurology/motor neuron disease/movement disorders/MS & Riyadh, Saudi Arabia demyelinating diseases/neuromuscular disorders/vestibular dis- bKing Faisal Specialist Hospital & Research Center, Genetics, Riyadh, orders and nutritional disorders or intoxications Saudi Arabia

A study of non-motor manifestations in patients with amyotro- Background phic lateral sclerosis The differential diagnosis for genetic dystonia parkinsonism is broad. Juvenile onset parkinsonism-dystonia, also known as dopa- A. Chowdhury, A. Biswas, A. Pandit mine transporter deficiency syndrome (DTDS), is an autosomal IPGME&R AND SSKM Hospital, Neurology, Kolkata, India recessive disorder with variable neurological manifestations and age of onset. We describe DTDS with a novel mutation. Introduction and background Amyotrophic Lateral Sclerosis(ALS) is a progressive neurodegen- Design/methods erative disorder of motor neurons. Spreading of the disease Case report pathology to nonmotor brain areas may lead to development of nonmotor symptoms. Most of these symptoms remain under- Results diagnosed, leading to significant distress to the patient. So detection We identified a 19-year-old male, who presented with history of and treatment of these nonmotor symptoms are essential for juvenile-onset parkinsonism. He had a normal birth and neonatal comprehensive care of such patients. This Study, which is probably course with normal developmental milestones. Cognitive, motor and the first of such kind in India, aims to investigate the various speech were reported to be normal in the first decade of life. At 10– nonmotor symptoms in ALS patients. 11 years of age, the patient started to have tremor and progressive ARTICLE IN PRESS

344 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx parkinsonian symptoms. He additionally starts to have speech WCN19-2174 difﬁculties. The parents are consanguineous, but there is no family history of movement disorders, Parkinson’s disease or other psychiatric illness. Other siblings (brother and sister) are unaffected. Poster shift 03 - Autonomic nervous system disorders/history of Neurological examination revealed loss of speech to single words, neurology/motor neuron disease/movement disorders/MS & rigidity, generalized bradykinesia, dystonia with dystonic tremor. demyelinating diseases/neuromuscular disorders/vestibular dis- Gene analysis showed a novel homozygous mutation in SLC6A3 gene orders and nutritional disorders or intoxications (g.1394863c N G) with both parents and siblings having a heterozygous mutation in the same gene. Compromise of self awareness and disease perception in multiple sclerosis Discussion/conclusions • DTDS is a rare disorder that should be considered in the E. Reicha, C. Torresa, E. Halaca, E. Ariasa, S. Plessb differential diagnosis of genetic parkinsonism of juvenile-onset. aHospital Municipal Julio Mendez, Neuroscience, Buenos Aires, Affected individuals show hyperkinesia with orolingual and Argentina limb dyskinesia, dystonia, and chorea with parkinsonian bNeuropsychology Department- Basel Hospital- Switzerland, Neurosci- features. ence and Neuropsychology, Basel, Switzerland • Atypical onset in adolescence or adulthood has been described. • SLC6A3 encodes the dopamine transporter (DAT) that is Background expressed predominantly within the substantia nigra and in Recognition of deﬁcits in MS is frequently dissociated from the the midbrain, which has a crucial role in mediating reuptake clinical picture. Anosognosia (impaired ability to recognize the and homeostasis of dopamine. presence or severity of disease) is well known in cortical processes, but poorly studied in MS. doi:10.1016/j.jns.2019.10.1456

Objective To assess the ability of deficit recognition and the prevalence of anosognosia in Multiple Sclerosis (MS) patients. WCN19-2172 Methods fi Poster shift 03 - Autonomic nervous system disorders/history of 43 patients with de nite MS were studied. Mean age was 36.2 and clinical picture was RR in 28 cases. Patients were evaluated with neurology/motor neuron disease/movement disorders/MS & clinical and neuropsychological standards. Specific scales to evaluate demyelinating diseases/neuromuscular disorders/vestibular dis- anosognosia were the Visual-Analogue Test assessing anosognosia orders and nutritional disorders or intoxications (VATA-m) and the Mayo-Portland Adaptability Inventory (MPAI). Severity was measured with the Bisiach Scale. Coincidences and A case report of delayed diagnosis and treatment of paediatric discrepancies between caregivers and patients in IADL were studied. multiple sclerosis: A lesson from paediatric neurology Correlation between MRI number, size, severity and localization of cerebral lesions was made. N. Waill Saadia, Q. Abed Fahadb aCollege of Medicine/Baghdad University, Pediatric, Baghdad, Iraq Results bConsultant radiologist- Baghdad Teaching Hospital, Medical City We observed a high prevalence of anosognosia (58%), with strong Complex – Baghdad /Iraq, Radiology, Baghdad, Iraq dissociation when scales were administered to patients and caregivers. The grade of anosognosia was 1 or 2 and the awareness of Multiple Sclerosis (MS) is a chronic inflammatory autoimmune impairment was mainly observed in neurocognitive tasks and fine disease of the central nervous system (CNS). Up to 10% of MS motility skills. Problems in IADL were underestimated. Our findings patients have onset in paediatric age group. Although the clinical correlated with EDSS, evolution time and the presence of a large profile of MS appears similar to that seen in adults, several features number of lesions located in the right temporo-parietal subcortical may differ and specific issues arise in children. Here, we reported a areas. No relationship with other variables was observed. 12-year old girl who presented with 3-year history of episodes of seizures and facial nerve palsy and finally fatigue and cognitive Conclusions impairment were noted and interfered with her academic perfor- Anosognosia is a highly prevalent disorder in MS, suggesting a mance and quality of life. Because of the presumed atypical clinical dysfunction in the mechanisms of self awareness, with implications presentations, the diagnosis was missed then dismissed despite in the ADL and with predictive value on poor functional outcome. neuroimaging features and CSF immunological findings that were MRI studies revealed more often localization of lesions in right highly suggestive of MS. Later, evolution of the disease by parieto- temporal hemispheric topography. neuroimaging helped confirming the diagnosis and directed toward the delayed therapy. doi:10.1016/j.jns.2019.10.1458 doi:10.1016/j.jns.2019.10.1457 ARTICLE IN PRESS

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WCN19-2187 Background Dermatomyositis is a rare autoimmune disease with primary involvement of skin, muscle and different organs. It is important to Poster shift 03 - Autonomic nervous system disorders/history of rule out overlap syndromes and hidden neoplasms. There is not neurology/motor neuron disease/movement disorders/MS & enough data in Colombia regarding of this disease. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Objectives To describe demographics, clinical characteristics, associations Description of a dermatomyositis in a cohort of Colombian and treatment used in a cohort of dermatomyositis patients from patients Colombia and compare them with foreign cohorts.

A.M. Ruiz Menesesa, J.H. Cajamarca Barona, E.J. Duran Moralesb, M.A. Materials and methods Silva Solera, A.M. Arredondo Gonzáleza A retrospective descriptive study was performed with records aHospital de San José, Bogotá capital district, Bogotá, Colombia from january 2016 and to june 2019 of patients from the Deparment bFundación Santa Fé de Bogotá, Capital district, Bogotá, Colombia of Rheumatology and Neurology of a teaching hospital in Bogotá, Colombia. ARTICLE IN PRESS

346 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Results in those who had a complete medical history with prior informed 32 patients were included in this study.Demographics, clinical consent, followed by a physical examination focused on neurological features and paraclinicals are summarized on Tables 1 and 2. It was examination and anthropometric measures. Blood samples were found a higher prevalence in females, with the median age of 49.The taken for genetic studies, which are in process. amyopathic pattern was less frequent,however, an study in Singa- pore has demostrated that this subphenotype is more common than Conclusions thought previously. In our study all patients had pathognomonic skin The diagnosis has been clinically conﬁrmed and through genetic lesions, the most frequent were gottron's papules(75%), similar studies. Additionally, risk factors were identiﬁed, especially consan- results were found in a study in China.The antisynthetase syndrome guinity, to increase the prevalence of the disease. (12%) was less frequently compared to other studies where it is estimated approximately 30%, and neoplasms were higher when doi:10.1016/j.jns.2019.10.1460 compared with 14% found in a systematic review. The most used treatment was steroids, as in other studies.

Conclusions WCN19-2202 We found similar characteristics between a cohort of Colombian patients with dermatomyositis and foreign cohorts reported on the literature. Amiopathic pattern and antisynthetase syndrome were Poster shift 03 - Autonomic nervous system disorders/history of less frequent. Hidden neoplasms were more frequent when com- neurology/motor neuron disease/movement disorders/MS & pared to European, Asian and American cohorts. demyelinating diseases/neuromuscular disorders/vestibular dis- ****I have obtained IRB approval. orders and nutritional disorders or intoxications

Clinical characteristics and proﬁle of patients suffering from doi:10.1016/j.jns.2019.10.1459 multiple sclerosis eligible for treatment with ocrelizumab and their treatment satisfaction level

I. Barbov, T. Petkovska-Boskova, I. Kuzmanovski, N. Tanovska, B. WCN19-2196 Boskovski, V. Bojkovski University Clinic for neurology, Multiple Sclerosis, Skopje, Macedonia Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Background Multiple sclerosis (MS) is an immune-mediated inﬂammatory demyelinating diseases/neuromuscular disorders/vestibular dis- demyelinating disease that represents a leading cause of disability in orders and nutritional disorders or intoxications young adults. The treatment is primarily focused on disease- modifying therapies (DMTs). There is lack of information about Characterization of an endogamic Colombian family with steinert clinical proﬁle of MS patients eligible to receive ocrelizumab and myotonic dystrophy their treatment satisfaction level.

a a a a a N. Gomez , O. Luján , M.F. Bustos , J. Bendeck , A. Arias ,M. Objective a a a a a Castellanos , C. Torres , C. Prieto , D. Polania , M. Martinez , M.C. Our primary objective was to assess the MS patient’s profile a a b a Casas , M. Estrada , I. Fernandez , L. Celis Gustavo eligible to receive ocrelizumab; the secondary was to investigate the a Universidad de la Sabana, Medicine, Bogota, Colombia level of patient’s treatment satisfaction from ocrelizumab. bPoliclinica Metropolitana, Unidad de Genética Médica, Caracas, Venezuela Methods A non-interventional, prospective study was conducted at Background University Clinic of Neurology in Skopje. A questionnaire was Myotonic dystrophy is a multisystemic disease characterized by developed in order to obtain patient’s characteristics and treatment disabling loss of muscle mass. Two main types of this disease have satisfaction, excluding any personal data. been described: Steinert's disease or myotonic dystrophy type I (DM I) and myotonic dystrophy type II or Ricker's syndrome (DM II). The Results IMD is an autosomal dominant inheritance pattern where the The study enrolled 75 MS patients eligible to receive ocrelizumab, anticipation phenomenon occurs characteristically. This refers to a whereby 88% were diagnosed with RMS (relapsing multiple progressive increase in the severity and / or decrease in the age of sclerosis) and 12% with PPMS (primary progressive multiple establishment of the disease in the following generations of a family. sclerosis). 55% of the study cohort were females; mean age was 39.4 years (range 19–58) and mean EDSS score was 3.8 (range 1–6). Methods 23% of RMS patients were treatment naïve, the rest were eligible for An inbred family is presented, from the municipality of Ricaurte, ocrelizumab as second line treatment. 43 out of 75 patients were on Cundinamarca in Colombia comprised of members who present treatment with ocrelizumab while 42 are expected to be treated in similar symptoms characterized by progressive muscular weakness the near future. 62% of the treated patients have denoted to be of the four members, gait disturbance, with subsequent immobility, satisfied from their treatment. severe disability and death. Conclusions Results Ocrelizumab as a highly efficient and well tolerated DMT is Two members of the family with a greater progression of the becoming standard of care under the current clinical practice for a disease, with greater impact on the quality of life, were emphasized wide spectrum of MS patients with different clinical and ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 347 demographic characteristics offering unprecedented benefits for WCN19-2209 quality of life improvement. doi:10.1016/j.jns.2019.10.1461 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications WCN19-2206 Technical accuracy of O-ARM frameless DBS without fiducial- markers Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & T. Tufoa, F. Boveb, B. Cionia, A. Bentivogliob, C. Pianob demyelinating diseases/neuromuscular disorders/vestibular dis- aPoliclinico Gemelli Foundation, IRCSS University Hospital, Neurosur- orders and nutritional disorders or intoxications gery, Rome, Italy bPoliclinico Gemelli Foundation, IRCSS University Hospital, Neurology, Assessment of autonomic nervous system function in type 2 Rome, Italy diabetes a war of new era The aim of our study was to investigate the accuracy of targeting V. Selvaraja, O. Mantrob using intraoperative O-arm during deep brain stimulation (DBS) aSaveetha Medical College Hospital-Chennai, Neurology, Chennai, India surgery without fiducials. bSaveetha Medical College Hospital-Chennai, Medicine, Chennai, India Material and methods Background twelve patient with advance PD were implanted bilaterally with The scope of our study is the early identification of Diabetic 24 deep brain stimulation leads using a modified frameless Autonomic Neuropathy (DAN) which is either clinically evident or stereotaxy methods. Implantation of the leads were performed using sub clinical using simple bedside clinical tests and neuro electro- a Nexframe system without the use of fiducial and coregistration of physiology techniques as there were only a few Indian studies the system in an intraoperative O-ARM acquired volumetric studies. addressing this issue. Preoperative MRI based plan were compared with post-procedure intraoperative location of the lead on O-ARM acquired volumetric Materials and methods images. All implanted leads euclidean localization error (vector error, A total of 66 patients enrolled in the study, done in Saveetha VE) was calculated by the root mean square sum formula: [x2 + y2 Medical College hospital, Chennai, Tamilnadu for a period of 6 + z2]1/2 the relative standard deviation, as well the error of each months after applying the inclusion and exclusion criteria,all the side. patients had type 2 DM of more than 5 years duration, all were above 25 years and below 80 years of age. Basic demographic details Results collected,blood investigations,ECG were done. Peripheral nervous The mean euclidean localization error for all 24 leads was system was assessed by questionnaire,clinical neurological examina- 2.09 mm ± 0.71, whether the mean VE for the right sided implanted tion and nerve conduction study. Autonomic function study was leads was 2.31 mm ± 0.47 and for the left sided implants 1.87 mm done by Cardiovascular Autonomic Reflex Function Tests(Ewing’s ± 0.86. battery of five tests) and sympathetic Skin Response (SSR) test. Discussion Results The accuracy of this new framless method when compared with Diagnosis of cardiac autonomic neuropathy was observed in 48.48% previous Nexframe using bone fiducials or frame based doesn’t differ patients.27.72% of patients showed positive results for expiration to significantly. inspiration ratio,39.39% were found to have 30:15 ratio positive, 37.87% have abnormal valsalva ratio,9.09% had positive results for Conclusion sustained hand grip, only 6.06% of patients had postural hypotension, The new described approach in our early experience suggest Parasympathetic involvement is earlier as compared to sympathetic advantages in term of patient compliance and decreased operating involvement. A statically significant correlation was found between time. This features impact positively outcome and procedural Nerve Conduction Study and ANS study (p-value is 0.00015). An satisfaction in patient with advance PD. increasing HBA1C correlates well with early ANS involvement. doi:10.1016/j.jns.2019.10.1463 Conclusion Recommendations to use simple bed side non-invasive ANS tests helps in early detection of Cardiac autonomic neuropathy (CAN) and prompt early intervention would be clinically meaningful. doi:10.1016/j.jns.2019.10.1462 ARTICLE IN PRESS

348 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-2211 C. Kramarza, V. Alakbarzadeb, A. Turnerb, B. McLeanb aUniversity of Derby and Burton NHS Foundation Trust, Department of Neurology, Derby, United Kingdom Poster shift 03 - Autonomic nervous system disorders/history of bRoyal Cornwall Hospital NHS Trust, Department of Neurology, Truro, neurology/motor neuron disease/movement disorders/MS & United Kingdom demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy in adults is an autosomal dominant disorder caused by a High-frequency spinal cord stimulation (HF-SCS) improves motor cytosine-thymine-guanine (CTG) repeat expansion in the 3′ untrans- as well as cognitive performances in a case of hereditary spastic lated region of the myotonic dystrophy protein kinase (DMPK) gene paraplegia interfering with the expression of many other genes1. Therefore, DM1 has a wide phenotypic spectrum, ranging from T. Tufoa, A. Danieleb, A. Bentivogliob, B. Cionia, C. Pianob oligosymptomatic forms to the life-threatening, multi-system dis- 1 aPoliclinico Gemelli Foundation - IRCSS University Hospital, Neurosur- ease . Overall, DM1 patients have a reduced life expectancy, mainly 2 gery, Rome, Italy due to respiratory or cardiac causes . To date, there is no curative bPoliclinico Gemelli Foundation - IRCSS University Hospital, Neurology, treatment for DM1; however, prompt, appropriate symptomatic Rome, Italy treatments are essential to limit disease-related complications.We present a case of subclinical DM1 manifested with recurrent type 2 We report a case of complicated hereditary spastic paraplegia respiratory failure. treated with spinal cord stimulation. DM1 ought to be considered in any patient presenting with A 40 year-old man with slowly progressive spastic paraparesis, neurogenic respiratory failure, due to the need for prompt recogni- moderate cognitive deficit,was submitted to the implant of a cervical tion and treatment with non-invasive ventilation in order to reduce epidural electrode for the neuromodulation of spasticity.After trial patient mortality. with 80Hz stimulation,patient referred some motor improvement, Family history is essential, even in the patients with acute but unpleasant paraesthesiae.We tested 10000Hz stimulation that manifestation. We did not consider DM1 as a probable diagnosis fi does not induce paraesthesiae.Before and after15days:neurological due to insuf cient initial medical and family history. status (Ashworth scale,Albert scale,VAS,Hamilton depression scale), doi:10.1016/j.jns.2019.10.1465 gait analysis,quality of life (SF-36),neuropsychological evaluation, brain single photon emission computer tomography(SPECT)with HMPAO to assess regional cerebral perfusion. The patient during HF-SCS referred a better endurance, walk and could lay down and stand up faster, with more fluent language,and a WCN19-2218 better vescico-sphincterial control.Ashworth scale decreased from 20 to 17,Albert scale improved from 143 to 149.Increase of gait speed by Poster shift 03 - Autonomic nervous system disorders/history of 26%, increased step length (by 40.8%),decreased double support(by neurology/motor neuron disease/movement disorders/MS & 43.5%).Kinematic analysis showed hip and knee decreased flexion. demyelinating diseases/neuromuscular disorders/vestibular dis- The Hamilton scale showed no signs of depression.The SF-36 improved,particularly the Physical Health Index (from 33 to 43). orders and nutritional disorders or intoxications The neuropsychological studies showed an improvement of dysarthria,marked improvement in the test of word reading,in the visual JNK as a biomarker of relapse and response to treatment in comprehension of names,denomination of names,constructive praxia multiple sclerosis tests along with a mild improvement in MMSE and short term memory.The brain SPECT during SCS showed an increased perfusion H. Rus, F. Anselmo, A. Tatomir, B. Boodhoo, V. Rus in the right frontal and temporal cortex and in the left fronto- University of Maryland, School of Medicine, Neurology, Baltimore, USA temporal cortex. The patient was submitted to chronic stimulation.The motor and Multiple sclerosis (MS) is an inflammatory demyelinating disease cognitive improvement was confirmed at the 12 months follow up of the central nervous system, with its pathogenesis mediated in part visit. by apoptosis-resistant autoreactive T cells. In this study, we HF-SCS improves motor as well as language and cognitive longitudinally investigated the role of JNK and phosphorylated Bcl- performances without inducing disturbing paraesthesiae in a case 2 (p-Bcl-2) as possible biomarkers of MS relapse and of glatiramer of complicated hereditary spastic paraplegia. acetate (GA) therapeutic response in relapsing-remitting MS (RRMS) patients. A cohort of 15 GA-treated patients was clinically monitored doi:10.1016/j.jns.2019.10.1464 and peripheral blood mononuclear cells were collected at 0, 3, 6, and 12 months after initiating GA therapy. We measured JNK1, JNK2, phospho-JNK and p-Bcl-2 expression by western blot analysis. We found that during relapse, patients had significantly higher levels of WCN19-2213 JNK1 p54 (p b 0.0001) and JNK2 p54 (p = 0.006) and significantly lower levels of p-Bcl-2 (p = 0.003) than did stable patients. Significantly higher levels of JNK1 p54 (p = 0.009) and JNK2 p54 Poster shift 03 - Autonomic nervous system disorders/history of (p = 0.001) and significantly lower p-Bcl-2 levels (p = 0.004) were neurology/motor neuron disease/movement disorders/MS & found in GA-treated patients who failed to achieve no evidence of demyelinating diseases/neuromuscular disorders/vestibular dis- disease activity (NEDA) 3 status. Using receiver operating character- orders and nutritional disorders or intoxications istic analysis the probability of accurately detecting relapse was 92% (p b 0.0001) for JNK1 p54, 79% (p = 0.004) for JNK2 p54, and 95% for Unusual presentation of myotonic dystrophy: case report and p-Bcl-2 (p = 0.008). The probability of detecting response to GA was literature review 75.5% for JNK1 p54 (p = 0.01), 83.4% (p = 0.0008) for JNK2 p54 and 94.6% for p-Bcl-2 (p = 0.004). Our data suggest that JNK1, JNK2, and ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 349 p-Bcl-2 could serve as potential biomarkers for MS relapse and during year 1 (20.1%), compared to those in SD group (8.9%). The therapeutic response to GA risk of losing NEDA comparing ED vs SD using Cox model showed the non-inferiority of ED regimen (HR: 1.03, 95% CI: 0.7–1.3, p = 0.9; doi:10.1016/j.jns.2019.10.1466 HR: 1.46, 95% CI: 1.0–2.0, p = 0.6); while MSMs did not (HR: 1.36, 95% CI: 1.0–1.8, p = 0.03). When comparing MD vs SD the analysis favored the SD group.

WCN19-2229 Conclusions Given the lack of consistency among the various methodologies used, there is no sufﬁcient evidence that ED regimen might reduce Poster shift 03 - Autonomic nervous system disorders/history of effectiveness of Natalizumab compared to SD. Differences in neurology/motor neuron disease/movement disorders/MS & effectiveness between ED and SD lessen with increasing treatment demyelinating diseases/neuromuscular disorders/vestibular dis- duration. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1467 Different regimen of natalizumab treatment in multiple sclerosis patients: A real world study in Italy

S. Ruggieria, A. Ianniellob, L. De Giglioc, M. Altieria, D. Centonzed,M. WCN19-2234 Copettie, A. Cortesea, R. Fantozzif, S. Galganig, C. Gasperinig,L. h d fi d i b i Grimaldi , D. Landi , G.A. Mar a , M. Mirabella , R. Nistri , V. Nociti , Poster shift 03 - Autonomic nervous system disorders/history of O. Oddoh, S. Romanoj, G. Salermik, M. Salvettij, C. Pozzillia neurology/motor neuron disease/movement disorders/MS & aSapienza University of Rome, Department of Human Neurosciences, demyelinating diseases/neuromuscular disorders/vestibular dis- Rome, Italy bSant'Andrea Hospital, MS Center, Rome, Italy orders and nutritional disorders or intoxications cSan Filippo Neri Hospital, Multiple Sclerosis Center- Neurology Unit, Rome, Italy Fused in sarcoma (FUS) pathology observed in an autopsy case of dUniversity of Rome "Tor Vergata", Department of Systems Medicine- ALS/MND-plus clinical syndrome Multiple Sclerosis Unit, Rome, Italy eIRCCS "Casa Sollievo della Sofferenza" Hospital, Unit of Biostatistics, San K. Asaia, H. Sumi-Akamarua, A. Nishikawaa, E. Shirahataa,R. Giovanni Rotondo, Italy Yamashitaa, N. Miyashitaa, H. Mochizukib, T. Nakaa fIRCCS Neuromed, MS Center, Pozzilli, Italy aHigashi-Osaka City Medical Center, Department of Neurology, Higashi- gSan Camillo-Forlanini, Department of Human Neurosciences- MS Osaka city, Japan Center, Rome, Italy bOsaka University, Department of Neurology, Osaka city, Japan hFondazione Istituto "G. Giglio", Neurology and Multiple Sclerosis Center, Cefalù, Italy We present an autopsy case of sporadic amyotrophic lateral i Fondazione Policlinico Universitario "A. Gemelli" IRCCS- Università sclerosis, who showed the vertical gaze palsy when she used the Cattolica del Sacro Cuore, MS Center, Rome, Italy eye ball movement and the blink for the communication. j Sapienza University of Rome, Center for Experimental Neurological Clinically, the pyramidal sign nor the dementia episode was Therapies- NESMOS Department, Rome, Italy observed, so she was diagnosed as progressive muscular atrophy. k University of Palermo, Department of Biomedicine- Neuroscience and She noticed the upper and lower limb weakness at the age of 69 Advanced Diagnostics, Palermo, Italy and could not walk from the age of 71. At the first neurological examination, the muscle weakness and atrophy of the tongue and Background four limbs was apparent. She required the mechanical ventilation To minimize the risk of PML in patients with Multiple Sclerosis and the gastric fistula from the age of 73. She died of the (MS) treated with Natalizumab, an extended-interval dose (ED) of pneumoniae at the age of 78. Post mortem examination showed 35 days or longer has been proposed. fused in sarcoma (FUS) pathology. Brain weight was 1280 g. The lower motor neurons in the spinal cord and the brain stem and Objective the upper motor neurons were severely degenerated. A lot of To assess the efficacy of ED vs Standard dose (SD) in terms of round basophilic inclusions were demonstrated widely in the annualized “no evidence of disease activity-3” (NEDA). upper and lower neurons. They were positive for FUS/P62 and phosphorylated neurofilament and negative for TDP-43. FUS/P62- Methods positive cytoplasmic round inclusions were observed widely in This is an independent, multicenter, retrospective study. Groups non-motor neurons including in the striatum, the subthalamic were defined: 1) SD: ≤35 days; 2) ED: N35 days and ≤56 days; 3) nucleus, the thalamus, the superior colliculus, the pontine nucleus, Mixed dose (MD): patients receiving SD for ≥12 to ≤24 months then the inferior olive nucleus, the dentate nucleus of the cerebellum, switched to ED. Univariate and multivariate Cox proportional hazard and scarcely in the other cerebral cortex than the primary motor regression models with time-dependent exposure and marginal area. The degeneration of superior colliculus was especially severe structures models (MSMs) were used. in addition to that of the motor systems. Progressive supra palsy- like distribution of FUS proteinopathy might be associated with Results the vertical gaze palsy. 775 patients were included in the analysis, 318 patients completed 4 years of follow-up. During the first year of treatment, NEDA was achieved by 80% of SD patients and 71.2% of ED patients doi:10.1016/j.jns.2019.10.1468 (p = 0.03). A greater proportion of patients in the ED group relapsed ARTICLE IN PRESS

350 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-2237 Objective To investigate trends in the incidence of Parkinson’s disease (PD) Poster shift 03 - Autonomic nervous system disorders/history of between 2006 and 2016. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Design orders and nutritional disorders or intoxications Cohort study in The Health Improvement Network (THIN): a large UK primary care database. Depression in Parkinson’s disease: Study of 38 algerian patients Study participants M. Mati Individuals actively registered with a practice within THIN aged Neurologist, Faculty of Medicine of Algiers, Tipaza, Algeria 50 years and over.

Measures of outcome Introduction The incidence of Parkinson’s disease between 2006 and 2016 Depression (D) is one of the most common neuropsychiatric using different case deﬁnitions: 1) PD diagnostic Read code; 2) PD disorders in Parkinson's disease(PD). diagnostic Read code OR symptom Read code; 3) PD diagnostic Read It is estimated around 20 to 50%. code OR symptom Read code and OR at least 1 antiparkinsonian drug The diagnosis of depression remains clinical. prescription; 4) PD diagnostic Read code OR symptom Read code plus at least 2 antiparkinsonian drug prescription. The effect of age Patients and methods and sex on these measures were investigated. To evaluate the frequency of depression in a population of Algerian patients living in the city of Tipaza with (PD) a prospective Results study on 38 patients followed by the specialized consultation in The overall crude incidence of PD for people over 50 years has neurology. increased in recent years. The rates increased with increasing age Screening for depression was based on the Hamilton Scale (HAM- and as expected, higher in men. Using the broadest case deﬁnition D) and the Geriatric Depression Scale (GDS). The quality of life (3), incidence rate of PD between 2006 and 2016 in men was 190 per assessment was based on the self-reliance scale. Katz. as well as a 100,000 person years (95% CI 187–193) and in women was 176 per clinical examination with UPDRS. 100,000 person years (95% CI 173–178). Incidence of PD was highest The exploitation of the results is done using EPIDATA. at age 90 (496 per 100,000 95% CI 471–522). Result Conclusion In our study, the frequency of depression in patients with PD is There is increasing diagnosis of PD in primary care setting. 68.4%. there is no relationship between sex (p = 0.7732) and depression or age (p = 0.7607). Depression can be seen at any stage of evolution. There is no relationship between depression and clinical form (p = 0.1661). We found a relationship between depression and autonomy loss (p = 0.003). These results are consistent with those doi:10.1016/j.jns.2019.10.1470 of the majority of the series reported in the literature.

Conclusion Depression in (PD) is underestimated, hence the need for systematic screening and systematic management to improve the WCN19-2253 quality of life of the patient. Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1469 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-2251 A rare case of balo’s concentric sclerosis mimicking stroke

Poster shift 03 - Autonomic nervous system disorders/history of A. Vidanagamagea, A.A. Chandrakumarab, A. De Silvab, A. Fernandoa a neurology/motor neuron disease/movement disorders/MS & National Hospital, Neurology, Colombo, Sri Lanka b demyelinating diseases/neuromuscular disorders/vestibular dis- National Hospital, Radiology, Colombo, Sri Lanka orders and nutritional disorders or intoxications Introduction Balo’s concentric sclerosis (BCS) is a rare variant of multiple Trends in the incidence of Parkinson's disease between 2006 and sclerosis which could present like a stroke. Early differentiation is 2016: Analysis of a large primary care database important in the management.

O. Okunoyea, A. Schraga, K. Waltersb, L. Marstonb Case report aUniversity College London, Clinical and Movement Neurosciences, A 56 years old male presented with progressive left hemiparesis London, United Kingdom with dysarthria over one week duration. He was imaged with NCCT bUniversity College London, Primary Care & Population Health, London, brain and was managed as for an ischemic stroke. United Kingdom ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 351

Three weeks later he presented with aphasia with altered level of administration of the drug. This might give us an insight into the consciousness. mechanisms behind CBD. Methylphenidate can prove to be a wonder On examination there was generalized hypertonia with positive drug in such cases, however, since the data available is insufficient Babinski sign bilaterally. There was asymmetric limb weakness with and large lacunae in pathophysiological mechanisms behind CBD left side more than right with upper motor type bulbar weakness and persist leaving room for more studies in this area so that this normal pupils and fundi. progressively debilitating disease can be taken care of. NCCT brain demonstrated a few low density round lesions involving the bilateral centrum semiovale. MRI scan confirmed these doi:10.1016/j.jns.2019.10.1472 as consisting of irregular concentric bands of hypo and hyper intensities in both T1 and T2 sequences. The periphery of the lesion demonstrates contrast enhancement. No diffusion restriction was noted. WCN19-2255 CSF protein was 50 g/dl with normal cells and sugar. CSF was positive for oligo clonal bands. Rest of the basic evaluation was normal. Further imaging for visceral malignancy was negative. He Poster shift 03 - Autonomic nervous system disorders/history of was managed with high dose steroids and plasma exchange with neurology/motor neuron disease/movement disorders/MS & recovery of conscious level with residual weakness. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Conclusion Atypical presentations of stroke need evaluation with advanced Concomitant synaptic impairment of neuromuscular junction imaging. BCS is a rare variant of multiple sclerosis (MS) with a and axonal polyneuropathies pathologic hallmark of concentric rings of demyelination. Knowledge of the typical MRI appearance leads to an early diagnosis. M.S.E. Elmagzouba, K. Sultana, H. Abdelraheemb, S. Misbahc,A. Ahmedd doi:10.1016/j.jns.2019.10.1471 aFaculty of Medicine, Ribat University, Neurophysiology, Khartoum, Sudan bFaculty of Medicine, Ribat University, Medicine, Khartoum, Sudan cFaculty of Medicine, Khartoum University, Medicine, Khartoum, Sudan WCN19-2254 dFaculty of Medicine, Khartoum University, Neurophysiology, Khartoum, Sudan Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Peripheral axonal neuropathy (PAN) is a progressive axonopathy marked by loss of motor, sensory and autonomic fibers with reduced demyelinating diseases/neuromuscular disorders/vestibular dis- stability of neuromuscular transmission. In a previous work in orders and nutritional disorders or intoxications demyelinating neuropathies, we showed that repetitive nerve stimulation (RNS) induced significant decremental responses in Methylphenidate - Straightens the Pisa, re-familiarizes the alien, amplitude and area of muscle action potentials (CMAPs). corrects the apraxia In this work we studied prospectively, 111 diagnosed patients (clinically and electrophysiologically) with PAN, by performing RNS a b N. Sawal , J. Kaur to assess the degree of weakened synaptic transmission at the NMJ a Government Medical College and Hospital- Sector 32- Chandigarh, and find out whether it is one of the aspects of PAN. General Medicine, Chandigarh, India Males constituted 61 patients while females are 50 with a mean b GMCH Sector 32 Chandigarh, General Medicine, Chandigarh, India age of 56 years. Nerve conduction study (NCS) and Electromyogram (EMG) revealed that patients with sensorimotor axonal neuropathy Corticobasal degeneration (CBD) is a rare, progressive neurolog- (n75), motor (n28) and the least (n8) were those with pure axonal ical disorder. The patients also show cognitive and behavioural sensory neuropathy. abnormalities and asymmetric atrophy of frontal and parietal cortical RNS of both axillary, musculocutaneous and Left Ulnar nerves, regions of the brain. Clinically it might manifest with features of showed 87%, 80% and 28% decrements in amplitude and 91%, 84% cortical involvement such as apraxia, parkinsonism, impaired eyelid and 43% in area for axillary, musculocutaneous and Left Ulnar nerves, or ocular motion. 'Alien limb' is a common initial symptom and a respectively in 85 patients. This would suggest lower safety factors in part of motor manifestation of the disease. The management mainly neuromuscular transmission. The proximal nerves were significantly relies upon treating the manifestations of the disease. We came more sensitive in revealing the deficit than the distal ulnar nerve. across three cases of CBD presenting as Pisa Syndrome, 'alien limb' and apraxia of eyelid opening. Pisa syndrome or pleurothotonus is a Conclusion form of atypical acute or tardive dystonia presenting with tonic, The highly significant decrements of CMAPs in this study, could reversible, lateral flexion of the trunk of at least 100. 'Alien limb' is an be attributed to disorders of peripheral nerves at the presynaptic intermittent involuntary movement disorder associated with com- level, induced by axonal loss. This leads to deficient exocytosis of plex, goal-directed activity in one hand that is not voluntarily Acetylcholine at the NMJ. Hence, this lack of availability of initiated. This is a common initial motor manifestation of CBD. The neurotransmitter would not be secure enough to stimulate the third patient presented with inability to open the eyelids for one and nicotinic postsynaptic receptors. a half year. We treated these patients with Methylphenidate which is a reuptake inhibitor of dopamine and norepinephrine. All three doi:10.1016/j.jns.2019.10.1473 patients partially responded with improvement in symptoms after ARTICLE IN PRESS

352 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-2268 bUniversity Erlangen-Nuremberg, Dept. of Neurology, Erlangen, Ger- many cParacelsus Medical University Nuremberg, Dept. of Psychiatry & Poster shift 03 - Autonomic nervous system disorders/history of Psychotherapy, Nuremberg, Germany neurology/motor neuron disease/movement disorders/MS & dIuliu Hațieganu University of Medicine & Pharmacy, Roneuro Institute demyelinating diseases/neuromuscular disorders/vestibular dis- Center for Research and Diagnosis of Neurological Diseases, Dept. of orders and nutritional disorders or intoxications Neurosciences, Cluj-Napoca, Romania eTongji Hospital & Medical College, Dept of Neurology, Wuhan, Hubei Myasthenia gravis with ptosis, ice pack test in the absence of Province, China edrophonium: Two case reports and review of the literature Background P.Y. Bassonga, S. Laah Njoyob, A.D. Magang Sadoc, N.E. Eyolec, N.C. Patients with a history of mTBI (post-mTBI-patients) may have Mbapehc long-lasting changes in autonomic responses to emotional stimuli. aUniversity of Bamenda, Faculty of Health Sciences, Providence Happy or fearful music triggers autonomic responses. So far, these Polyclinic, Neurology, Bamenda, Cameroon responses have not been evaluated in post-mTBI-patients. bUniversity of Bamenda, Faculty of Health Sciences, Providence Polyclinic, Radiology, Bamenda, Cameroon Objective cProvidence Polyclinic, Neurology, Bamenda, Cameroon To assess cardiovascular-autonomic responses to happy and fearful music in post-mTBI-patients. Background and objectives Myasthenia gravis (MG) is a rare autoimmune disease of the Methods neuromuscular junction. It is characterized by weakness of one or In 24 post-mTBI-patients (35.3 ± 2.7 years; 6 women, 32.8 ± 5.6 several of the following muscle groups: ocular, bulbar, respiratory months post-injury) and 26 healthy persons (30.4 ± 2.3 years; 10 and the muscles of the limbs. Edrophonium test brings additional women), we monitored respiration, RR-intervals (RRIs), systolic and argument to the MG diagnosis. In some hospital settings lacking diastolic blood pressure (BPsys, BPdia) before and during presentation “ ” Edrophonium, ice pack test, a safer and bedside test should be of 3-minute excerpts of happy music (Marriage of Figaro, Overture, “ ” considered in MG with ptosis in addition to other tests. The objective K. 492) and fearful music (The miraculous Mandarin, Suite, Op. 19). “ ” “ ” of this study was to highlight the necessity of using all the clinical Participants rated happiness and fear on 1 to 5 Likert-scales. tools available to diagnose MG with ptosis in pharmacologically limited hospital settings. Results Likert-scores assigned to happy and fearful music were similar in “ ” Contents patients and controls. Happy music accelerated respiration only in We report two cases of MG patients with ptosis and a positive ice controls (14.1 ± 0.7 vs 16.0 ± 0.7 bpm), increased BPsys in controls pack test. Only an Electromyography and a contrast chest CT were (134.0 ± 3.5 vs 137.7 ± 3.5 mmHg) and patients (129.6 ± 3.0 vs “ ” available in our hospital and town settings. In this paper, the 134.0 ± 3.2 mmHg), but did not change BPdia and RRIs. Fearful literature review emphasizes on the speciﬁcity and sensitivity of the music did not change respiration or RRIs, increased BPsys in controls ice pack test when compared with the Edrophonium test. (130.9 ± 3.1 vs 135.1 ± 3.3 mmHg) and patients (130.0 ± 3.6 vs 134.3 ± 3.7 mmHg), but increased BPdia only in controls (67.4 ± 1.9 Conclusion vs 68.8 ± 2.1 mmHg). We suggest that, in the absence of available Edrophonium, in addition to other tests, ice pack test should be considered in Conclusion suspected cases of MG with ptosis. Similar emotional ratings of the music-excerpts in both groups suggest that the patients’ emotional perception of “happy” and “ ” ﬁ Keywords: Myasthenia gravis, Neuromuscular junction, Electromy- fearful music was not signi cantly compromised at the time of ography, Edrophonium, Ice pack test study. In contrast, lack of respiration-acceleration with happy music and BPdia-increase with fearful music in the post-mTBI-patients indicates persistent subtle changes in sympathetic processing of musical stimuli even months to years after mTBI. doi:10.1016/j.jns.2019.10.1474 doi:10.1016/j.jns.2019.10.1475

WCN19-2273 WCN19-2279 Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Poster shift 03 - Autonomic nervous system disorders/history of demyelinating diseases/neuromuscular disorders/vestibular dis- neurology/motor neuron disease/movement disorders/MS & orders and nutritional disorders or intoxications demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Altered autonomic responses to happy and fearful music in patients with a history of mild traumatic brain injury Unusual lingual-mandibular continuous myoclonus

M.J. Hilza, R. Wangb, K.M. Höslc, D.F. Mureșanud, M. Liue R. Zaidana, N. Al Zahranib aUniversity Erlangen-Nuremberg, Icahn School of Medicine at Mount aCollege of Medicine, Imam Mohamad Ibn AbdulAziz University, Clinical Sinai New York NY- USA, Depts. of Neurology, Erlangen & New York- Neurosciences, Riyadh, Saudi Arabia NY- USA, Germany bPrince Mohammad Hospital, Medinice/Neurology, Riyadh, Saudi Arabia ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 353

Background conﬁrmed by using CT Scan and diagnosed by neurologist. Other data Lingual-mandibular myoclonus is a very unusual movement obtained from electronic stroke registry. Every patients and/or disorder. patients’ family who refused to be a subject would be excluded. TCD examination was using TD-DOP 9000 equipment with a 2-MHz Materials and methods probe to examined intracranial circulation. We report a very unusual case of continuous myoclonus invloving the tongue in a 34 years old patient. She is a case of cerebral palsy Results with undetermined etiology and she developed right anterior The data obtained from 28 patients, consist of 57.1% male and cerebral infarction. 42.9% female. The age of patients were older than 60 years old in 22 patients (78.6%). The diagnosis of the patients were acute ischemic Results stroke (42.9%), spontaneous subarrachnoid hemorrhage (35.7%), and Consecutively she started to have rhythmic and persistent intracerebral hemorrhage (21.4%). The TCD result showed hypoper- rythmic abnormal movements involving the tongue and the jaw in fusion in 8 patients, stenosis of either anterior circulation in 12 the same time. Her Imaging studies including brain CT scan, MRI and patients, vasospasm in 4 patients, increasing pulsality index that MRA showed occlusion of the right anterior cerebral artery with indicate high intracranial pressure in 6 patients. infarction in whole its territory.The abnormal movements subsided near completely on Depakine PO. The EEG monitoring failed to show Conclusion epileptiform discharge. Intracranial stenosis is the most common hemodynamic distur- The pathophysiology of this exceptionally rare abnormal move- bance in stroke patients, especially in anterior circulation. TCD ments involving both the tonguye and the jaw is discussed. examination give an important information of cerebral hemodynamic in critical patients. TCD is a non-invasive and mobile device, Conclusion therefore it is safe to be performed in critical patients. This is a very unusual presentation of abnormal movement responding to Depakine. doi:10.1016/j.jns.2019.10.1477 doi:10.1016/j.jns.2019.10.1476

WCN19-2037

WCN19-0016 Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ Poster shift 04 - Ataxia and cerebellar disorders/autoimmune epilepsy - Part II/functional disorders and behavioral neurology/ disorders - Part II/CNS infections - Part II/dementia - Part II/ headache/metabolic and mitochondrial diseases/neuro-critical epilepsy - Part II/functional disorders and behavioral neurology/ care/neurogenetics/neuroophthalmology/neurophysiology/neu- headache/metabolic and mitochondrial diseases/neuro-critical rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells care/neurogenetics/neuroophthalmology/neurophysiology/neu- and gene therapy - Part II/training in neurology - Part II/child rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells neurology and neuroimaging and gene therapy - Part II/training in nuerology - Part II/child neurology andneuroimaging Posterior reversible encephalopathy syndrome after massive transfusion in a patient with postpartum vaginal hematoma The use of transcranial doppler for cerebral hemodynamic examination in critical care setting J.H. Parka, W. Shinb aInje University Sanggye Paik Hospital, Inje University College of R.D.L.R. Sanyasia, R.T. Pinzonb Medicine, Departments of Neurology, Seoul, Republic of Korea aDuta Wacana Christian University, School of Medicine, Neurology, bKyungHee Medical Center at Gangdong, KyungHee University School of Semarang, Indonesia Medicine, Department of Neurology, Seoul, Republic of Korea^ bDuta Wacana Christian University, School of Medicine, Neurology, Yogyakarta, Indonesia Objectives Posterior reversible encephalopathy syndrome (PRES) is a clinical Background and radiological syndrome characterized by headache, potentially Cerebral hemodynamic changing is common among acute stroke reversible neurological deﬁcits. PRES has been described in various patients. Transcranial doppler (TCD) is a diagnostic tool to identify clinical settings, such as severe hypertension, eclampsia, and any intracerebral hemodynamic changing. The study of intracerebral chemotherapy. Transfusion related PRES are rare.^ hemodynamic disturbance in stroke patients in Indonesia is limited. Methods Objective We report a patient with postpartum vaginal hematoma who This study aimed to identify cerebral hemodynamic using TCD. manifested PRES after massive packed red blood cell transfusion conﬁrmed by brain MRI and clinical features. Method This study was a cross sectional study, conducted at Bethesda Results Hospital, Yogyakarta, Indonesia. Subjects included in this study meet A 34-year-old woman gravida 1, para 1 admitted for surgery of the following criteria: (i) male/female, (ii) age N18 years, (iii) postpartum vaginal hematoma, rectum repair, sphincter repair. hospitalized in neurology critical care unit, and (iv) had a stroke, Twelve hours ago, she was delivered. After delivery her uterine ARTICLE IN PRESS

354 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx contraction was poor and vaginal bleeding was persistent. On WCN19-0140 admission, her hematocrit was at 27.2%, and her hemoglobin was at 9.0 g/dl. She received 18 packed red blood cell transfusion and 10 packed fresh frozen plasma. Her hematocrit and hemoglobin Poster shift 04 - Ataxia and cerebellar disorders/autoimmune increased 41.1% and 13.9 g/dl, and platelet increased up to 78,000/ disorders - Part II/CNS infections - Part II/dementia - Part II/ ul. After 2 days of the transfusion, she had a left eye ball deviation epilepsy - Part II/functional disorders and behavioral neurology/ and 5 min prolonged confusion. Her blood pressure was normal, and headache/metabolic and mitochondrial diseases/neuro-critical no other contributing factors for PRES were found. Brain MRI showed care/neurogenetics/neuroophthalmology/neurophysiology/neu- regional T2 signal change in right occipital lobe including cortex, and rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells small signal change in left occipital lobe. and gene therapy - Part II/training in neurology - Part II/child neurology and neuroimaging Conclusion In this case, some relation may have existed between late postpartum eclampsia and PRES, but the pathogenesis remains unclear. Snake bite poisoning presenting as pseudo brain death Although blood transfusion is a common procedure with rare neurological complications, great caution should be taken with anemic M. Thakre patients because a rapid elevation in hemoglobin may precipitate PRES. Al Zahra Hospital Dubai, Neurology, Dubai, United Arab Emirates^ doi:10.1016/j.jns.2019.10.1478 Background Krait (elapid snake) bite may be painless, without evidence of flange marks, and the patient or family may be unaware of the bite. This can present with neuroparalysis which may mimic brain death. WCN19-0020 Due to lack of snakebite history and nonavailability of specific diagnostic tests, this diagnosis is challenging for critical care physician, and it requires a high index of suspicion. Poster shift 04 - Ataxia and cerebellar disorders/autoimmune 31 years old indian gentleman, came with history of severe disorders - Part II/CNS infections - Part II/dementia - Part II/ ^ ^ abdominal cramps on waking up^, went to ER and was given injection epilepsy - Part II/functional disorders and behavioral neurology/ diclofenac. ^He went home but was brought back with progressive headache/metabolic and mitochondrial diseases/neuro-critical difficulty in breathing and loss of consciousness. He was immediately care/neurogenetics/neuroophthalmology/neurophysiology/neu- intubated and ventilated. ^Pupils was middilated^, non reacting^,no fl rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells corneal re ex, No dolls eye movement^, no cough^, no gag^,no respiratory effort, are flexia, no limb movements, planter mute. MRI and gene therapy - Part II/training in neurology - Part II/child ^ ^ ^ brain, toxicology screen, CSF study, liver, renal functions, EEG were neurology and neuroimaging ^ ^normal. Anticholinesterase levels and Neostigmine test was negative. ^Family gave history that his mother in law who came to visit her and Diagnostic and management challenges of patients with recur- slept in the place on the floor was admitted with snake bite rent tolosa hunt syndrome poisoning in another facility. We started Anti Snake Venom empirically and after 4^days of being in Glasgow coma scale 3/ 15 N. Kalea, S. Yazıcı Akkasa, S. Omerhocaa, S. Erdogana, İ. Serifoglub,S. started opening the eye with slight eye movements and recovered Asık Nacarogluc fully after 1^week. aBagcilar Training and Research Hospital, Neurology, Istanbul, Turkey bBagcilar Training and Research Hospital, Radiology, Istanbul, Turkey Conclusion cBagcilar Training and Research Hospital, Ophthalmology, Istanbul, Turkey. ^Snake bite poisoning should be considered as a potential treatable cause of acute neuroparalysis even without history. Tolosa Hunt Syndrome (THS) includes painful ophthalmoplegia with periorbital or hemicranial pain, ipsilateral ocular motor nerve doi:10.1016/j.jns.2019.10.1480 palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. The pathology is usualy localised to the region of the cavernous sinus/superior orbital fissure and may be usually due WCN19-0038 to trauma, neoplasm, aneurysm, and inflammation. Rarely patients may present withâ relapsing and remitting course. Comprehensive patient evaluation is essential in establishing the correct diagnosis. Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ Management is usually with ^systemic corticosteroid therapy and sometimes immunosuppression (IS) may be warranted. In this epilepsy - Part II/functional disorders and behavioral neurology/ abstract we aim to present cases with recurrent THS and further headache/metabolic and mitochondrial diseases/neuro-critical discuss management strategies and diagnostic challenges. care/neurogenetics/neuroophthalmology/neurophysiology/neu- Case series:We^would like to report 3 patients (2 females, ages rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells between 36 and^45) with recurrent THS. The relapse ocurred within and gene therapy - Part II/training in neurology - Part II/child the first year after the initial presentation and patients were all neurology and neuroimaging treated with high dose steroids and IS (azaotioprine).^Patients have been relapse free with IS and slow corticosteroid tapering. THS is a diagnosis of exclusion and careful diagnostic approach is warranted. Anticephalgic photoprotective premedicated mask: A report of a successful study of a treatment for migraine and/or tension doi:10.1016/j.jns.2019.10.1479 headaches ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 355

M. Hyson Background UNLV School of Medicine, Neurology, Las Vegas, USA Headache is the most common cause of neurological referral. An International Classification for headache produced by the Interna- Objectives tional Headache Society (IHS) is regularly updated.^ Analyzing an anticephalgic photoprotective mask in conjunction with a topical medication containing bryonia and rhus Objectives toxicodendron in the treatment of migraine and/or tension The aims of the study were to define prospectively the clinical headache. and economic burden of the different types of headache and cranio- cervical pains in 202 patients referred to Benghazi neurological Background clinics. Many clinicians are seeking headache treatment modalities with improved safety profiles. A premedicated mask would serve not Methods only as a delivery system for benign topical medication, but 202 adult Libyan patients with headache referred to the simultaneously provide photorelief and exert external pressure neurology clinics and emergency rooms of Benghazi Teaching which may alleviate vascular headaches by collapsing painfully Hospitals^, Libya, (^80males and 122 females^)^were studied prospec- distended extracranial arteries and reducing peripheral tively both clinically and using a questionnaire. sensitization. Results Methods Stress was a prominent trigger for headache in 91.6^%, the most Thirty-three patients were given masks and tubes of topical common comorbidity was arterial hypertension in 27.7%. The most medication containing the bryonia and rhus toxicodendron. They common single primary headache was migraine in 47^%,followed by were instructed to apply the medication to their frontalis and/or tension headache in 20.8%^, both types occurred in 6.4% patients,ând temporalis regions in the event they should suffer a headache and the most common single causes of ^secondary headaches were apply a photoprotective mask. Furthermore, they were instructed sinusitis 9.4% and cervicogenic headache 8.9%, analgesia excess to take their usual oral or parenteral medications if required for headache occurred in 27.7^%.^ the relief of the headache. They subsequently filled out forms Genetic phenotypes were found in 11.9% patients and epilepsy in rating the degree of relief which they attributed to the topical 16.3%. Most of the patients were highly educated 62.6% but, only medication and the mask using a 0-10 scale. At the interview 42.6% had an economical status consistent with a professional of following the completion of their participation in the study, the their educational level.^ patients were also simply asked if this form of treatment helped The patients who were aware that headache prophylaxis is or not. possible were 31.7^% and 62.3%were imaged. Further analysis of the headache types^, trigger and relieving factors and management was Results performed in this study. Thirty out of 33 patients stated the medication and the mask were effective over and above the normal degree of relief they were Conclusion receiving from their oral and/or parenteral medications. This study Educating students, medical and paramedical staff into the demonstrated a significant efficacy rate (91%) in the treatment of various aspects of the pathophysiology and management of head- migraine and/or tension headache with the anticephalgic mask in aches and facial pains can have huge economical and health-related conjunction with a topical cream containing bryonia and rhus beneficial impact. toxicodendron. Keywords: ^Headache, ^Facial pain, ^Management, ^Types, Benghazi, doi:10.1016/j.jns.2019.10.1481 Libya^

doi:10.1016/j.jns.2019.10.1482 WCN19-0042

Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ WCN19-0058 epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical Poster shift 04 - Ataxia and cerebellar disorders/autoimmune care/neurogenetics/neuroophthalmology/neurophysiology/neu- disorders - Part II/CNS infections - Part II/dementia - Part II/ rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells epilepsy - Part II/functional disorders and behavioral neurology/ and gene therapy - Part II/training in neurology - Part II/child headache/metabolic and mitochondrial diseases/neuro-critical neurology and neuroimaging care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells ﬂ Re ection of the burden of headache and facial pain on the and gene therapy - Part II/training in neurology - Part II/child community and underlying pathologies: A prospective study from neurology and neuroimaging benghazi libya

A novel MT-CO2 M.8277 T N C mitochondrial DNA variations are H.A. El-Zawawi highly associated with aura migraine headache Benghazi Medical Center, Neurological Medicine, Benghazi, Libya^ ARTICLE IN PRESS

356 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

S. Gulera, H. Gürkanb bALneelain University, Community Medicine, Khartoum, Sudan a ̇ c Trakya University, Department of Neurology, Edirne, Turkey International University of Africa, medicine, Khartoum, Sudan^ bTrakya University Faculty of Medicine, Department of Genetics, Edirne,̇ Turkey^ Background Bell's palsy is an acute peripheral facial palsy of unknown cause. Background The condition leads to the partial or complete inability to voluntarily Over the last three decades mitochondrial dysfunction has been move facial muscles on the affected side of the face. Treatments are postulated to be a potential mechanism in migraine pathogenesis. generally designed to improve facial function and facilitate recovery. The study aimed to assess the grade of Bell's palsy before and Methods after the management by using House and Brackmann grading In this cross-sectional study a total of 50 individuals, who had system.^ been diagnosed with aura migraine between^January 2016 and July 2018, at the Neurology Department, Trakya University Medical Methodology Faculty, Edirne, Turkey, and 50 healthy controls were recruited. This was analytic cross sectional multicenter based study. It was μ ^Genomic DNA was isolated by, taking 400 l from the peripheral conducted in most Khartoum State physiotherapy centers (three in venous blood, taken from 50 patients and 50 volunteers for routine hospital and seven in private sectors). The sample size was 70 cases examination. The concentration and purity of the isolated genomic of Bell's palsy. The cases that fulfilled the inclusion criteria were DNA (A260/A280 ratio should be between 1.8 and ^2.0) was collected by a method of total coverage during the working hours. evaluated using the nanodrop device. Standardized administered questionnaire was filled during the interview by a trained physiotherapist. The collected data were then Results analyzed using SPSS software program, version 20.^ As a result of the analysis, mT-DLOOP2_73A N G, mT-DLOOP2_263A N G, mT-DLOOP2_295CN, mT-DLOOP2_310T N C, mT-DLOOP2_311C N T, Results mT-DLOOP2_489T N C, mT-CO2_8277T N C, mT ND6_14470T N C, mT- Most of the patients were females (57.1%), the commonest age N N N – DLOOP1_16126T C, mT-DLOOP1_16519T C, mT-DLOOP2_295C T^, group was 18 40 years (44.3%), left side was more affected (54.3%), mT_DLOOP2_462C N T, mT-DLOOP1_16278C N T between the patient most of the patients were employees (44.3%). About (77%) of the and the control group, thirteen mtDNA variations were determined patients didn't carry any investigation while 58.6% of them captured fi b N statistically signi cant (p ^ ^.05). However, mT-CO2_8277T ^C the disease early morning (57.7%). The patient's facial palsy grade variation CC genotype was found to be higher in the patient group change was statistically significance, it improved after the manage- compared to the control group (p =^.001). Mutant genotypes of the ment from the grade at the beginning of the onset about (0.712) other variations were found to be high in the control group with 95% confidence interval p value (0.000). b compared to the patient group (p ^.05). Conclusion^ Discussion The grade of Bell's palsy before after management in the study fi A high ^prevalence of âura migraine in persons with the MT- showed statistical signi cant difference. It is recommended to N CO2 m.8277 T C mitochondrial DNA variations was ^more conduct longitudinal study to document the progress of Bell's palsy. common. The present study is the first to demonstrate an association between mitochondrial dysfunction and the suscepti- doi:10.1016/j.jns.2019.10.1484 N bility to migraine^with auraîn carrying the MT-CO2 m.8277 T C mutation. Additionally, knowing the level of cytochrome C oxidase in these patients may be predictive of the phenotype/genotype ^relationship. WCN19-0061

doi:10.1016/j.jns.2019.10.1483 Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ WCN19-0059 headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- Poster shift 04 - Ataxia and cerebellar disorders/autoimmune rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells disorders - Part II/CNS infections - Part II/dementia - Part II/ and gene therapy - Part II/training in neurology - Part II/child epilepsy - Part II/functional disorders and behavioral neurology/ neurology and neuroimaging headache/metabolic and mitochondrial diseases/neuro-critical The correlation between cochlea otic capsule and ear bone care/neurogenetics/neuroophthalmology/neurophysiology/neu- density towards femur and spine density (study on patients with rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells a decrease in bone density) and gene therapy - Part II/training in neurology - Part II/child neurology and neuroimaging Y. Yueniwati, R. Rosa Faculty of Medicine Universitas Brawijaya, Radiology, Malang, Indonesia^ Grading of bell's palsy among Sudanese patients in Khartoum STATE 2016 Background Osteoporosis and osteopenia are systemic disorders that affect all a b c A. Elaalem , M.K.M. Elnimeiri , O.E.A. Hamid bones. Osteoporosis in the ear bone and cochlea otic capsule a ALNeelain University, Neurology, Khartoum, Sudan contribute to the occurrence of conduction and sensorineural deaf. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 357

The osteoporosis prevalence in RSSA is 38%; meanwhile hearing Methods interference in osteoporosis patients is 66.67%. The femur and This cross sectional, descriptive study included all the diagnosed spine DXA are the golden standard to calculate the bone mineral cases of type 2 Diabetes (n =^20; age: 56 ± 9.5) referred from OPD. density. The temporal bone density can be measured with CT scan Nerve conduction study (NCS) were performed in median, ulnar, using the Hounsfield Unit (HU) units. There is a hypothesis that tibial, common peroneal and sural nerve using Nihon Kohden the femur bone mass density can illustrate the temporal bone machine in Neurophysiology lab 2, BPKIHS. density. Result Aim NCS were performed in 20 patients out of whom 17 patients i.e. This study aimed to analyzes the correlation between the density 85% had axonal pattern, 3 patient i.e.15% had mixed (axonal and of the cochlea otic capsule and ear bone towards the femur and spine demyelinating) pattern of neuropathy. Among sensory nerve; density in patients with decrease in bone density. median, ulnar and sural were involved and among motor nerve; median and tibial nerve were mostly involved. Distal amplitude of Methods and Samples median nerve showed significant negative correlation with HbA1c − − This was a cross-sectional observational type analytic retrospec- (rt; r = 0.621, p =^.004; lt; r = 0.690, p =^.001) and nerve tive study analyzing 30 samples of patients with a decrease in bone conduction velocity of tibial nerve showed significant negative density that have HRCT Mastoid examination results during 2017 at correlation with HbA1c respectively. RSSA Malang's osteoporosis polyclinic. Conclusion Results In type 2 Diabetes patients mostly axonal pattern of neuropathy is From 30 samples, most were female with an age range of 51–60 seen. Distal amplitude and nerve conduction velocity were nega- ^years old. The average femur, spine, ear bone and cochlea otic capsule tively correlated with HbA1c. of the osteoporosis group was lower than the osteopenia group. The spine density was significantly correlated with the otic capsule density. doi:10.1016/j.jns.2019.10.1486 Conclusions There was a positive significant correlation between the spine density towards the otic capsule density. The lower the spine density, the lower the otic capsule became. WCN19-0071 doi:10.1016/j.jns.2019.10.1485 Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ WCN19-0070 headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- Poster shift 04 - Ataxia and cerebellar disorders/autoimmune rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells disorders - Part II/CNS infections - Part II/dementia - Part II/ and gene therapy - Part II/training in neurology - Part II/child epilepsy - Part II/functional disorders and behavioral neurology/ neurology and neuroimaging headache/metabolic and mitochondrial diseases/neuro-critical Progressive ataxia and palatal tremor (PAPT). A clinical case care/neurogenetics/neuroophthalmology/neurophysiology/neu- ^ ^ rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells M. Fuentealba and gene therapy - Part II/training in neurology - Part II/child Universidad de Concepcion, Especialidades, Concepcion, Chile^ neurology and neuroimaging

68 ^year old Patient ^with no relevant family or personal morbid Nerve conduction studies in patients with type 2 diabetes mellitus background ^that begins in a progressive way a year ago with and its correlation with HBA1C disturbanceôf the march and balance adding frequent forgetfulness and lack of initiative in their daily life.To the neurological S. Guptaa, N. Limbua, D. Thakura, P. Subedia, R. Maskeyb examination this vigil, oriented but with attention failures, is forgetful aB.P.Koirala Institute of Health Sciences, Basic and Clinical Physiology, fl and fails in the work memory test^, affective attening even with Dharan, Nepal anosognosia of some symptoms, with severe ataxia of the midline gait bB.P.Koirala Institute of Health Sciences, Internal Medicine, Dharan, without lateralization that increases blindly, speech disorder with Nepal – ^ scanty dysarthria and an irregular myoclonic tremor (^2 3hz)în the palate (^palatal tremor)^without audible click. No other neurological Background signs are found.The study has not shown any metabolic disturbance Diabetes mellitus is the most common cause of neuropathy and body CT shows no primary neoplasia.MR of Encephalus with worldwide. Electrophysiological parameters may be abnormal even gadolinium shows hypersignal (T2) in^bilateral olive without taking in newly diagnosed cases of Diabetes mellitus as it is insidious in gadolinium contrast and slight vermian cerebellar atrophy. onset. Syndromatic^Diagnosis Objective ^Palatar Tremor^, Vermian Cerebellar Syndrome^,Cerebellar Cogni- tive affective Syndrome. 1. ^To study pattern of neuropathy in type 2 Diabetes. ^2. To ^ correlate nerve conduction parameters with HbA1c. ARTICLE IN PRESS

358 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Topographic Diagnostic Background ^Olive-Cerebellar System^ . DRPLA is a rare, autosomal dominantly-inherited CAG repeat disorder of the ATN1 gene characterized by myoclonus, epilepsy, Etiological Diagnosis ataxia, and dementia. DRPLA is most commonly seen in populations of Japanese ancestry. An African-American family with “Haw River ^Progressive Ataxia and Palatal Tremor (PAPT). It is reported case of very low frequency worldwide (1) being the Syndrome” was studied in North Carolina, but was later determined ﬁrst reported in our environment and was added with the that Haw River Syndrome and DRPLA are the same disorder. registration of magnetic resonance images and video of palatal tremor. Methods Retrospective analysis of three cases of DRPLA affecting a father, (1) Gao^Movement disorders vol 32 No 10^2017. son, and daughter of African descent; a 50 year-old male from Sierra doi:10.1016/j.jns.2019.10.1487 Leone with 61 CAG repeats in allele 1 of ATN1 gene and his 2 offspring, a son (71 repeats) and daughter (69 repeats).

Results WCN19-0080 Father became clinically manifest at age 38 with executive dysfunction, then progressive ataxia. Son showed signs of developmental delay at age 4 and later developed refractory, generalized Poster shift 04 - Ataxia and cerebellar disorders/autoimmune epilepsy at age 7. He was treated with multiple anti-epileptic disorders - Part II/CNS infections - Part II /dementia - Part II / medications and vagal nerve stimulation as well as deep-brain epilepsy - Part II /functional disorders and behavioral neurology/ stimulation for severe dystonia. Daughter exhibited developmental headache/metabolic and mitochondrial diseases/neuro-critical delays in early childhood and started to have generalized seizures at age 11. She has less refractory epilepsy which has been care/neurogenetics/neuroophthalmology/neurophysiology/neu- controlled with anti-epileptic medications, vagal nerve stimulation, rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and CBD oil. and gene therapy - Part II/training in nuerology - Part II /child neurology and neuroimaging Conclusions DRPLA is not commonly seen in individuals of African descent. In Case series of 3 individuals of African descent with dentatorubral- these three cases, the clinical presentation differed among the Pallidoluysian atrophy offspring who predominantly experienced seizures, dystonia, and intellectual disability, whereas, the father presented predominantly C. Kutza, C. Bundukamarab with ataxia and cognitive dysfunction. aColorado Springs Neurological Associates, Neurology, Colorado Springs, USA bMentally Strong, Psychiatry, Colorado Springs, USA doi:10.1016/j.jns.2019.10.1488 Objective To analyze the genetic history and clinical presentation of three individuals of African descent with Dentatorubral-Pallidoluysian Atrophy (DRPLA). ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 359

WCN19-0081 whereas, HCPs goals were focused towards medication and disease management. Understanding these differences may lead to improved communication between TD patients and their healthcare Poster shift 04 - Ataxia and cerebellar disorders/autoimmune providers which may enhance treatment satisfaction. disorders - Part II /CNS Infections - Part II /dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- doi:10.1016/j.jns.2019.10.1489 rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in nuerology - Part II/child neurology andneuroimaging WCN19-0116 Patient-Centered approach to elicit and compare the treatment goals of patients with tardive dyskinesia, their care partners, and Poster shift 04 - Ataxia and cerebellar disorders/autoimmune healthcare providers disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ C. Kutz headache/metabolic and mitochondrial diseases/neuro-critical Colorado Springs Neurological Associates, Neurology, Colorado Springs, care/neurogenetics/neuroophthalmology/neurophysiology/neu- USA rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in nuerology - Part II/child Objective neurology and neuroimaging To elicit and compare the treatment goals of patients with tardive dyskinesia (TD), their care partners, and healthcare providers (HCP). Moyamoya disease case report. Background Tardive dyskinesia (TD) is a repetitive, involuntary movement S. Murzabaeva disorder caused by prolonged exposure to a medication that inhibits National Center of Maternity and Childhood Care, Perinatal Pathology, the dopamine receptor. Understanding what matters to patients is Bishkek, Kyrgyzstan critical in medical decision making with TD. Moyamoya disease (MMD) is a chronic, occlusive cerebrovas- Methodology cular disease characterized by progressive stenosis at the terminal NGT was utilized to elicit the treatment goals of 3 groups: portion of the internal carotid artery and an abnormal vascular patients with TD, TD care partners, and healthcare providers. Each network at the base of the brain. MMD was originally considered group was asked to identify their goals for TD treatment. Participants exclusive to East Asia, with particular prevalence in Japan, but it is were then asked to silently identify and rank their 10 most now increasingly diagnosed throughout the world, and represents important goals (1-most important treatment goal to 10-least an important cause of childhood stroke. We present a case report important treatment goal). of a 6-year-old Asian girl with moyamoya disease who presented with a 2-year history of recurrent strokes and a change in Results personality. During examination her mother said that the disease 5 neurologists, 5 neurology PAs, and 3 psychiatry NPs, 7 TD was started with an ischemic episodes characterized by left patients, and 5 TD care partners were interviewed using NGT. Each extremities weakness and loss of vision. No family history of early participant ranked their top 10 treatment goals. The data was sorted stroke, ischemic heart disease, or autoimmune disease was to determine the overall top 10 treatment goals for each group. reported. Other systemic examination revealed no abnormality. Healthcare providers top treatment goal for their TD patients was Cerebral angiography conﬁrmed the diagnosis of moyamoya “improve quality of life as able”, TD patients top goal was “for this to disease (Fig. 1). The following medicines were prescribed: go away”, and TD care partners was “reduce symptoms without acetylsalicylic acid 250 mg per day and referred to follow-up with adding new side effects”. pediatric neurosurgeon.

Conclusions Differences between treatment goals generated by TD patients and HCPs were identiﬁed. Goals generated by patients typically doi:10.1016/j.jns.2019.10.1490 involved improvement of day-to-day symptoms and functioning, ARTICLE IN PRESS

360 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-0117 Statement of the problem A 3 yrs old girl presented with unilateral facial erythema and sweating after gustatory stimuli. She had history of mycoplasma and Poster shift 04 - Ataxia and cerebellar disorders/autoimmune Brucella infection. She developed left facial palsy that has been disorders - Part II /CNS infections - Part II/dementia - Part II/ resolved. She received antibiotics and corticosteroids. CT scan and epilepsy - Part II/functional disorders and behavioral neurology/ MRI brain were normal. Interestingly, the child fulﬁll the clinical headache/metabolic and mitochondrial diseases/neuro-critical diagnosis of Frey syndrome. care/neurogenetics /neuroophthalmology/neurophysiology/neu- Frey's syndrome is characterized by unilateral sweating and ﬂ rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells ushing of the skin in the area of the parotid gland, occurring during meal, or on salivary stimulation. It is a common complication and gene therapy - Part II/training in nuerology - Part II/child following surgery/injury in the parotid gland region/temporoman- neurology andneuroimaging dibular joint surgery; however, very few cases had been reported following herpetic infection. The condition may be misdiagnosed as Unilateral facial erythema following food chewing contact dermatitis or food allergy, but these can be excluded by the normal skin texture and typical unilateral distribution on the pre- S. Almuntaser, M. Saleh, G. Aithala auricular area and cheek. The diagnosis is made on clinical grounds, Dubai health authority, Pediatric Neurology, Dubai, United Arab and further testing is not needed. It is important for the clinician to Emirates recognize its unusual symptoms as early as possible to prevent its ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 361 potential negative social and psychological effect on the patient. B. Hirosea, K. Ikedaa, D. Yamamotoa, S. Shimohamaa, Y. Asadab,T. Mycoplasma infection, as in this case, is a rare cause of Frey’s Imaib syndrome. Here we highlight the importance of considering Frey aSapporo Medical University School of Medicine, Neurology, Sapporo, syndrome in these cases as Patients can be reassured of the benign Japan nature of the condition and that symptoms usually resolve bSapporo Medical University Graduate School of Health Sciences, spontaneously in children. Disability Studies of Peripheral Nerve and Muscle, Sapporo, Japan doi:10.1016/j.jns.2019.10.1491 Objective The aim of this study is to elucidate the impairment of excitation- contraction (E-C) coupling in ICU-acquired weakness (ICUAW) using a unique electrophysiologic method in patients received the WCN19-0118 intensive care in ICU.

Method Poster shift 04 - Ataxia and cerebellar disorders/autoimmune We examined 9 patients with ICUAW and 10 normal controls. We disorders - Part II/CNS infections - Part II/dementia - Part II/ performed successive recordings of electrophysiological tests after epilepsy - Part II/functional disorders and behavioral neurology/ ICU admission. First, CMAPs of abductor hallucis muscle (AH) and headache/metabolic and mitochondrial diseases/neuro-critical sural SNAPs were recorded using a conventional method. In addition, care/neurogenetics/neuroophthalmology/neurophysiology/neu- the tibial nerve was stimulated at the popliteal fossa using a rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells supramaximal rectangular pulse to record CMAPs from soleus muscle and gene therapy - Part II/training in nuerology - Part II/child (SOL). Simultaneously, movement-related potential (MRP) was recorded using an accelerometer (SV1101) taped at the base of neurology andneuroimaging hallux and calculated E-C coupling time (ECCT) based on the latency differences between soleus CMAP and MRP. Novel gene mutation of molybdenum cofactor deficiency Results S. Almuntaser, M. Saleh, P. Kashyape MRPs as well as CMAP amplitudes of AH and SOL were Dubai Health Authority, Pediatric Neurology, Dubai, United Arab significantly smaller in patients than in controls. Also, ECCTs and Emirates CMAP durations of AH were significantly longer in patients than in controls. Nine patients were subdivided into 6 patients with critical Molybdenum cofactor deficiency is a rare metabolic condition illness myopathy (CIM) and 3 patients with critical illness poly- characterized by brain dysfunction (encephalopathy) that worsens neuropathy (CIP) based on the sural SNAP abnormality. All CIM over time. Molybdenum cofactor deficiency is caused by mutations in patients consistently showed significantly prolonged ECCTs, and 5 the ΜΟSC1, ΜΟSC2, or GPHN gene. There are three forms of the CIM patients showed significantly decreased MRPs. These abnormal- disorder, named types A, B, and C. The forms have the same signs ities occurred within several days after ICU admission. and symptoms but are distinguished by their genetic cause: ΜΟSC1 gene mutations cause type A, ΜΟSC2 gene mutations cause type B, Conclusion and GPHN gene mutations cause type C. The E-C coupling may be impaired in the very early stages of CIM. Herein we are reporting a 2 months old child who presented to Noninvasive measurement of MRP and ECCT is easy to perform, and our hospitals with seizure disorders, post cardiac arrest. After clinical may be an useful indicator of muscle involvement in the early stages evaluation, and thorough investigations, uric acid was undetectable. of ICUAW. MRI brain showed periventricular leukomalacia- HIE picture, with bilateral frontal lobe white matter loss with extensive cystic doi:10.1016/j.jns.2019.10.1493 formation and thinning of corpus callosum. Molecular genetic testing showed pathogenic deletion c.604_624del p.( Gly202_glu208del) in exon 4 of MOCS1 gene in homozygous state. to best of our knowledge this variant was not yet reported in any literature yet. WCN19-0122 doi:10.1016/j.jns.2019.10.1492 Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ WCN19-0120 headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- Poster shift 04 - Ataxia and cerebellar disorders/autoimmune rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells disorders - Part II/CNS infections - Part II/dementia - Part II/ and gene therapy - Part II/training in neurology - Part II/child epilepsy - Part II/functional disorders and behavioral neurology/ neurology and neuroimaging headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- Visualization of electrophysiological activity in the cervical spinal cord using magnetospinography rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in nuerology - Part II/child S. Sumiyaab, S. Kawabatabc, S. Ushiob, T. Sasakid, J. Hashimotob,Y. neurology and neuroimaging Hoshinoc, K. Sekiharac, T. Watanabede, Y. Adachif, A. Okawad aSaku Medical Center, Orthopedics and Spine Surgery, Saku-City, Japan The impairment of excitation-contraction coupling in bTokyo Medical and Dental University- Department of Orthopedics icu-acquired weakness Surgery, Orthopedics and Spine Surgery, Tokyo, Japan ARTICLE IN PRESS

362 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx cTokyo Medical and Dental University- Advanced Technology in Hybrid Assistive Limb (HAL) is a wearable-type exoskeleton Medicine, Orthopedics, Tokyo, Japan robot, which can assist wearer’s movement in volition-triggered way dTokyo Medical and Dental University- Department of Orthopedics by virtue of human-machine interaction. We planned a pilot study to Surgery, Orthopedics, Tokyo, Japan explore the validity of neurorehabilitation utilizing HAL for acute eRICOH Company- Ltd., Technology, Tokyo, Japan stroke. Among patients admitted to our hospital, acute stroke fKanazawa Institute of Technology- Applied Electronics Laboratory, patients in the second week with functional ambulation categories Technology, Kanazawa-City, Japan (FAC) of 0 to 2 were included. The patients included in a year from November 2016 were alternatively assigned to two HAL groups, Introduction where gait training wearing bilateral-leg version HAL for 40 min was Magnetic fields generated by neuronal currents are less affected performed 5 days a week (5d-HAL group) or 3 days a week (3d-HAL by surrounding tissues and so may be used for high-resolution group) for four week. Patients included in the next one year were surface recordings of neural activity. We have developed a mag- assigned to conventional group (CPT group), where conventional gait netospinography system with highly sensitive superconducting training was performed for four weeks. Safety outcome was the quantum interference device sensors for noninvasive electrophysio- occurrence of severe adverse event. Efficacy outcomes, including logical analysis of spinal cord and spinal nerve function. In this study, FAC, gait speed, and modified Rankin Scale (mRS) were evaluated we imaged neural activity in the cervical spine by surface before and after the protocol. Twenty-two patients were assigned to magnetospinography. HAL group; eleven to 5d-HAL group and eleven to 3d-HAL group. Ten patients assigned to CPT group. No serious adverse event was seen in Methods two HAL groups. A stroke recurrence was occurred in CPT group. Ten healthy volunteers were placed in the supine position on a After excluding the patient in CPT group and one dropped-out newly developed 120-channel magnetospinograph. Neuromagnetic patient in 3d-HAL group, efficacy outcomes were evaluated for thirty fields were measured at the dorsal neck surface in response to surface patients. Significant improvement in the efficacy outcomes was stimulation of the median nerve at the elbow (3 Hz; monophasic pulses; observed after the protocol in HAL group. The pre-post changes ware 0.3 ms width; constant current of 3.6–11 mA) and 2,000 responses were not different between two HAL groups, while were significantly averaged. Current sources producing the magnetic fields were esti- higher than in CPT group. mated using spatial filtering methods, and the estimated current field was superimposed on X-ray images of the cervical spine. doi:10.1016/j.jns.2019.10.1495

Results Neuromagnetic ﬁelds were successfully recorded over the skin surface of all subjects. Estimated electric currents entered the lateral WCN19-0138 cervical spine from C4/5 to Th1/2. In the spinal canal, these signals changed direction and propagated caudal to cranial at 74.9 m/s. The Poster shift 04 - Ataxia and cerebellar disorders/autoimmune largest estimated currents were observed at the C6/7 and C7/Th1 intervertebral foramen. disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ Conclusion headache/metabolic and mitochondrial diseases/neuro-critical Our magnetospinography system could noninvasively image care/neurogenetics/neuroophthalmology/neurophysiology/neu- – electric activity entering the C5 Th1 nerve roots and ascending the rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells spinal cord. The originating nerve roots were consistent with the and gene therapy - Part II/training in neurology - Part II/child conduction pathway of the median nerve, and the conduction neurology and neuroimaging velocities in the spinal cord were equivalent to previous estimates. doi:10.1016/j.jns.2019.10.1494 Sinking skin ﬂap syndrome presenting as autonomic dysautonomia

M. Thakre WCN19-0134 Al Zahra Hospital, Neurology, Dubai, United Arab Emirates

Objective Poster shift 04 - Ataxia and cerebellar disorders/autoimmune To demonstrate a rare complication of decompressive Craniotomy disorders - Part II/CNS infections - Part II/dementia - Part II/ and its presentation as dysautonomia epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical Background ﬂ care/neurogenetics/neuroophthalmology/neurophysiology/neu- Sinking skin ap syndrome is a rare complication of a large decompressive craniotomy surgery. It occurs due to atmospheric rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells pressure exceeding intracranial pressure causing paradoxical herni- and gene therapy - Part II/training in neurology - Part II/child ation. It is often precipitated by CSF drainage procedure. neurology and neuroimaging Case report A pilot study to explore the safety and efﬁcacy of hybrid assistive 44 years old gentleman presented with acute left hemiplegia with limb for acute stroke NIHSS score 12. He was thrombolysed intravenously but unfortunately developed a large basal ganglia hemorrhage the following day. K. Yamaguchi He underwent emergency decompressive craniotomy as he started Ichinomiya Nishi Hospital, Neurology, Ichinomiya, Japan herniating. Post surgery developed high grade fever for which he ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 363 required a guarded lumbar puncture done on day 14. His fever and Results consciousness improved in the next 2 weeks and he was able to Fifty-eight patients from Slovakia, Macedonia and Poland were communicate and was mobilized to wheel chair. 4th week he started included with mean age of 62.04 ± 11.48 years, with 23 (39.66%) becoming drowsy, unresponsive with episodes of dysautonomia in women. NurAiD II was started at 100.45 ± 225.95 days since the form of high temperature, severe sweating, tachypnoea and severe time of stroke onset. Median NIHSS for the patients was 9 (1-33) at tachycardia. CT brain done showed sunken skin flap with paradoxical baseline (B), 6.0 (0–19) at M (month) 1, 5 (0–42) at M2 and 3 herniation. Conservative methods failed and replacement of bone (0–42) at M3 (Fig. 1). mRS was improving over time (Fig. 2). No side flap ultimately helped and patient recovered. effects were reported. 2 patients died due to causes deemed not related to NurAiD II. Results Replacement of bone flap is the definitive treatment of sinking Conclusions skin flap syndrome which if unrecognized can be fatal. NurAiD II was safe in ischemic stroke in the Eastern European population who improved on functional, neurological and cognitive Conclusions measures. Sinking skin flap syndrome is a rare complication of decompressive craniotomy and can present with dysautonomic symptoms. Replacement of bone flap remains definitive treatment doi:10.1016/j.jns.2019.10.1497 doi:10.1016/j.jns.2019.10.1496

WCN19-0174 WCN19-0151

Poster shift 04 - Ataxia and cerebellar disorders/autoimmune Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ disorders - Part II/CNS Infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in neurology - Part II/child and gene therapy - Part II/training in neurology - Part II/child neurology and neuroimaging neurology and neuroimaging Analysis of PDE10A mutations in suspected Huntington disease MLC901 in post stroke recovery in the eastern European patients with normal CAG repeats in Singapore population: A preliminary result of a multicentre study S.K. Sonu a b A. Arsovska , J. Opara Singapore General Hospital, Department of Neurology, Singapore, a University Clinic for Neurology, Urgent Neurology, Skopje, Macedonia Singapore bRepty Upper-Silesian Rehabilitation Centre, Rehabilitation, Tarnowskie Gory, Poland Introduction Huntington disease (HD) a rare neurodegenerative disorder, Background and aims diagnosis is based on clinical presentation, family history and genetic Treatments for post-stroke recovery are limited. MLC901 (NurAiD testing of the expansion with N 36 CAG repeats in HTT gene. II) is a combination of natural products has been used to improve Unfortunately, genetic testing for CAG expansion may not account recovery after ischemic stroke. This study aims to assess the safety for all HD patients. Emerging evidences suggest that PDE10A and potential efficacy of NurAiD II in the early stage after stroke, not mutation is associated with HD. As such, we aim to determine later than six months after stroke onset in the Eastern European whether our local suspected HD patients with normal number of population. CAG repeats (non-pathogenic) in HTT gene would have PDE10A mutation to confirm their diagnosis. Methods The NeuroAiD Safe Treatment (NeST) Registry (clinicaltrials.gov Method NCT02536079) is a product registry provides information on the use Since 1995 to 2017, a total of 200 patients were suspected with and safety of NeuroAiD in clinical practice. We analysed anonymized HD and underwent genetic testing for CAG repeats in HTT gene. Out information of ischemic injury from Slovakia, Macedonia and Poland of 200 patients, 87 had CAG expansion in HTT gene and had been was included in the NeST Registry (www.neuroaid.com/en/nest/ confirmed as HD positive. We selected 20 recently (2013–2017 main/index). Patients consented are prospectively entered using years) suspected HD patient for this study. The age of the patients online forms for baseline and subsequent visits. Data collected ranged between 24 and 80 years old. 55% of them were Chinese and includes demographics, diagnosis, medical history, modified Rankin 60% were female. Next, we performed DNA sequencing on the Score (mRS), Glasgow Coma Scale (GCS), National Institute of Health reported hot spot areas of PDE10A gene. These include exon 3, 4, 11 Stroke Scale (NIHSS), compliance and side effects. and 12 of PDE10A. ARTICLE IN PRESS

364 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

Results and conclusion expression of IFN-γ, IL-17 and elevated the expression of IL-10 both Our study revealed that no mutation in PDE10A was found in the in T cells and in the supernatant of cultured T cells. The levels of 20 subject samples, indicating that there was no correlation between ROCK-II, CCL20, p-NF-kB, TLR-4 were decreased and the levels of IL- PDE10A mutation and HD for this current batch of samples. The 10, Foxp3 were enhanced in Fasudil-modified T cells. result obtained was inconsistent with other reported studies and it could be due to low sample size in our study and different genotypes Conclusion in different geographical regions. Fasudil-modified T cells have therapeutic potential in EAE possibly through inducing the polarization of regulatory T cells and doi:10.1016/j.jns.2019.10.1498 inhibiting inflammatory responses. (NNSF of China 81501032, Shanxi Scholarship Council of China 2014-7, the 2011 Cultivation Project of SUTCM 2011PY-1. Ma and Xiao are corresponding authors). doi:10.1016/j.jns.2019.10.1499 WCN19-0182

Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - PART II/CNS infections - PART II/dementia - PART II/ WCN19-0187 epilepsy - PART II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical Poster shift 04 - Ataxia and cerebellar disorders/autoimmune care/neurogenetics/neuroophthalmology/neurophysiology/neu- disorders - Part II/CNS infections - Part II/dementia - Part II/ rorehabilitation/pain - PART II/sleep disorders - PART II/stem epilepsy - Part II/functional disorders and behavioral neurology/ cells and gene therapy - PART II/training in neurology - PART II/ headache/metabolic and mitochondrial diseases/neuro-critical child neurology and neuroimaging care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells Fasudil modiﬁed T cells: A promising therapy for experimental and gene therapy - Part II/training in neurology - Part II/child autoimmune encephalomyelitis neurology and neuroimaging

C.Y. Liua, S.D. Guoa, J.C. Liub, H.Z. Zhangb, J.Z. Yuab, J.J. Huangbc,L. The effect of integrated volitional control electrical stimulation Fenga, B.G. Xiaod, C.G. Maab (IVES) toward chronic stroke patients aShanxi Datong University, Institute of Brain Science, Shanxi Key fl Laboratory of In ammatory Neurodegenerative Diseases, Datong, China Y. Asai b Shanxi University of Chinese Medicine, The Key Research Laboratory of Yokohamashintoshi Neurosurgical Hospital, Department of Rehabilita- fi Bene ting Qi for Acting Blood Circulation Method to Treat Multiple tion, Yokohama, Japan Sclerosis of State Administration of Traditional Chinese Medicine, fi Scienti c and Technological Innovation Team of Integrated Chinese Purpose and Western M, Jingzhong, China c Facilities, introducing integrated volitional control electrical General Hospital of Datong Coalmine Group, Dept. of Neurosurgery, stimulation (IVES) toward chronic stroke patients are increasing in Datong, China d Japan. In our hospital, the therapy using IVES was started in 2017. Fudan University, Institute of Neurology, Huashan Hospital, Institutes of Chronic stroke patients are trained using IVES for 4 weeks in Brain Science, State Key Laboratory of Medical Neurobiology, Shanghai, China admission intensively. We report the result reviewed for the cases.

Background Method + EAE is a CD4 T cell-mediated autoimmune demyelinating Ten chronic stroke patients (above 6 month after onset) were animal model. ROCK inhibitor Fasudil shows therapeutic effects in objected from April 1, 2017 to March 31, 2019. Evaluation items are EAE, but the boundedness such as smaller safety window and no oral age, sex, disease (cerebral hemorrhage or cerebral infarction), Fugl- tablet limit its clinical application. Myer assessment (FMA) upper extremity, and stroke impairment assessment set (SIAS). Statistics was used Wilcoxon signed rank test. Objective fi To explore the therapeutic potential of Fasudil-modi ed T cells in Result EAE, and provide a theoretical basis for the future clinical application. The average age was 58.6 ± 11.2 (SD), male was seven and female was two. The diseases were 2 cerebral infarction and 8 Material and methods cerebral hemorrhage. FMA on admission (A) was 25.4 ± 17.2, SIAS Splenic encephalitogenic T cells from mice immunized with on A was 41.1 ± 9.4. FMA on discharge (D) was 29.7 ± 17.7. SIAS on MOG35-55 on day 9 postimmunization(p.i) were obtained by D was 43.6 ± 8.9. Significant improvement was recognized both magnetic bead and treated with/without Fasudil in vitro for 72 FMA, SIAS on D better than on A. hours. Cells were resuspended and transferred by intraperitoneal 7 injection with 5×10 on day 3 and 11 p.i to EAE mice, which were Conclusion divided into PBS-T cells group and Fasudil-T cells group. Improvement in almost cases were seen after IVES therapy. Because of various conditions of hemiplegia, the effect were Results different. IVES therapy will be comfortable for middle stage of fi Fasudil-modi ed T cells delayed the onset and reduced clinical hemiplegia. However, IVES therapy should be adapted in more fl score of EAE. Demyelination and in ammation in spinal cords of patients of different stage. Fasudil-T cells group were significantly decreased compared to those of PBS-T cells group. Fasudil-modified T cells effectively reduced the doi:10.1016/j.jns.2019.10.1500 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 365

WCN19-0201 for patients with chronic TTH should be complementary treatment (pharmacotherapy+HRV-BBT+IE).

Poster shift 04 - Ataxia and cerebellar disorders/autoimmune doi:10.1016/j.jns.2019.10.1501 disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- WCN19-0215 rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in neurology - Part II/child Poster shift 04 - Ataxia and cerebellar disorders/autoimmune neurology and neuroimaging disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II/functional disorders and behavioral neurology/ The efﬁcacy of heart rate variability biofeedback-based training headache/metabolic and mitochondrial diseases/neuro-critical in preventing tension type headache in adolescents care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells a b K. Stepanchenko , V. Marchenko and gene therapy - Part II/training in neurology - Part II/child a Kharkiv Medical Academy of Postgraduate Education, Department of neurology and neuroimaging Neurology and Child Neurology, Kharkov, Ukraine bKharkiv Medical Academy of Postgraduate Education, Department of Electronyuromiographic indicators of post-operation of the Tibia Neurology, Kharkov, Ukraine

B. Muminova, G. Boymurodovb, R. Matmurodova Objectives aTashkent medical academy, Neurology, Tashkent, Uzbekistan To assess the efficacy of non-pharmacological therapy (heart rate bTashkent, Traumatology, Tashkent, Uzbekistan variability biofeedback-based training (HRV-BBT) and exercise) in the model of differentiated therapeutic intervention in adolescents with tension-type headache (TTH). Electroneuromyographic (ENMG) studies is a unique method for evaluating the surgical method of treating a tibial fracture. Methods 137 adolescents (ages 13–18) were examined. We formed four Material and methods groups of adolescents with episodic (ETTH) and chronic TTH 45 patients were examined before surgery and after surgery. In 27 (CTTH) who received only drug therapy and only non-drug therapy patients, a surgical method of compression - distraction osteosynthesis and 5th group – adolescents with CTTH who received combination was performed, in 18 patients the usual method. All patients underwent of drug and non-drug therapy. We used VAS; self-esteem scale ENMG studies. Compression - distraction osteosynthesis is the most Spielberger-Hanin; Phillips method; scale of depression by V.A. unique surgical method for the treatment of proximal tibial fractures. Zhmurova; PedMIDAS; HRV, EEG. Adolescents received conventional pharmacotherapy for 1 month. Non-drug therapy was in the Results form of HRV-BBT (10–12 sessions) and a complex of isometric The results show that the M-amplitude of the tibial nerve was in exercises (IE). healthy legs 2.5 ± 1.17 mV, in the affected legs before treatment 0.29 ± 0.47 mV. After surgical treatment (Compression - distraction – Results osteosynthesis, after 3 6 months of surgery) during the rehabilitation Groups of non-drug therapy in comparison with only pharmaco- period, the M-response amplitude was 1.48 ± 0.32 mV. The results of therapy groups had statistically more pronounced decrease in the the study of the M-response amplitude of the peroneal nerve in intensity of headache, a decrease in reactive anxiety and depression healthy legs 2.48 ± 0.22 mV, in the affected legs to treatment 0.5 ± level, the improvement of the quality of life, an increase in total 0.11 mV and after surgery 1.46 ± 0.53 mV. The speed of the impulse spectral power (TP), an increase in %HF and a decrease in %LF and % (SPI) of the tibial nerve in healthy legs was 49.1 ± 1.14 m/s, in the VLF of HRV, a reduction of dysrhythmia and disorganization EEG, a affected legs before treatment 24.5 ± 1.3 m/s and after treatment 31.6 fi significant increase in spectral power of alpha rhythm, a decrease in ± 1.52 m/with. When, as in the bular nerve, these indicators were slow-wave-EEG activity, positive shifts in the level of chaotic 44.8 ± 1.27 m/s - 24.5 ± 1.32 m/s - 34.8 ± 1.79 m/s. neurodynamic (pb0,05). Conclusion Conclusion ENMG is an invasive method, with a fracture of the proximal part Pathogenetic non-drug therapy (HRV-BBT and IE) has high of the tibial, before and after treatment, the rehabilitation period efficiency in the treatment of TTH with a stable catamnestic effect. provides information on the effectiveness of surgical treatment. The efficacy of non-drug therapy in adolescents with episodic form of doi:10.1016/j.jns.2019.10.1502 TTH is the same as pharmacotherapy. The most rational appointment ARTICLE IN PRESS

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WCN19-0229 Their diagnosis was established 1 to 10 years after the clinical onset. LG muscle weakness with prominent involvement of lower limbs and axial muscles was present in all of the affected. Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ Conclusion epilepsy - Part II/functional disorders and behavioral neurology/ The rarity of the disorder, variable clinical presentation, and headache/metabolic and mitochondrial diseases/neuro-critical overlap of symptoms with other neuromuscular disorders often care/neurogenetics/neuroophthalmology/neurophysiology/neu- result in delays in diagnosis and treatment for many patients. ﬁ rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells Targeted screening of unclassi ed patients with LGMD by DBS can be a valuable tool for identiﬁcation of Pompe patients. and gene therapy - Part II/training in neurology - Part II/child neurology and neuroimaging doi:10.1016/j.jns.2019.10.1503

Targeted screening for detection of Pompe disease in patients with unclassiﬁed Limb-Girdle Muscular Dystrophy (lGMD) using Dried-Blood Spot (DBS) test: Bulgarian experience WCN19-0233

a a b c T. Chamova , A. Taneva , I. Sinigerska , M. Gospodinova ,V. Poster shift 04 - Ataxia and cerebellar disorders/autoimmune Bojinovad, K. Genove, I. Staykovf, H. Dimitrovag, D. Bogdanovah,A. disorders - Part II/CNS infections - Part II/dementia - Part II/ Kaprelyani, T. Todorovj, A. Todorovak, I. Tournevl aMedical university- Sofia, Department of Neurology, University hospital epilepsy - Part II/functional disorders and behavioral neurology/ Alexandrovska, Sofia, Bulgaria headache/metabolic and mitochondrial diseases/neuro-critical bMedical University-Sofia, National Genetics Laboratory, Sofia, Bulgaria care/neurogenetics/neuroophthalmology/neurophysiology/neu- cMedical Institute of Ministry of Interior Affairs, Department of rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells Cardiology, Sofia, Bulgaria and gene therapy - Part II/training in neurology - Part II/child d fi Medical University-So a, Clinic of Child Neurology, University Hospital neurology and neuroimaging of Neurology and Psychiatry Sv. Naum, Sofia, Bulgaria eMilitary Medical Academy, Clinic of Neurology, Sofia, Bulgaria Multiphase CT angiography in acute ischemic stroke - Rashid fTokuda Hospital, Clinic of Neurology, Sofia, Bulgaria hospital, Dubai experience gMilitary Medical Academy, Department of Neurology, Sofia, Bulgaria hMedical University-Sofia, University hospital of neurology and psychi- D.S. Iqbala, A. Ayoubb atry Sv. Naum, Bulgaria a i fi Rashid Hospital, Radiology Department, Dubai, United Arab Emirates Medical University- Varna, Department of Neurology, So a, Bulgaria b jGenetic Medico-Diagnostic Laboratory Genica, Genetics, Sofia, Bulgaria Rashid Hospital, Radiology, Dubai, United Arab Emirates kMedical University- Sofia, Department of Medical Chemistry and ’ Biochemistry, Sofia, Bulgaria Dubai Health Authority run Rashid Hospital s Stroke unit is lMedical University-Sofia, Department of Neurology, University Hospital advanced stroke unit in region awarded an international accredita- Alexandrovska, Sofia, Bulgaria tion for the establishment and functioning of the unit following European protocols in the treatment and management of strokes. We Introduction have incorporated this mCTA technique in our institution at the most Pompe disease (PD) is a rare autosomal-recessive lysosomal advanced Radiology department in Rashid hospital, Dubai since Jan storage disorder due to deficiency of acid alpha-glucosidase (GAA) 2018 with CT Brain at patients presentation to our emergency with and clinically characterized by progressive damage of respiratory, AIS (Acute ischemic stroke), this fast modern technique has reduced cardiac, skeletal, and smooth muscles. radiation exposure, ensured that diagnostic test preserves patient safety and has reduced door to needle time frame significantly from Aim 95 to 45 min. In April 2012 a screening program, called “Prevalence study of Multiphase CT Angiography (mCTA) provides an overview of PD” was initiated in Bulgaria. Its main aim was to determine the cerebrovascular hemodynamics, acute stroke pathophysiology, and prevalence of PD among patients with progressive limb-girdle (LG) collateral circulation, accounting for these variables allows physi- muscle weakness with or without respiratory insufficiency and with cians to make more individualized therapeutic decision. or without elevated creatine kinase levels. Imaging of collateral circulation and determination of collateral grade may predict greater reperfusion, lower hemorrhage risk, Material and methods and better functional outcome. mCTA asseses cerebral circulation, fl The study was based on two main stand points- retrospective from arterial in ow routes to the micro-circulation and downstream study of patients with undiagnosed myopathies from the registries of venous channels involved in sustaining collateral perfusion and Bulgarian expert center for hereditary neurologic and metabolic protect the brain against ischemia and sustain the penumbra disorders and prospective study of a cohort of patients who are The currently available knowledge has increased the pathophysi- visiting thirty centers, spread throughout Bulgaria, working in close ologic understanding of intrinsic compensatory vascular mechanisms, collaboration. The In total 488 patients underwent evaluation of the supports the use of mCTA technique to rapidly evaluate AIS (Acute activity of GAA on DBS. ischemic stroke), and provides evidence for therapeutic decisions. Role of collaterals is important critical selection criteria for Results intervention, with triage based on collateral score rather than time For the last 7 years from the 488 tested individuals, 12 had alone. decreased activity of GAA on DBS and 8 were subsequently doi:10.1016/j.jns.2019.10.1504 genetically verified. They had non-classical form of the disorder. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 367

WCN19-0246 WCN19-0257

The diabetes drug metformin reverses cognitive impairment and Distribution preponderance of cerebral microbleeds according to membrane functions in diabetic aging female rat brain: A link severity of leukoaraiosis in Alzheimer's disease with diabetes and Alzheimer’s disease M. Park, D. Shin P. Kumar, N. Baquer Yeungnam University Medical Center, Neurology, Daegu, Republic of Jawaharlal Nehru University, School of Life Sciences, Delhi, India Korea

Objectives Background The objective of this study was to investigate effects of metformin Cerebral microbleeds (CMBs) and leukoaraiosis (LA) are common on body weight, blood glucose levels, glucose transporter (GLUT1), findings in Alzheimer's disease (AD). But clinical or topographic interleukin 1 β (IL-1β) and protein kinase B expression, antioxidant relation of CMBs associated with LA in AD has been lacking. This enzymes, expression of synaptic molecules, biomarkers of oxidative study was performed to determine frequency and topographic stress, lipid peroxidation, membrane fluidity and acetylcholinester- preponderance of CMBs according to severity of LA in AD and ase (AChE) in diabetic aging brain of female rats. compare the cognition and behavior of the AD patients according to severity of LA in AD, also. Methods Male Wistar rats, young (3 months) adult (12 months) and aged Methods (24 months) were diabetic by using alloxan monohydrate. Metfor- min was administered i.p. at a dose of 200mg/kg/day for 30 days to • 101 AD patients were classified in three groups as patients with both control and diabetic aging rats. A detailed study was carried on minimal LA (n=55), moderate LA (n=30) and with severe LA expression of inflammatory cytokines, insulin receptor pathology (n=16). and synapse, GLUT 1, biomarkers of oxidative stress and AChE • CMBs were counted using mainly 1.5 Tesla SWI (n=90) and activity. gradient-echo (n=10) imaging • The Microbleed Anatomical Rating Scale (MARS) was used to Results localize each CMB location. Present study shows that there was a similar pattern of increased • Leukoaraiosis was rated on T2-weighted MRI using the CREDOS expression of interleukin, protein kinase B, lipid peroxidation, blood white matter hyperintensity visual rating scale. glucose, insulin receptor expression and RNS levels with AChE • Numbers and location of CMBs in moderate to severe LA group were activity, and a decrease in membrane fluidity, antioxidant enzymes statistically compared with those of CMBs in minimal LA group. activity and (GLUT1) expression in brain of both aging and diabetes. • Moreover, we compared neuropsychology between the two groups On the other hand, metformin treated groups exhibited significant by Seoul Neuropsychological Battery-1 reduction in helped to reverse the age related changes studied, to normal levels. Metformin treatments improved attention and Results memory functions with enhanced the levels of synaptic molecules • Total CMBs in moderate to severe LA group (CMBs = 77) was synaptophysin and synapsin I. Our data showed that exogenous significantly more than CMBs (CMBs=32) in minimal LA group administration of metformin brought these changes to near nor- (P=0.024*, t-test) malcy in diabetic aging female rats. • There is not distribution predominance of CMBs in AD according to leukoaraiosis (P=0.775, Chi-square test) Conclusions • There is no statistically significant cognitive differences between The results illuminate mechanisms of neuroprotection by met- two groups. formin, and applying new strategies for control of neurodegenerative diseases including metabolic syndrome and Alzheimer’s disease. Conclusion doi:10.1016/j.jns.2019.10.1505 • As LA progresses, total number of CMBs increases without affecting cognition and behavior in AD patients and these findings suggest ARTICLE IN PRESS

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there is no distribution predominance of CMBs in AD according to G. Halilia, M. Papajanib, S. Grabovab, A. Rrojib, J. Krujaa leukoaraiosis aFaculty of Medicine, University of Medicine, Tirana, Neurology, Tirana, doi:10.1016/j.jns.2019.10.1506 Albania bUHC Mother Teresa, Neurology, Tirana, Albania

Abstract Fahr disease or Fahr syndrome is a rare neurological disorder WCN19-0269 characterized by abnormal calcified deposits in basal ganglia and cerebral cortex. As the disease progresses, it was found that lateral Poster shift 04 - Ataxia and cerebellar disorders/autoimmune thalamus, dentate nucleus of cerebellum and the frontal cortex get disorders - Part II/CNS infections - Part II/dementia - Part II/ affected as well. Aim of the study was to analyze 5 cases of Fahr epilepsy - Part II/functional disorders and behavioral neurology/ syndrome highlighting any possible correlation between the progressive nature of the disease, clinical spectrum and neuroimaging data. headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- Methods rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells ^Five patients aged 19, 31, 43 and 53 and 56 were studied in Albania. and gene therapy - Part II/training in nuerology - Part II/child They were hospitalized in the Neurology service, each showing a neurology and neuroimaging different clinical spectrum. One patient presented with seizures, another one with headache and two patients developed neuropsychi- Migraine with aura as a stroke mimic atric and cognitive disorders. Another patient was hospitalized for an acute stroke. After a thorough evaluation, brain computed tomography scans revealed calcifications in basal ganglia, with one case revealing D.W. Kim, H.Y. Kim calcifications in basal ganglia, globus pallidus, cerebellar white matter Konkuk University Hospital, Department of Neurology, Seoul, Republic of and frontal subcortical white matter. Korea

Results Background Although the progressive nature of the disease is estimated, Migraine with aura is one of the causes of stroke mimics, but different neurological manifestations account for different cases. there are only a few studies on patients with migraine with aura who Family history and comorbidities should always be kept in mind initially presented with stroke-like symptoms. when analyzing the neurological signs and symptoms.

Methods Conclusion We retrospectively reviewed the 10 years' medical records of No clear cut correlation is found between the progressive nature patients who were treated with acute stroke management protocol. of the disease, neurological manifestations and neuroimaging data. We studied the frequency and characteristics of patients with a final The concept of frontal subcortical circuits, a system of loops linking diagnosis of migraine with aura. the basal ganglia and frontal brain regions, and functional neuroimaging may give additional data regarding the pathophysiology and Results clinical signs of the disease. Among the 2,397 patients with stroke mimics, migraine with aura was the final diagnosis in 36 patients (1.5%). The most common auras were sensory and brainstem auras followed by motor, visual and speech/language auras. One patient had transient atrial fibrilla- doi:10.1016/j.jns.2019.10.1508 tion during the migraine attack.

Conclusions Migraine with aura is not a rare cause of stroke mimic. Sensory WCN19-0321 and brainstem auras, rather than visual aura, were common in our patients. The co-occurrence of atrial ﬁbrillation and migraine should Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune be considered in these patients. disorders - Part II /CNS infections - Part II / dementia - Part II / doi:10.1016/j.jns.2019.10.1507 epilepsy - Part II / functional disorders and behavioral neurology /headache / metabolic and mitochondrial diseases / neuro-critical care / neurogenetics /neuroophthalmology / neurophysiology / neurorehabilitation / pain - Part II / sleep disorders - Part II / WCN19-0320 stem cells and gene therapy - Part II / training in nuerology - Part II / child neurology andneuroimaging Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune disorders - Part II /CNS infections - Part II / dementia - Part II / Ceroid lipofuscynosis type 7 in a patient with a novel homozy- epilepsy - Part II / functional disorders and behavioral neurology gous mutation in the MFDS8 gene due to chromosome 4 maternal isodisomy /headache / metabolic and mitochondrial diseases / neuro-critical care / neurogenetics /neuroophthalmology / neurophysiology / K. Ziora-Jakutowicza, D. Szczesniaka, M. Bednarska-Makaruka,I. neurorehabilitation / pain - Part II / sleep disorders - Part II / Stepniaka, L. Kotulaa, G. Witkowskib, M. Bozekb, R. Szymanczakc,E. stem cells and gene therapy - Part II / training in nuerology - Part Gajewskac, J. Gzikc, A. Boguszewska-Chachulskac, H. Sienkiewicz- II / child neurology andneuroimaging Jaroszb aInstitute of Psychiatry and Neurology, Department of Genetics, Fahr syndrome: A case series analysis Warsaw, Poland ARTICLE IN PRESS

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WES revealed a novel, homozygous missense variant c.1391C N T, p.(Ala464Val) in the MFDS8 gene. Familial segregation showed the maternal inheritance of the variant. Homozygosity of the variant, resulting from the chromosome 4 maternal isodisomy, was con- ﬁrmed using four short tandem repeats loci.

Conclusions^ The symptoms and signs observed in the affected male are compatible with the literature description of CLN7 and, therefore suggest that this novel variant in the MFSD8 gene is the cause of the disease. For proper genetic counseling uniparental disomy should be considered as a cause of an autosomal recessive disease.

doi:10.1016/j.jns.2019.10.1509

WCN19-0337

Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune disorders - Part II /CNS infections - Part II / dementia - Part II / epilepsy - Part II / functional disorders and behavioral neurology /headache / metabolic and mitochondrial diseases / neuro-critical care / neurogenetics /neuroophthalmology / neurophysiology / neurorehabilitation / pain - Part II / sleep disorders - Part II / stem cells and gene therapy - Part II / training in nuerology - Part II / child neurology andneuroimaging

New approaches to the early recovery of children with various forms of cerebral palsy b Institute of Psychiatry and Neurology, I-st Department of Neurology, R. Matmurodova, M. Egamovab Warsaw, Poland aTashkent, Neurology, Tashkent, Uzbekistan c Genomed HealthCare Center, Molecular Genetics Laboratory, Warsaw, bTashkent Medical Academy, Neurology, Tashkent, Uzbekistan Poland The disability of children with cerebral pathology ranks first in Background^ the field of child disability in terms of neurological profile. The neuronal ceroid lipofuscinosis type 7 (CLN7) is an autosomal recessive neurodegenerative, lysosomal storage disorder. Uniparen- Objective tal isodisomy is a rare cause of a disease inherited in an autosomal To develop a new special algorithm for early rehabilitation of recessive pattern. children with various forms of cerebral palsy (CP).

Objective^ Materials and research methods We report a case of CLN7 in a patient with a novel homozygous We examined 35 children aged 2 to 6 years with various forms of mutation in the MFDS8 gene due to chromosome 4 maternal CP. All children with CP received basic therapy, massage, and isodisomy (graph). physical therapy for 10 days. The control group (22 children), we continued to engage only at home. Patients and methods^ We reviewed data from a patient who was admitted to Genetic Results Counseling Unit with symptoms of a neurodegenerative disease and we tested and rejected the most effective points of influence, in negative family history. Whole exome sequencing (WES) and total over 50 for various parts of the body. At the initial stage of subsequent microsatellite loci analyses were performed. cerebral palsy treatment is a positive result after a relaxing mass of relaxing exercises. With the help of a planned set of exercises aimed Results^ at developing sensations, it is possible to improve the state of the The patient is 28-year-old Polish man, whose symptoms began at motor sphere in a child with a defect in the nervous system. We are the age of 8 with rapid cognitive decline, drug resistant epilepsy and engaged in relatively easy learning exercises that require continuous vision deterioration. Now the patient presents with blindness, movement. And with the astatic form, short-term exercises, which fl anarthria, ataxia, spasticity, hyperre exia and walking disability. make it possible to rest more often between exercises. Atonic form Ophtalmological examination revealed a cherry red spot in fundus of put forward several other problems. Children with this form of the eye. Biopsy of conjunctiva showed ceroid accumulation without paralysis especially suffered from exercises to maintain balance. A fl curvilinear intracellular accumulation of auto uorescent lipopigment variety and innovations in conducting classes were updated and storage material. repeated to consolidate the successes achieved. ARTICLE IN PRESS

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Conclusion WCN19-0367 The introduction of new approaches in complex therapy CP allows for rehabilitation in accordance with multidisciplinary approaches, providing a positive impact on its effectiveness and Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune improving the physical capabilities of patients. disorders - Part II /CNS infections - Part II / dementia - Part II / epilepsy - Part II / functional disorders and behavioral neurology doi:10.1016/j.jns.2019.10.1510 /headache / metabolic and mitochondrial diseases / neuro-critical care / neurogenetics /neuroophthalmology / neurophysiology / neurorehabilitation / pain - Part II / sleep disorders - Part II / stem cells and gene therapy - Part II / training in nuerology - Part WCN19-0344 II / child neurology andneuroimaging

Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune Targeted anterior cervical epidural blood patch in a patient with disorders - Part II /CNS infections - Part II / dementia - Part II / spontaneous intracranial hypotension epilepsy - Part II / functional disorders and behavioral neurology a a a b /headache / metabolic and mitochondrial diseases / neuro-critical W.S. Shahul Hameed , K.S.Y. Julienne , K. Chen , T. Chow Wei ,Y. Ming Huia, S. Ravindra Singha care / neurogenetics /neuroophthalmology / neurophysiology / aNNI-Singhealth, Neurology, Singapore, Singapore neurorehabilitation / pain - Part II / sleep disorders - Part II / bSingapore General Hospital, Radiology, Singapore, Singapore stem cells and gene therapy - Part II / training in nuerology - Part II / child neurology andneuroimaging Aims Spontaneous Intracranial Hypotension (SIH) often occurs second- Quantitative electroencephalographic (QEEG) for predicting func- ary to CSF leakage found in the spine. Patients most commonly tional outcome in ischemic stroke patients present with an orthostatic headache. Our aim is to discuss imaging findings of SIH and suggest a non-conventional targeted anterior G. Candrasaria, P. Paryonob, A. Asmedib approach epidural blood patch (EBP) as treatment alternatives for aRsup dr. Sardjito, Neurology, Sleman, Indonesia spontaneous intracranial hypotension (SIH) to a more conventional bgadjah mada university, Neurology, Yogyakarta, Indonesia. posterior approach EBP, provided that the site of cerebrospinal fluid (CSF) leakage is located anteriorly. Stroke is a leading cause for adult disability, which in many cases causes motor deficits leading to special health care and significant Methods high cost. Knowledge of important factors that may affect the We report a case of a 45-year-old gentleman^, with severe outcome of the therapy is necessary to make a reasonable prediction headache of 6 weeks duration, orthostatic in nature and not for individual patients. It has been reported that qEEG provides a responding to usual analgesics. map of cerebral activity reflecting the evolution of tissue lesions and penumbra tissue which predicts potential improvements. Results Magnetic resonance imaging (MRI) of the brain shows bilateral Objective subdural hygromas, diffuse pachymeningeal enhancement, reduced In this study we assessed the relationship between qEEG prepontine space and slit-like ventricles suggesting a diagnosis of measures and the functional outcome in acute stroke patients to SIH. Our patient underwent radionuclide cisternography with evaluate the utility of QEEg indices to predict functional outcome. intrathecal injection of technetium (Tc-99 m) diethylenetriamine pentaacetic acid (DTPA), showing focal extrathecal tracer accumu- Methods lation at the right anterolateral aspect of the C7/T1 level, likely Thirty patients were studied within 120 h of stroke ischemic representing the site of CSF leak. Following the initial attempt of a onset and thirty qEEG recordings were obtained. Absolute powers of conventional fluoroscopy-guided posterior-approach cervical EBP, 4 frequency bands (alpha, beta, delta and theta) and delta-theta/ the patient's symptoms recurred two days later. Thus we alpha-beta ratio (DTABR). The functional outcome was evaluated employed an anterior-approach cervical EBP under Computed using National Institutes of Health Stroke Scale (NIHSS). Tomographic (CT) guidance to better target the anterolateral leakage site. Results Following the targeted anterior-approach cervical epidural blood We found that theta power (r = 0.463; p = .010) and DTABR patch, our patient experienced immediate relief and is now symptom were significantly related to NIHSS during discharge (r = 0.381;p = free. .038). Delta power (r = 0.26), theta power (r = 0.77), DTABR (r = 0.377;p = .04) and Delta-alpha ratio (DAR) (r = 0.382;p = .037) Conclusion were significantly related to NIHSS score change. With the successful outcome of our patient, we strongly recommend considering the use of a direct anterior-approach EBP Conclusion for cases of refractory SIH secondary to an anterior cervical dural tear We Conclude that QEEG performed within the first 120 h of requiring an epidural blood patch. ischemic stroke might be a powerful tool predicting short-term outcome. doi:10.1016/j.jns.2019.10.1512 doi:10.1016/j.jns.2019.10.1511 ARTICLE IN PRESS

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WCN19-0371 TeleNeuroforma system for 30–60 min every day for 6 weeks. The control group maintained usual level of activity. All participants underwent a neurological and neuropsychological evaluation before Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune the final inclusion and after 6 weeks. disorders - Part II /CNS infections - Part II / dementia - Part II / epilepsy - Part II / functional disorders and behavioral neurology Results^ /headache / metabolic and mitochondrial diseases / neuro-critical The highest level of exercises difficulties performed by HD care / neurogenetics /neuroophthalmology / neurophysiology / patients in the motor functions were acheived by 39% of patients, neurorehabilitation / pain - Part II / sleep disorders - Part II / in cognitive excercises only by 26% of cases. The execution of 87% exercises of daily sessions was achieved by 71% HD patients. There stem cells and gene therapy - Part II / training in nuerology - Part was statistically significant improvement (p = .01) of motor func- II / child neurology andneuroimaging tioning in the training group assessed with UHDRS Motor, including walking abilities (8 MWT) and in the cognitive assessment with the – Clinical evaluation of the effectiveness of teleneuroforma A Stroop Color-Word Interference. There were no statistically signifi- home rehabilitation tool for patients with Huntington's disease cant changes in the control group.

K. Ziora-Jakutowicza, M. Rakowiczb, I. Stepniaka, M. Kruszyńskic,G. Conclusion^ Witkowskib, W. Krysaa, M. Pawliszc, A. Suleka, M. Polakc, D. Lisd,A. Systematic training resulted in improvement of motor, cognitive Chabudae, H. Sienkiewicz-Jaroszb and psychological functioning in HD patients. Our study provides aInstitute of Psychiatry and Neurology, Department of Genetics, evidence for the effectiveness of TeleNeuroforma for home neuro- Warsaw, Poland rehabilitation. It can be supplementary to standard rehabilitation. bInstitute of Psychiatry and Neurology, First Department of Neurology, The study was funded by Research and Development Project 18/ Warsaw, Poland IS-2/230/NCBR/2015–2017. cTitanis Ltd, Programmist Department, Warsaw, Poland dPolish Association of Huntington Disease, Board of Directors, Warsaw, doi:10.1016/j.jns.2019.10.1513 Poland eUniversity of Warsaw, Faculty of Physics, Warsaw, Poland

Background^ WCN19-0411 TeleNeuroforma is a computer program for home rehabilitation of motor and cognitive functions. It detects and analyses patient's movements via a webcam (motion-capture technology) and allows Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune for introducing telerehabilitation procedures – modifying exercise disorders - Part II /CNS infections - Part II / dementia - Part II / programs online and monitoring patients` progress. epilepsy - Part II / functional disorders and behavioral neurology /headache / metabolic and mitochondrial diseases / neuro-critical Objective^ care / neurogenetics /neuroophthalmology / neurophysiology / The aim of the study was to evaluate the efﬁcacy of the neurorehabilitation / pain - Part II / sleep disorders - Part II / TeleNeuroforma in rehabilitation of patients with Huntington's stem cells and gene therapy - Part II / training in nuerology - Part disease (HD). II / child neurology andneuroimaging

Material and methods^ 25 HD patients were assigned to the training group and 11 to the Mutational analysis of glutaryl CoA dehydrogenase gene in control group (table). The training group exercised with the Polish glutaric aciduria type-I patients: A study from India ARTICLE IN PRESS

372 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

M. Shaika, K.V. Tumkur Puttasidappab, V. Anakala Bassappaa bTokyo Medical and Dental University, Advanced Technology in aKarnatak University Dharwad, Biotechnology, Dharwad, India Medicine, Tokyo, Japan bNational Institute of Mental Health and Neurosciences, Neurochemis- cKanazawa Institute of Technology, Applied Electronics Laboratory, try, Bangalore, India Kanazawa, Japan dRicoh Institute of Future Technology, Ricoh Company- Ltd., Ricoh, Background Tokyo, Japan e Glutaric aciduria type I (GA-I), is a rare neurometabolic disorder Ricoh Institute of Future Technology, Ricoh Company- Ltd, Healthcare of amino acid metabolism caused due to mutations in glutaryl-CoA Business Center, Tokyo, Japan dehydrogenase (GCDH). In India, there are limited studies and controversial reports on its genetic etiology. Background Magnetic resonance imaging (MRI) is very useful, but we often Objective experience that nerve compression on MRI does not necessarily This study involves documentation of prevalent mutations in cause neurological symptom. Electrophysiological testing is another GCDH gene among GA-I patients from India. good method to diagnose spinal cord and spinal nerve function, but it is not suitable for recording neural activities in deep and complex Materials and methods structures such as the spine. On the other hand, Mag- The study was approved by institutional ethics committee. Forty- netospinography (MSG) can non-invasively visualize neural activi- two confirmed GA-I patients from unrelated families were recruited ties in the spinal canal because neuromagnetic field measurement is based on clinical, neuroimaging and biochemical profiles. Informed not affected by the surrounding tissue. In this study, we report MSG consent was obtained from study participants. The GCDH gene findings of patients with lumbar radiculopathy. analysis of GA-I patients was performed using polymerase chain reaction and Sanger’s sequencing. Methods We measured the neuromagnetic fields in five patients with L5 or Results S1 radiculopathy due to lumbar disc herniation using sup- Thirty-three different mutations were identified in GCDH gene. erconducting quantum interference device (SQUID) magnetometer Sixteen (48.5%) were novel and 17 (51.5%) were known or reported in response to the peroneal nerves and tibial nerves stimulation mutations. Among novel mutations, 9 (56.3%) were missense (averaging 2^000 times). Neural currents in the spinal canal were (I152M, S189T, W225S, R383S, E99G, R234P, Q144P, R372G and calculated from recorded magnetic signal and current distributions T344I), 1 (6.3%) nonsense (E414X), 1(6.3%) deletion (g.11634delG), were superimposed on X-ray images of the lumbar spine. 2(12.5%) insertion (g.10205_10206 Ins CTATGATCATC and g.11628_11629insT), and 3(18.8%) were silent (Y295Y, Q29Q and Results Q333Q). Five (31.3%) low excretor mutations (R94Q, R383S, I152M, The neural current calculated from MSG passed through the P286S and L221P) were found in this study. Exons 7, 8 and 10 of intervertebral foramen and flowed into the spinal canal and GCDH gene are hotspots for mutations. propagate to the cranium on the healthy side, in contrast, the neural current on the affected side attenuated at the L4/5 or L5/S1 disc Conclusion herniation site. In this preliminary large cohort study, we conclude that despite ethnic diversity R402W, is commonly occurring mutation in patients Conclusion with GA-I from India. The W225X mutation was found exclusively Magnetospinography visualizes conduction of spinal nerve exci- among GA-I patients from southern origin. The low excretor tation with high spatial and temporal resolution. We were able to phenotype of GA-I exists in Indian GA-I patients and P286S, is the evaluate quantitatively the decreased action current in lumbar most common low excretor mutation found among them. radiculopathy. Magnetospinography is expected to be a clinically useful modality. doi:10.1016/j.jns.2019.10.1514 doi:10.1016/j.jns.2019.10.1515

WCN19-0414 WCN19-0425 Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune disorders - Part II /CNS infections - Part II / dementia - Part II / Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune epilepsy - Part II / functional disorders and behavioral neurology disorders - Part II /CNS infections - Part II / dementia - Part II / /headache / metabolic and mitochondrial diseases / neuro-critical epilepsy - Part II / functional disorders and behavioral neurology care / neurogenetics /neuroophthalmology / neurophysiology / /headache / metabolic and mitochondrial diseases / neuro-critical neurorehabilitation / pain - Part II / sleep disorders - Part II / care / neurogenetics /neuroophthalmology / neurophysiology / stem cells and gene therapy - Part II / training in nuerology - Part neurorehabilitation / pain - Part II / sleep disorders - Part II / II / child neurology andneuroimaging stem cells and gene therapy - Part II / training in nuerology - Part II / child neurology andneuroimaging Magnetospinography is a noninvasive and useful modality to visualize electrophysiological activities in the lumbar CTA/CTG repeat sizes in the atxn8os gene afefct age at onset and radiculopathy phenotype in spinocerebellar ATAXIA type8

J. Hashimotoa, S. Kawabatab, Y. Hoshinob, K. Sekiharab, Y. Adachic,T. M. Samukawaa, M. Hiranoa, K. Saigoha, S. Kawaia, Y. Hamadab,D. Sasakia, T. Watanabed, Y. Miyanod, T. Yamagae, A. Okawaa Takahashic, Y. Nakamurad, S. Kusunokia aTokyo Medical and Dental University, Orthopaedic and Spinal Surgery, aKindai University, Faculty of Medicine, Neurology, Osaka-sayama, Tokyo, Japan Japan ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 373 bIzumi City General Hospital, Izumi, Japan M. Khan, M.S. Bin Mahfoodh, E. Cupler, H. Khan, G. Karim, P. Khalid, cNational Hospital Organization Osaka Minami Medical Center, Vascular Y. Alsaid Neurology, Kawachinagano, Japan King Faisal Specialist Hospital and Research Center, Jeddah- KSA, dSakai City Medical Center, Sakai, Japan Neurophysiology - Neurosciences, Jeddah, Saudi Arabia

Spinocerebellar ataxia type 8 (SCA8) is a rare autosomal Introduction^ dominant neurodegenerative disease typically characterized by pure Motor Evoked Potentials (MEPs) can be used to assess the cerebellar ataxia, and is caused by expanded CTA/CTG repeats in the integrity of the descending corticospinal tract. With Trans-magnetic untranslated region of the ATXN8OS gene. Some patients had Stimulator (TMS), is positioned over the scalp to elicit MEPs form parkinsonism, without causal explanation. We analyzed the ATXN8OS upper or lower limbs. MEPs can be tested by recording Compound gene in 257 Japanese patients with ataxia and 80 patients with muscle action potentials (CMAPs) of several muscles. Parkinson's disease or related disorders. We systematically reassessed 123 patients with SCA8, both our patients and those Material and methods^ reported in other studies for analyzing repeat sizes, age at onset, and TMS of the brain was performed on 25 (13 male; 12 female) adult phenotypes. Two patients with progressive supranuclear palsy (PSP) healthy volunteers with height ranged between 158 and 173 cm. had mutations in the ATXN8OS gene. Systematic analyses revealed CMAPs recorded from abductor Pollicis Brevis (APB) and Tibialis that patients with parkinsonism had signiﬁcantly shorter CTA/CTG Anterior (TA) muscles Peripheral Nerve Conduction Studies performed repeat expansions and older age at onset than those with to record M- Latencies and F- Latencies. Central conduction time (CCT) predominant ataxia. We also found a signiﬁcant inverse relationship assessed by subtraction of peripheral latencies from central latencies. between repeat sizes and age at onset in all patients, which has not been reported previously. We also analyzed 93 unaffected individ- Results^ uals with repeat expansions in the ATXN8OS gene. Available data The normative data was analyzed on SPSS version 14 showed: suggests that only approximately 20% of those individuals surpassed the oldest ages for patients with equivalent repeat sizes, suggesting ^Conclusion that several unaffected individuals might come to be affected later in The normative data for local Saudi Arabian population is very life. We also summarized the results of brain functional imaging in much comparable with the international normative data. There was the present and reported patients with SCA8, including our patient a positive correlation between CCT to height. In general, TMS is with PSP. Our results may contribute to genetic counseling, improve useful when the clinical question pertains to certain segments of understanding of the pathomechanism, and extend the clinical proximal / central conduction delay. It is non invasive and less phenotype of SCA8. expensive but very useful investigation. doi:10.1016/j.jns.2019.10.1516 doi:10.1016/j.jns.2019.10.1517

WCN19-0459 WCN19-0475

Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune disorders - Part II /CNS infections - Part II / dementia - Part II / disorders - Part II /CNS infections - Part II / dementia - Part II / epilepsy - Part II / functional disorders and behavioral neurology epilepsy - Part II / functional disorders and behavioral neurology /headache / metabolic and mitochondrial diseases / neuro-critical /headache / metabolic and mitochondrial diseases / neuro-critical care / neurogenetics /neuroophthalmology / neurophysiology / care / neurogenetics /neuroophthalmology / neurophysiology / neurorehabilitation / pain - Part II / sleep disorders - Part II / neurorehabilitation / pain - Part II / sleep disorders - Part II / stem cells and gene therapy - Part II / training in nuerology - Part stem cells and gene therapy - Part II / training in nuerology - Part II / child neurology andneuroimaging II / child neurology andneuroimaging

To report trans-cranial magnetic stimulation to describe the Blood ﬂow volume measurement in cervical and intracranial NORMAL LATENCIES OF major nerve with central conduction time arteries using quantitative magnetic resonance angiography and among Saudi Arabian population; a hospital based study duplex sonography (BOCACCIA) – A prospective observational study ARTICLE IN PRESS

374 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

M. Roubeca,D.Školoudíka, T. Fadrnaa, J. Fiedlerb, M. Reiserc,J. WCN19-0498 Kubáled, T. Hrbáče,P.Kešnerováf, K. Langovág, R. Herzigh aFaculty of Health Sciences Palacký University Olomouc, Center for Research and Science, Olomouc, Czech Republic Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune bČeské Budějovice Hospital, Department of Neurosurgery, České disorders - Part II /CNS infections - Part II / dementia - Part II / Budějovice, Czech Republic epilepsy - Part II / functional disorders and behavioral neurology cČeské Budějovice Hospital, Department of Neurology, České Budějovice, /headache / metabolic and mitochondrial diseases / neuro-critical Czech Republic care / neurogenetics /neuroophthalmology / neurophysiology / dČ ě Č ě eské Bud jovice Hospital, Department of Radiology, eské Bud jovice, neurorehabilitation / pain - Part II / sleep disorders - Part II / Czech Republic stem cells and gene therapy - Part II / training in nuerology - Part eUniversity Hospital Ostrava, Department of Neurosurgery, Ostrava, II / child neurology andneuroimaging Czech Republic f2nd Medical Faculty of Charles University, University Hospital Motol, Department of Neurology, Praha, Czech Republic Spontaneous intracranial hypotension with endolymphatic gFaculty of Medicine and Dentistry Palacký University Olomouc, hydrops: A case report Institute of Molecular and Translational Medicine, Olomouc, Czech Republic K. Nama, J.H. Parkb hCharles University Faculty of Medicine and University Hospital Hradec aCollege of Medicine- Dongguk University, Departments of Physical Králové, Department of Neurology, Hradec Králové, Czech Republic Medicine & Rehabilitation, Goyang-si, Republic of Korea bInje University College of Medicine, Department of Neurology, Seoul, Background Republic of Korea Cerebral blood flow volume (BFV) is important factor for accurate diagnosis of neurovascular diseases and treatment indication. Study Aim of investigation aims to assess correlations of BFV measurements in cervical and Spontaneous intracranial hypotension may be defined as a intracranial arteries between quantitative magnetic resonance angi- postural headache syndrome without preceding major head trauma ography (qMRA) and duplex sonography (DS). or dural puncture, associated with low cerebrospinal fluid volumes and opening pressure. Orthostatic headache is a cardinal clinical Methods manifestation, associated symptoms may include neck stiffness, Consecutive patients with suspicion of cerebral vascular pathol- nausea, diplopia, vertigo, tinnitus and deafness in spontaneous ogy were included in prospective study. All patients underwent intracranial hypotension. We describe a woman presented with a 1 qMRA and DS of the cervical and intracranial arteries with week history of headache who was diagnosed with endolymphatic measurement of BFV in bilateral common (CCA), internal (ICA) and hydrops after cerebrospinal fluid analysis. externa carotid arteries, vertebral arteries (VAs), middle, anterior and posterior cerebral arteries, posterior communicanting arteries Methods and basilar artery (BA). DS was performed using two ultrasound A 40 years old woman presented with a 1 week history of machines by experienced sonographers. Two patients were exam- headache. The pain was described as generalized non-throbbing and ined twice. Correlations between BFV measurements were evaluated complain a mild febrile sensation. She admitted a local hospital but using Spearman's correlation coefficient and inter-class correlation her symptoms were continued. On admission, she had severe coefficient (ICC). orthostatic headache, nausea, vomiting and both ear fullness, tinnitus. No photophobia, phonophobia, and other autonomic Results dysfunction were exist. She had never been previous head or back Totally 21 subjects (15 males, mean age 56.3±6.2 years) were trauma and cerebrospinal fluid tapping history. No abnormality in included to the study. DS inter-investigator correlation was excellent laboratory result. cerebrospinal fluid exam showed 4cmH2O pres- (ICC=0.972) as well as intra-investigator correlations of both qMRA sure, RBC 780, WBC 0, glucose 39 (serum 83), protein 95. Brain MRI and DS (ICC˃0.990). Mostly high correlations were recorded between showed diffuse prominent dural enhancement by gadolinium. Pure qMRA and DS CBF measurements in particular cervical arteries but tone audiogram: decreased hearing in low tone. It suggested only low to moderate correlations were obtained for intracranial endolymphatic hydrops. arteries. Mean differences between BFV measurements in CCA and its branches were 10.9±8.1%/15.0±11.9% when using qMRA/DS, in ICA Results siphon and its branches 13.5±11.8%/35.4±34.2% when using qMRA/ She treated by hydration and symptomatic treatment and DS, and in both VAs and BA 24.1±19.7%/44.9±44.0% when using symptoms were improved. qMRA/DS, resp. Conclusions Conclusion In conclusion, a compensatory expansion of the endolymphatic DS and qMRA are highly reproducible methods for blood flow compartment would induce endolymphatic hydrops and in the volume measurement in cervical and intracranial arteries in routine result, it causes low tone hearing loss. Because spontaneous clinical practice. intracranial hypotension may cause endolymphatic hydrops, it is Supported by MHCR grants 16-29148A, 16-30965A, 17-31016A and highly probable that audiometry is necessary to the orthostatic UP JG_2019_004. hypotension patient with ear fullness. doi:10.1016/j.jns.2019.10.1518 doi:10.1016/j.jns.2019.10.1519 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 375

WCN19-0499 WCN19-0508

Varicella zoster meningitis with only headache Possible neuroprotective effect of cholorogenic acid and protocatechuic acid combinational approach against scopol- K. Nama, J.H. Parkb amine-induced cognitive impairment in a mouse model as a aCollege of Medicine, Dongguk University, Departments of Physical multitargeted ligand Medicine & Rehabilitation, Goyang-si, Republic of Korea bInje University College of Medicine, Department of Neurology, Seoul, E. Yadav Republic of Korea SHUATS, Pharmaceutical Sciences, Prayagraj, India

Aim of investigation Cholrogenic acid and protocatechuic acid are natural polypheno- Varicella zoster virus (VZV) can infect the central nervous system lic compounds utilized in various neurodegenerative diseases which (CNS), and complications include meningitis and encephalitis in acted on CNS by crossing the blood brain barrier (BBB) either intact elderly or immunocompromised patients. In CNS VZV infection, high or in the form of metabolite. In present study we estimated the fever and skin eruption are key diagnostic hallmarks, and diagnosis is synergistic neuropharmacological potential of cholorogenic acid and confirmed with cerebrospinal fluid analysis. We describe a 40-year- protocatechuic acid (CA+PA) on hippocampal memory deficit old healthy man presented with only headache without fever or skin induced by scopolamine injection in rodents. In vitro anticholines- eruption who was diagnosed with VZV meningitis after cerebrospi- terase activity along with its inhibition kinetics as well as in silico nal fluid analysis. molecular docking studies for molecular interaction was performed on CA+PA. Further considering the in vitro results, antiamnesic Methods effect of CA+PA was explored by in vivo method. Swiss albino mice We report a case through review of medical records. He were orally administered with CA+PA, donepezil and vehicle for complained of a 1-week history of severe headache and nausea. 21days and then cognitive function was assessed by using behavioral Prior to presentation, he experienced migraines once or twice per models (Elevated plus maze and Hebbs William maze task) as well month, which were different than the headache that caused him to as homogenized brain hippocampus was used for enzymatic visit the outpatient clinic. The headache was severe and persistent antioxidant level, AChE inhibitory activity determination and gene with no relieving factors, including any drug. The neck was supple expression determination. Scopolamine injection elevated the TL without any stiffness. The other neurological assessments were (transfer latency) and TRC (Time taken to reach the reward within normal limits. A brain magnetic resonance imaging showed chamber) in mice, which were significantly reversed (pb0.05) by no acute intracranial abnormalities, such as bleeding, infarction, or pretreatment with CA+PA in a dose dependant manner. Further- malignancy. CSF examination confirmed aseptic meningitis, and VZV more, hippocampal acetylcholinesterase, antioxidant enzymes level, DNA was detected on CSF polymerase chain reaction. neurogenesis and their concerning pathways were distinctly altered by scopolamine, which were markedly ameliorated with CA+PA Results pretreatment. Brain derived neurotrophic factor (BDNF) and phos- The patient was admitted to the hospital, and, after the CSF study, phorylated cAMP response element-binding protein (pCREB) gene he was treated with full hydration and acyclovir. The headache was expression were significantly accelerated by CA+PA completely reversed after one week. preadministration. The current investigation suggests that CA+PA could be a strong neuroprotective agent against amnesia in AD like Conclusions pathology. Physicians should consider VZV meningitis in a healthy young patient without any infectious signs, such as fever or skin eruption. doi:10.1016/j.jns.2019.10.1521 Detailed CSF analysis, including PCR for DNA, should be evaluated when symptoms do not improve by common medication.

Acknowledgements and/or funding source None doi:10.1016/j.jns.2019.10.1520 ARTICLE IN PRESS

376 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-0509 WCN19-0522

Poster shift 04 - ATAXIA and cerebellar disorders / autoimmune Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II /CNS infections - Part II / dementia - Part II / disorders - Part II/CNS infections - Part II/dementia - Part II/ epilepsy - Part II / functional disorders and behavioral neurology Epilepsy - Part II/functional disorders and behavioral neurology/ /headache / metabolic and mitochondrial diseases / neuro-critical headache/metabolic and mitochondrial diseases/neuro-critical care / neurogenetics /neuroophthalmology / neurophysiology / care/neurogenetics/neuroophthalmology/neurophysiology/neu- neurorehabilitation / pain - Part II / sleep disorders - Part II / rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells stem cells and gene therapy - Part II / training in nuerology - Part and gene therapy - Part II/training in neurology - Part II/child II / child neurology andneuroimaging neurology and neuroimaging

Exploratory trial to determine an optimum parameter set for The evaluation of a role of the inconsistency of connective tissue real-time neuromonitoring in cerebellopontine angle surgery in the development of pathological deformations of cerebral vessels in patients with primary headaches S.I. Hirano Osaka Dental University, Physiology, Osaka, Japan M. Salokhiddinova, E. Rosetteb aTashkent Medical Academy, Neurology, Olmazor, Uzbekistan b Background University of Aberdeen, Medical Imaging, Aberdeen, United Kingdom Cerebellopontine angle (CPA) surgery sometimes causes an acoustic dysfunction due to surgical manipulation. Although many Background neurophysiological methods were introduced, none meet the speciﬁc Vascular malformations of the brain may cause headaches, needs of intraoperative monitoring. seizures, strokes, or bleeding in the brain. Some researchers believe that the type of malformation determines the symptoms and Objective progression of the disease. To realize an intraoperative acoustic monitoring that will provide a rapid functional alert, I investigated a real-time technique with Objective processing the 40 Hz auditory steady-state response (40Hz-ASR) in To analyse manifestations of connective tissue failure in patients CPA surgery. with pathological deformations of cerebral vessels

Materials and methods Methods I have obtained an approval from Institutional Animal Use The study included 60 patients aged 17-46 years with primary Committee. Twelve rabbits underwent surgical interventions. The headaches treated in 1st Republic Clinic in Tashkent between 2016– 40Hz-ASR signals were immediately analyzed by the Fast Fourier 2018 years. Patients were divided into 2 groups: patients with (30 Transformation (FFT) method. The spectral power (SP) and the patients) and without (30 patients) abnormalities of cerebral vessels. phase coherence measure (PC) were calculated simultaneously. The The examination included a clinical neurological examination, MRI reproducibility and stability of the SP and PC were studied under a with angiography and duplex scan variety of experimental conditions. Results Results 1st group showed that the folded skin was easily pulled back by This analytical method proved the usability of this response. several centimeters, and then quickly returned to its original The PC increased as the number of sweeps that were averaged position. At the time of the study, chronic arthralgia without signs increased. In contrast, the SP declined. In the experiment to seek of joint inflammation was found in 13.7% of patients. Scoliosis, the optimum parameters of this series, both the PC and the SP kyphosis, their combination, as well as flat feet were noted. At the reached a plateau at an average of approximately 30 sweeps. At a same time, in the observed patients, the breakdown in the strength sample size of 15 or 20, the dispersion of PC values was minimal. of the internal organs tissues manifested itself in the form of hernia, The calculation of PC at an average of 30 sweeps and a sample 7.8% of cases, the lowering of the internal genitalia-9.7%, in 21.3% in size of 15 to 20 permitted evaluation of the 40Hz-ASR with the anamnesis, premature rupture of the membranes, premature sufficient reliability and sensitivity. birth and postpartum hemorrhage

Conclusion Conclusion The optimum values for each parameter proved to permit real- Diffuse inconsistency of connective tissue was revealed in time monitoring with sufﬁcient accuracy and rapidity. The on-line patients with deformations of cerebral vessels. Furthermore, to measurement of the PC and SP will enable rapid evaluation of identify pathology from the connective tissue, it is necessary to acoustic and brainstem functions. include a screening of connective tissue in the primary headache examination doi:10.1016/j.jns.2019.10.1522 doi:10.1016/j.jns.2019.10.1523 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 377

WCN19-0526 WCN19-0548

Clinical evaluation of application percutaneous electrical nerve The spectrum of brain MRI ﬁndings of methanol intoxication stimulation in patients with chronic tension headache after the methanol affair in the Czech Republic

M. Salokhiddinova, E. Stamoub M. Vaneckovaa, J. Klempirb, D. Pelclovac, O. Bezdicekb, I. Liskovab,H. aTashkent Medical Academy, Neurology, Olmazor, Uzbekistan Brozovab, K. Polakovab, P. Diblikd, M. Miovskye, J. Hubacekf,J. bUniversity of Aberdeen, Medical Imaging, Aberdeen, United Kingdom Hlusickac, K. Kotikovac, E. Ruzickab, Z. Seidla, B. Marechalghi,T. Koberghi, S. Zacharovc a Background Charles University- First Faculty of Medicine and General University Tension-type headache can develop into chronic tension-type Hospital, MR unit- Department of Radiology, Prague, Czech Republic b headache when it becomes more disabling Charles University- First Faculty of Medicine and General University Hospital, Department of Neurology and Center of clinical neuroscience, Objective Prague, Czech Republic c To study and assess the features of the course of the anomaly of Charles University- First Faculty of Medicine and General University cerebral vessels in patients without cephalic syndrome Hospital, Department of Occupational Medicine- Charles University, Prague, Czech Republic d Methods Charles University- First Faculty of Medicine and General University The study involved 50 patients treated in 2nd Clinics of Tashkent Hospital, Department of Opthalmology, Prague, Czech Republic e Medical Academy between 2015–2017 years. Anomalies of cerebral Charles University- First Faculty of Medicine and General University vessels without cephalic syndrome were examined. Clinical-neuro- Hospital, Department of Addictology, Prague, Czech Republic f logical, neurological imaging (MSCT with angiography, DS ACS) was Institute for Clinical and Experimental Medicine, Center for Experimen- performed tal Medicine, Prague, Czech Republic gSiemens Healthcare AG, Advanced Clinical Imaging Technology, Results Lausanne, Switzerland; h The study showed in 43 (86%) cases, the combination of École Polytechnique Fédérale de Lausanne EPFL, Signal Processing deformation of vertebral arteries with its hypoplasia was revealed Laboratory LTS 5, Lausanne, Switzerland i in 3 patients (6%), only vertebral arteries (VA) deformity, in one Lausanne University Hospital and University of Lausanne, Department cases, one-sided in combination with asymmetry of blood flow in the of Radiology, Lausanne, Switzerland internal carotid arteria (ICA). In the remaining two cases, the tortuosity of both VA and ICA was observed. At one patient with Background and purpose the diagnosis chronic leptomeningitis of fossae cranii posteriori To demonstrate the variability of brain magnetic resonance (FCP), hypoplasia of VA is revealed. It should be noted that in 3 (6%) imaging (MRI) findings in patients of acute methanol poisoning. patients with deformities only ICA, and one-sided, in the absence of headaches, dizziness was noted, which requires subsequent dynamic Material and methods monitoring and recruitment of patients. Patients with VA anomalies A total of 51 patients were scanned an a 3T MAGNETOM Skyra also had different degrees of ICA deformity in 9 (18%) cases in scanner (Siemens Healthcare, Erlangen, Germany) with the same combination with its hypoplasia, without hemodynamically signifi- protocol. The MR protocol included 3D T1-weighted, 2D T2- cant disorders of cerebral circulation. weighted, fluid-attenuated inversion recovery, susceptibility- weighted imaging as well as coronal T2-weighted images with fat Conclusion sat for visualization of the optic nerves. 3D T1-weighted images were Thus, in the group of patients without cephalic syndrome, but processed using the MorphoBox prototype software yielding estima- with anomalies of cerebral vessels, significant changes in VA were tion of regional brain volumes. observed, while pathological tortuosity's and underdeveloped ICA, although present, were hemodynamically insignificant in relation to Results cerebral blood circulation Imaging methods revealed a positive finding associated with methanol intoxication in 24 patients (47.1%), mostly seen as doi:10.1016/j.jns.2019.10.1524 symmetrical lesions in the putamen, which afflicted 16 patients (31.4%) (Fig. 1). Haemorrhage was detected in 16 cases (31.4%), ARTICLE IN PRESS

378 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx deposits in white matter with localisation primarily subcortically in 5 siderosis of the central nervous system. The etiological assessment cases (9.8%), and lesions in the region of the pallidum in 7 cases for a source of bleeding was performed, including: a medullary MRI (13.7%) (Fig. 2). There were lesions in the brainstem in 7 patients and arteriography. Not objecting any obvious cause of bleeding. A (13.7%), and lesions in the cerebellum were found in one case. neuropsychological assessment was performed revealing a deterio- Compared to a control group, survivors of methanol poisoning had ration of cognitive functions for one patient. The diagnosis of smaller putamina, caudates and pallida (p b 0.01). idiopathic SS-CNS was retained for all patients.

Conclusions Conclusion The most common finding showed damage of the putamen, SS-CNS should be highly suspected in patients with progressive which is a predilection area of methanol poisoning. An interesting sensorineural hearing loss, ataxia, and signs of pyramidal tracts, MRI finding was the relatively frequent occurrence of selective lesions of of brain and whole spinal cord should be undertaken to confirm the the pallidum, which is found more often in other types of diagnosis. The cause of hemorrhage is not identified in most cases. intoxication. Atrophy of basal ganglia was prominent in patients after intoxication. doi:10.1016/j.jns.2019.10.1526 Supported by grant No. 16-27075A, RVO-VFN 64165. doi:10.1016/j.jns.2019.10.1525 WCN19-0566

Poster shift 04 - Ataxia and cerebellar disorders/autoimmune WCN19-0552 disorders - Part II/CNS infections - Part II/dementia - Part II/ Epilepsy - Part II/functional disorders and behavioral neurology/ Poster shift 04 - Ataxia and cerebellar disorders/autoimmune headache/metabolic and mitochondrial diseases/neuro-critical disorders - Part II/CNS infections - Part II/dementia - Part II/ care/neurogenetics/neuroophthalmology/neurophysiology/neu- Epilepsy - Part II/functional disorders and behavioral neurology/ rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells headache/metabolic and mitochondrial diseases/neuro-critical and gene therapy - Part II/training in neurology - Part II/child care/neurogenetics/neuroophthalmology/neurophysiology/neu- neurology and neuroimaging rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in neurology - Part II/child Quality of life and caregivers' burden of Parkinson's disease: neurology and neuroimaging Analysis of a cohort from Rio de Janeiro/Brazil

Idiopathic superficial siderosis of the central nervous system: V. Neriabc, C. Rosasc, A.C. Leite Ribeiroc, A.M. Nunesc, M. Alvesc,A. Experience of the neurology department of UHC Hassan II of Fez Fernandesc, G. Licassalib and review of the literature aUniversidade Federal do Estado do Rio de Janeiro, Neurology, Rio de Janeiro, Brazil R. Benkhadra, N. Chtaou, A. Midaoui, Z. Souirti, M.F. Belahsen bCentro de Alzheimer e Parkinson, Parkinson Disease, Campos dos UHC Hassan II, Neurology, Fès, Morocco Goytacazes, Brazil cFaculdade de Medicina de Campos, Neurology, Campos dos Goytacazes, Introduction Brazil The superficial siderosis (SS) of the CNS is an acquired neurological disorder resulting from hemosiderin deposition in the Objectives subpial layers of the brain, cranial nerves and spinal cord producing a This study focused on the impact of the clinical and social features progressive white matter and neuronal damage. on the quality of life (QoL) and to recognize the burden felt by Parkinson’s disease (PD) caregivers. Objective To compare the neurological results of three patients suffering Methods from progressive ataxia due to superficial siderosis with the Included PD caregivers and was undertaken at the Alzheimer and magnetic resonance imaging (MRI) findings. Parkinson Disease Center, Rio de Janeiro. QoL of the caregivers was evaluated by Flanagan's Quality of Life Scale (1982) and Zarit Burden Methods Inventory (ZBI) assessed caregiver burden. Clinical features of PD This is a retrospective study of 3 patients whose main symptom patients were assessed using Hoehn and Yahr and Schwab and was progressive ataxia, 2 of them also had a hearing loss. Cerebral England Scale. MRI showed SS-CNS. These 3 patients are the only cases diagnosed in the neurological department in Fez since September 2014 until Mars Results 2019. 61 caregivers were interviewed (mean age 52.07, ±15.4). The majority were women (78.7%). 96.7% were relatives, and 49.2% were Results only activity as caregivers. The mean scoring in the Flanagan Scale The average age of our patients was 59 years old. Gait instability was 78,4 (±14,5), among men, mean 83,6 (±11,8), and women 77 was the main symptom of all patients, two of them presented also a (±15,02). The items of greatest satisfaction were related to physical hearing loss and only one patient had spasticity of the 2 lower limbs. well-being/health and material, and relationship with people. The All patients underwent cerebral MRI T2 gradient echo showing a items of greatest dissatisfaction were personal development and ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 379

recreation. Mean ZBI scores were 29.3 (±13.7); among women, the Objective mean was 29.7 (±14.4), men, 27.5 (±11.2). There was a statistical Parkinsonʼs disease (PD) and essential tremor are two of the most signiﬁcance between ZBI and the caregiver's perception of QoL (p b common movement disorders and are still associated with high rates 0.001); there was no statistical relationship between ZBI and Hoehn of misdiagnosis. New smart device system (SDS) together with & Yahr, Schwab and England Scale, p N 0.05. smartphone applications are being examined and tested for their potential to monitor and manage patients with PD. To develop Conclusions methods for analyzes and monitor of gait data via SDS and identify QoL was considered moderate according to the Flanagan Scale, new phenotypical characteristics in PD patients. with higher levels of satisfaction in the areas of physical well-being and relationships with people. According to ZBI, the mean overload Method was considered mild-moderate, maintaining this result between men Total of 68 PD patients were taken as subject with an equal ratio and women. of male and female and age group between 60 to 75 years. SDS (Wearable monitoring device MI band) was put on the wrist of PD doi:10.1016/j.jns.2019.10.1527 patients for 30 days. The electronic questionnaires will cover data on medication, family history and non-motor symptoms. Wearable assistive bands, automatically provides a cueing sound when freezing of gait (FOG) is detected and which stays until the subject resumes WCN19-0570 walking.

Result Poster shift 04 - Ataxia and cerebellar disorders/autoimmune Present results shown that SDS reading showed there was a disorders - Part II/CNS infections - Part II/dementia - Part II/ normal heart rate, more calorie burnt with better control of sugar Epilepsy - Part II/functional disorders and behavioral neurology/ control and average good sleep count in more physically workout, headache/metabolic and mitochondrial diseases/neuro-critical include walking in PD patients compared to less physically workout ﬁ care/neurogenetics/neuroophthalmology/neurophysiology/neu- PD patients, identi ed by professional physiotherapists. Smart device reading showed that after changing lifestyle routine among less rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells physically active PD patients, their FOG events normalize with less and gene therapy - Part II/training in neurology - Part II/child requirement of drug dose. neurology and neuroimaging Conclusion A smart device system (wearable technology band) to identify Future work considers integration with deep brain stimulation, new phenotypical characteristics in Parkinsonʼs disease patients dissemination into further sites and patient's home setting as well as integration with further data sources as neuroimaging and biobanks. V. Sharma, S. Sharma University College of Medical Sciences UCMS, Department of Neurology, doi:10.1016/j.jns.2019.10.1528 Delhi, India ARTICLE IN PRESS

380 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-0575 WCN19-0616

A rapid screening method for CADASIL by means of dot blot Russian versions of scales for acute disorders of consciousness analyses using biopsied skin samples (Glasgow Coma Scale, Full Outline of Unresponsiveness): Valida- tion study Y. Ma, M. Ueda, A. Ueda, T. Yamashita, Y. Ando Kumamoto University, Neurology, Kumamoto, Japan K. Ilinaa, I. Lunevab, D. Yusupovac, A. Ziminc, D. Sergeevb, A. Zaitsevd, M. Domashenkoe, V. Samorukovf, Y. Ryabinkinab, N. Suponevac,M. Abstract Piradovg Cerebral autosomal dominant arteriopathy with subcortical aMoscow State University, Faculty of Fundamental Medicine, Moscow, infarcts and leukoencephalopathy (CADASIL) is a hereditary cerebral Russia small vessel disease caused by NOTCH3 mutations. As the diagnosis bResearch Center of Neurology, Department of Anesthesiology and for CADASIL, we often perform histopathological analyses such as Intensive Care, Moscow, Russia immunostainings for detecting vascular Notch3 deposits and elec- cResearch Center of Neurology, Department of Neurorehabilitation and tron microscopic analyses for detecting granular osmiophilic mate- Physiotherapy, Moscow, Russia rial (GOM) deposits using skin biopsy specimens, in addition to dI.M. Sechenov University, Department of Foreign Languages, Moscow, genetic testing for the NOTCH3 gene. However, detection of Notch3 Russia and GOM deposits are technically difﬁcult in skin vascular walls by eCentral Clinical Medical Unit, Magnitogorsk, Russia those histopathological analyses. fBotkin Municipal Hospital, Department of Intensive Care №35, Moscow, Russia gResearch Center of Neurology, Moscow, Russia Objective The aim of this study is to evaluate the usefulness of dot blot Background analyses using biopsied skin samples in patients with and without The aim of this study is to explore Russian adaptation of Glasgow CADASIL. Coma Scale (GCS) and Full Outline of Unresponsiveness (FOUR) with Patients, materials and methods: We used biopsied skin samples the assessment of psychometric properties. (See Fig. 1.) obtained from 11 patients with CADASIL and 12 control patients. To detect Notch3 deposits in those biopsied skin samples, we performed Methods dot blot analyses using tissue homogenates of skin samples with The original versions of GCS and FOUR were translated into three different kinds of antibodies against Notch3, in addition to Russian by two independent interpreters and then back-translated immunostainings for the vascular Notch3 deposits and electron into English. The resulting version considering linguistic features was microscopic analyses for detecting GOM in the skin vascular walls. approved for use. Two researchers (neurology residents) prospectively evaluated 60 patents with FOUR and GCS in a neurological Results critical care unit. On the ﬁrst day, rater 1 and 2 independently scored In dot blot analyses, we detected Notch3 in insoluble fractions of each patient, the comparison of their evaluations was used to assess tissue homogenates of the skin samples obtained from CADASIL patients, but we did not detect Notch3 in insoluble fractions of tissue homogenates of the skin samples obtained from control patients.

Conclusion Dot blot analyses may be a useful diagnostic tool to detect Notch3 deposits in the skin vascular Notch3 deposits in CADASIL patients. doi:10.1016/j.jns.2019.10.1529 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 381 inter-rater reliability. In 15 mins break rater 1 had to make second WCN19-0623 evaluation, by this test-retest reliability was measured. In 24 hrs observer 1 had to terminate third evaluation (Fig.1). The comparison of 1st and 3rd assessment by the same person was used to deﬁne the Poster shift 04 - Ataxia and cerebellar disorders/autoimmune sensitivity to changes in the state of patients with acute disorders of disorders - Part II/CNS infections - Part II/dementia - Part II/ consciousness. Epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical Results care/neurogenetics/neuroophthalmology/neurophysiology/neu- ﬁ As a result of completed phase of the linguistic rati cation we have rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells Russian versions of GCS and FOUR, that are adapted for use in our country. and gene therapy - Part II/training in neurology - Part II/child 35 patients in acute period of stroke have gone through the assessment, neurology and neuroimaging 140 evaluations have been made with the developed versions.

fi Discussion and conclusions Role of clinical neurophysiology in classi cation of neuropathy in The Russian versions of both scales are available for use in clinical the absence of nerve biopsy practice. Further patient recruitment is going on in order to confirm validity of our versions by statistics. B. Rajendran, G. Ram Kauvery Hospital, Neurology and Neurophysiology, Chennai, India doi:10.1016/j.jns.2019.10.1530 Neuropathy is quite a common neurological problem that we come across in a country like India, where we many people are afflicted with Diabetes Mellitus and B12 deficiencies. Inflammatory WCN19-0620 neuropathies like Guillian Barre Syndrome are also common in this part of the world. All these patients are not referred for nerve biopsies and in Poster shift 04 - Ataxia and cerebellar disorders/autoimmune certain centres outside main cities, there is also no accessibility to disorders - Part II/CNS infections - Part II/dementia - Part II/ nerve biopsy facility. Epilepsy - Part II/functional disorders and behavioral neurology/ In such scenarios, neurophysiology done properly following headache/metabolic and mitochondrial diseases/neuro-critical correct protocols can elucidate a lot of information required for fi care/neurogenetics/neuroophthalmology/neurophysiology/neu- classi cation and for planning treatment for these patients. rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells doi:10.1016/j.jns.2019.10.1532 and gene therapy - Part II/training in neurology - Part II/child neurology and neuroimaging

Spontaneous nontraumatic pneumocephalus presenting as thun- WCN19-0704 derclap headache

K.H. Kima, S.H. Jeongb Poster shift 04 - Ataxia and cerebellar disorders/autoimmune aYoungwol Hospital, Neurology, Yeongwol-Gun, Republic of Korea disorders - Part II/CNS infections - Part II/dementia - Part II/ bYonsei University College of Medicine, Department of Neurology, Seoul, Epilepsy - Part II/functional disorders and behavioral neurology/ Republic of Korea headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- Pneumocephalus indicated the presence of presence of air within rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells the cranial cavity. It is a rare condition caused by several etiologic and gene therapy - Part II/training in neurology - Part II/child factors, such as head injuries, surgical interventions, infections and neurology and neuroimaging neoplasms. We described a patient with spontaneous nontraumatic pneumocephalus presenting as thunderclap headache. A 40-year-old man with no relevant medical history visited our Reversible cerebral vasoconstriction syndrome coexisting con- hospital with a sudden onset of severe headache without any vexal subarachnoid hemorrhage and watershed infarction neurological deﬁcit. He coughed once severely while wearing a motorcycle helmet. After 10 mins, his headache was developed. Brain J.W. Shina,E.Oha, D.H. Kimb computed tomography (CT) scan was performed to rule out aChungnam National University Hospital, Neurology, Daejeon, Republic secondary headache. Brain CT revealed small volume of air in near of Korea the vertical sinus. He was admitted and nasal oxygen was provided bDong-A University Hospital, Neurology, Busan, Republic of Korea with absolute bed rest. His symptom was gradually improved, and he was discharged 3 days after the development of pneumocephalus. He The coexistence of cerebral infarction and subarachnoid hemor- refused an additional CT scan. After 3 months of follow-up, he did rhage (SAH) in patient with reversible cerebral vasoconstriction not complain of any symptoms. syndrome (RCVS) is rarely reported. We report a patient with RCVS Thunderclap headache requires medical attention because it may coexisting convexal SAH and watershed infarction. A-56-year old be a symptom of secondary headache which needs immediate female complained recurrent thunderclap headache, which started treatment. Spontaneous nontraumatic pneumocephalus though rare 14 days ago. Her headache began during swimming and resolved should be considered as a differential. within 20 mins. Next day, headache reemerged during swimming, and resolved within 30 mins. However, her headache persisted doi:10.1016/j.jns.2019.10.1531 without relief since last 10 days. Noncontrast brain CT showed no ARTICLE IN PRESS

382 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx evidence of hemorrhage. However, serial lumbar puncture showed instrumental for converting glutamate to GABA. By preventing pinkish color of cerebrospinal ﬂuid (CSF) with elevated RBC upto conversion to GABA, anti-GADab can accumulate glutamate causing 15520. Brain MRI depicted convexal SAH in left frontal lobe and hyperexcitability of the burst neurons and reverberation in their multiple small watershed cerebral infarction in right hemisphere. reciprocally innervating circuits causing SO. Via reducing the levels Brain angiography showed multiple stenosis of bilateral intracranial of anti-GADab, PLEX reduces hyperglutamatergic hyperexcitable vessels. She was diagnosed with reversible cerebral vasoconstriction state to treat SO; but it cannot restore the function of structurally syndrome complicating SAH and cerebral infarction. We started oral damaged Purkinje neurons – thus the DBN remains unchanged. nimodipine and her headache resolved within 4 days. The cerebral In addition to providing insights into the principles of angiogram performed at 3 months showed complete resolution of restorability of the motor function, our results also provide proof previous stenosis. We described a rare presentation of RCVS of membrane-based model of reciprocally innervating circuit as a complicating SAH and cerebral infarction simultaneously. CSF study cause of SO. could be important diagnostic tool for the evaluation of thunderclap headache. Imaging study using MRI rather than CT could be helpful doi:10.1016/j.jns.2019.10.1534 in these patients. Loss of control for maintaining vascular tone could be the most important cause of RCVS. In this patient, convexal SAH would be supposed to be the early manifestation of small arteriolar involvement and cerebral infarction as the later progression of larger WCN19-0727 artery vasoconstriction. doi:10.1016/j.jns.2019.10.1533 Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ Epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical WCN19-0725 care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells Poster shift 04 - Ataxia and cerebellar disorders/autoimmune and gene therapy - Part II/training in neurology - Part II/child disorders - Part II/CNS infections - Part II/dementia - Part II/ neurology and neuroimaging Epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical Visualization of nerve activities along the brachial plexus after median/ulnar nerve stimulation using magnetoneurography care/neurogenetics/neuroophthalmology/neurophysiology/neu- system rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in neurology - Part II/child T. Watanabeab, S. Kawabatac, Y. Hoshinoc, T. Sasakib, S. Ushiob,M. neurology and neuroimaging Akazad, Y. Miyanoa, I. Ozakie, Y. Adachif, K. Sekiharac, A. Okawab aRicoh Company- Ltd., Healthcare Business Group, Tokyo, Japan Determinants of motor restoration in the syndrome of anti-GAD bTokyo Medical and Dental University, Department of Orthopedic antibody Surgery, Tokyo, Japan cTokyo Medical and Dental University, Department of Advanced F. Wangabc, G. Wilmotd, A.G. Shaikhabc Technology in Medicine, Tokyo, Japan aUniversity Hospitals Cleveland Medical Center, Neurological Institute, dTokyo Medical and Dental University, Respiratory and Nervous System Ceveland, USA Science, Tokyo, Japan bCase Western Reserve University, School of Medicine, Cleveland, USA eAomori University of Health and Welfare, Faculty of Health Sciences, cLouis Stokes Cleveland VA Medical Center, Neurology Service, Cleve- Aomori, Japan land, USA fKanazawa Institute of Technology, Applied Electronics Laboratory, dEmory University, Department of Neurology, Atlanta, USA Ishikawa, Japan

Motor restoration depends on type, location, extent, and Introduction chronicity of the damage. Autoimmune cerebellar motor function Evaluation of detailed lesions of conduction block in brachial is the classic example of chronicity-dependent restoration. Acute plexus is still difficult because of surrounding non-uniform conduc- immune-mediated cerebellar ataxia responds to immune-suppres- tivity. We have developed magnetoneurography (MNG) system and sion therapy, while its chronic counterpart, such as the syndrome reported the visualization of neural activities. In this study, we of anti-glutamic acid decarboxylase antibody (anti-GADab) is present a visualization of electrical activity along the brachial plexus relatively refractory to therapeutic interventions. We suggest that (BP) and consider the clinical application of MNG as a functional chronicity alone is not the only determinant of restoration of imaging method for the BP. cerebellar motor deficit; the disease mechanism also matters. We test this hypothesis in an unusual combination of downbeat Methods nystagmus (DBN) and saccadic oscillations (SO) that were present Neuromagnetic fields over BP of 5 healthy volunteers (Average; in the same patient who had increased titers of anti-GADab. We 32.8 y.o) were measured after median/ulnar nerve stimulation used a high-resolution ocular tracker to quantitatively measure eye (supramaximal stimulus; 5Hz) using our MNG system. Equivalent movements before and after immunotherapy with plasmapheresis currents were computationally reconstructed by a spatial filter (PLEX). The therapy eliminated SO. However, there was no change method and the equivalent currents map was superimposed over in the DBN. The paucity of gamma-aminobutyric acid (GABA) or X-ray images. Waveforms of currents at each virtual electrode set Purkinje neuron loss secondary to increased anti-GADab titers can along conduction pathway were calculated. Compound nerve disinhibit the Purkinje target neurons leading to DBN. GAD is action potentials (CNAPs) was measured at Erb’s point. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 383

Results S. Karmastajia, M. Anwerb, S. Farooqa We successfully visualized neural activities of BP over X-ray aDubai health authority - Rashid Hospital, Neurology, Dubai, United images in all subjects. The equivalent currents distribution showed Arab Emirates axonal activity pattern and passed medially to the coracoid bDubai health authority - Rashid Hospital, Radiology, Dubai, United process, crossed the clavicle, and reached the C5/C6 to C7/T1 or Arab Emirates C6/C7 to T1/T2 intervertebral foramina after median/ulnar nerve stimulation. The peak latencies of the current waveforms at the Moyamoya disease is a fundamental cerebrovascular occlusive virtual electrode closest to Erb’s point and CNAPs showed a high disorder characterised by escalating stenosis of the distal internal linear correlation. carotid arteries, the proximal aspects of the middle cerebral arteries and the anterior cerebral arteries, with subsequent collateral vessel Conclusion formation. This study demonstrated that MNG visualizes neural activity in The ivy sign represents neovascularisation over the cerebral the BP and differentiates the conduction pathway of the median and mantle via leptomeningeal conjugation through collateral circula- ulnar nerve components. MNG could be a novel functional imaging tion, and represents diffuse engorgement of the pia vasculature. modality for brachial plexus pathologies that is easily comprehensi- There are many cases in which this sign is seen in Fluid attenuated ble even for physicians not specialized in neurology. inversion recovery (FLAIR) and post contrast T1 sequence of MRI. However, we report a case in which IVY sign can be more doi:10.1016/j.jns.2019.10.1535 prominently seen in SWI sequence of MRI A 19 yrs old male patient with 3 days history of headache and right visual impairment presented to our department for evaluation. The neurological examination was signiﬁcant for global aphasia and right WCN19-0728 homonymous hemianopia. On (FLAIR) and Diffusion weight images in cranial MRI showed acute infarction in the frontal-parietal region at convexity level consistent with the Middle cerebral artery irrigation Poster shift 04 - Ataxia and cerebellar disorders/autoimmune area and the left temporal occipital lobe consistent with posterior disorders - Part II/CNS infections - Part II/dementia - Part II/ cerebral artery area. Other than acute infarct IVY sign can be seen in Epilepsy - Part II/functional disorders and behavioral neurology/ FLAIR however it is more prominent on SWI. On the DSA examination headache/metabolic and mitochondrial diseases/neuro-critical performed, there was severe stenosis of terminal Internal carotid care/neurogenetics/neuroophthalmology/neurophysiology/neu- artery and its bifurcation with prominent perforators, the basic rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells collateral pathways involved include the basal moyamoya vessels from perforators, dominant left ophthalmic artery with External and gene therapy - Part II/training in neurology - Part II/child carotid artery to Internal carotid artery collaterals. Consequently all neurology and neuroimaging these ﬁndings were suggestive of moyamoya disease.

Is susceptibility weighted imaging more speciﬁc for ivy sign in doi:10.1016/j.jns.2019.10.1536 Moyamoya disease? ARTICLE IN PRESS

384 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx

WCN19-0729 WCN19-0737

Functional network efﬁciency driving the association between Assessment of pituitary dysfunction in patients of idiopathic cerebral small vessel disease and cognitive performance intracranial hypertension with empty sella

H. Chen, Y. Xu N. Prabhat, A. Takkar, V. Lal, A.K. Awasthy, N. Tandyala Drum Tower Hospital, Neurology, Nanjing, China Post Graduate Institute of Medical Education and Research PGIMER- Chandigarh, Neurology, Chandigarh, India Background Cerebral small vessel disease (SVD) is a major cause of cognitive Background decline in the elderly. We aimed to investigate how these changes in Idiopathic Intracranial Hypertension (IIH) has been associated functional network were related to cognitive decline and whether with endocrine disturbances since the condition is found typically in they could be used in discriminant analysis for identifying SVD young, obese women, with menstrual irregularities or taking individuals with cognitive impairment. hormonal preparations. Empty sella is a commonly described imaging sign on MRI Brain in patients of IIH. Whether empty sella Methods has any functional implications in causing pituitary hormonal We recruited 36 SVD with normal cognition (SVD-NC), 38 SVD disturbances needs to be understood. Our objective was to assess with cognitive impairment (SVD-CI) and 38 healthy control (HC) the incidence of pituitary hormonal disturbances in patients of IIH individuals. The functional network was constructed from resting- and to determine any association between empty sella and these state functional magnetic resonance imaging. The measures of hormonal abnormalities. efficiency using graph theory were calculated. Mediation analyses were computed to assess the relationship between SVD burden, Methods network measures and neurocognitive performance. The functional 80 treatment naïve patients of IIH were recruited as per the network measures were subsequently applied to discriminant predefined inclusion criteria. MRI Brain with a detailed sella analysis using a support vector machine (SVM) classification imaging and hormonal profile was done in all patients on day 3 of method. menstrual cycle including s.T3/T4/TSH (thyroid stimulating hormone), s. LH (luteinizing hormone)/FSH (follicular stimulating Results hormone), s. Estrone, s. Prolactin, s. Cortisol (8 a.m.) and s. We found that global and local efficiency of the whole brain were Testosterone in males. significantly decreased in SVD-CI. The subnetwork analysis indicated that SVD-CI showed reduced functional efficiency, particularly in Results fronto-parietal network and cingulo-opercular network. In multiple Partial empty sella was seen in 56/80 patients (70%). Hormonal regressions models, nodal efficiency of prefrontal and parietal abnormalities were detected in 30/80 patients (37.5%), hyp- regions were related to information processing speed function and ocortisolemia in 20%, hyperprolactinemia in 13.8%, hypothyroididsm mediated the associations between periventricular white matter in 3.8%, hypogonadism in 1.25%, elevated levels of gonadotropins hyperintensity and cognition. Using the SVM algorithm, SVD-CI was were found in 5% of cases. On subgroup analysis, hormonal differentiated from SVD-NC using the selected functional efficiency disturbances were found to have a negative correlation with partial measures and the accuracy reached up to approximately 82% with empty sella (p = 0.313). leave-one-out cross-validation during the discrimination process. Conclusion Conclusion Partial empty sella in patients of IIH may just be an anatomical Our findings provide evidence that functional network efficiency correlate with no significant role in causation of pituitary hormonal might mediate the association between SVD and cognitive perfor- disturbances in these patients. mance. The classification results indicate that network efficiency measures might provide an useful tool as disease marker. doi:10.1016/j.jns.2019.10.1538 doi:10.1016/j.jns.2019.10.1537 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 385

WCN19-0747 Keywords: Myotonic dystrophy type 1 and type 2, TCS ﬁndings, The basal ganglia

Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ Epilepsy - Part II/functional disorders and behavioral neurology/ doi:10.1016/j.jns.2019.10.1539 headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and gene therapy - Part II/training in neurology - Part II/child WCN19-0749 neurology and neuroimaging Poster shift 04 - Ataxia and cerebellar disorders/autoimmune Characteristics of transcranial parenchymal sonography and disorders - Part II/CNS infections - Part II/dementia - Part II/ correlation with psychiatric symptomatology in patients with Epilepsy - Part II/functional disorders and behavioral neurology/ myotonic dystrophy type 1 and 2 headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neu- A. Stojanovic, M. Mijajlovic, J. Savkic rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells University of Belgrade School of Medicine, Clinic of Neurology, Belgrade, and gene therapy - Part II/training in neurology - Part II/child Serbia neurology and neuroimaging Introduction Myotonic dystrophy type 1 and 2 (dystrophia myotonica, DM) is Visualization of neural activities in lumbar spine in response to an autosomal dominant genetically determined progressive muscu- the sciatic nerve stimulation by magnetoneurography lar dystrophy associated with multiple organs involvement. Neuro- a b c b b imaging techniques allow visualization and quantification of S. Kimu , S. Kawabata , M. Akaza , K. Sekihara , Y. Hoshino ,T. d ab a a a a pathoanatomic changes, while the parenchymal transcranial sonog- Sasaki , T. Watanabe , Y. Miyano , S. Sato , Y. Mitani , T. Yamaga ,Y. e d raphy (TCS) allows accurate visualization of deep brain structures Adachi , A. Okawa a (basal ganglia, raphe nuclei, and the chamber system). Ricoh Company- Ltd., Healthcare Business Group, Tokyo, Japan bTokyo Medical and Dental University, Department of Advanced The aim Technology in Medicine, Tokyo, Japan c Determining the basal ganglia echogenicity change (substantia Tokyo Medical and Dental University, Respiratory and Nervous System nigra-SN, n. raphe-BR, n. ruber-NR, n. lentiformis-NL) and third Science, Tokyo, Japan d ventricle diameter (DTV), by using TCS in DM1 and DM2 patients Tokyo Medical and Dental University, Department of Orthopedic and correlating the ultrasonography results with the psychiatric Surgery, Tokyo, Japan e clinical manifestation. Kanazawa Institute of Technology, Applied Electronics Laboratory, Kanazawa, Japan Materials and methods TCS and neuropsychological testings (Scales: HAMD, FSS, DSS) Objective were conducted on 163 patients with DM1 and DM2, age 30-62 years. Since the magnetic field is not affected by the surrounding bone and soft tissue, magnetoneurography (MNG) has been attracting attention Results as a new accurate electrophysiological examination of neural activities. Borderline statistical significance (p = 0.05) is determined by We had developed MNG systems using SQUID magnetometers and had comparing the right SN surface in DM1 patients (0.14 ± 0.06 cm2) reported MNG of the cauda equina after the peroneal and tibial nerve and is lower than the right SN in DM2 patients, average size 0.16 ± stimulations. However, the magnetic signal intensity was not enough to 0.07 cm2. BR hypoechogenicity in DM1 patients was more common diagnosing conduction block lesions in patients. In this study, we aimed in older (32–53 years) and in patients with depression (p b 0.05). to obtain strong signals from neural activities in lumbar spine using Enlarged DTV present in DM1 patients correlated with older age (p b sciatic nerve stimulation and examine possibility in MNG. 0.01). Borderline statistical significance was determined by comparing the BR hypoechogenic images of DM1 patients with fatigue (p = Methods 0.05). Daytime sleepiness is more frequent in correlation with Neuromagnetic fields over lumbar area of five healthy volunteers hypoechogenic BR, hyperechogenic SN (p b 0.05) and enlarged DTV were measured with MNG system after the sciatic nerve stimulation in DM2 patients (p b 0.01). at the popliteal fossa. To confirm the supramaximal stimulation was performed during measurements, compound muscle action potential Conclusion of extensor digitorum brevis muscle and abductor hallucis muscle TCS is a practical, easily applicable, highly sensitive neuroimaging were monitored. Current distributions around lumbar spine were method of great importance in diagnosing and treatment planning of calculated using spatial filter methods and superimposed on X-ray patients with DM1 and DM2. images. For comparison, neuromagnetic fields after peroneal ARTICLE IN PRESS

386 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx stimulation at the ﬁbula head and tibial nerve stimulation at the (8 zone), the worm of the cerebellum, the cortex and the precuneus, ankle were also measured. wedge, angular gyrus and posterior divisions of the cingulate gyrus (part of the RSP – the working network of the rest by default).The Results obtained data are the ﬁrst, pilot and require further detailed study. In all subjects, current distributions of neural activities were visualized and propagated in the spinal canal. Peak intensity of doi:10.1016/j.jns.2019.10.1541 calculated currents at L4 spinal canal level was 2.7 times and 9.7 times stronger than that of peroneal and tibial nerve stimulation respectively.

Conclusion WCN19-0946 Using the sciatic nerve stimulation, we obtained sufficiently large signals and detailed current distribution. MNG is expected to contribute to the clinical diagnosis and treatment of lumbar spinal disease. Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ doi:10.1016/j.jns.2019.10.1540 Epilepsy - Part II/functional disorders and behavioral neurology/ headache/metabolic and mitochondrial diseases/neuro-critical care/neurogenetics/neuroophthalmology/neurophysiology/neurorehabilitation/pain - Part II/sleep disorders - Part II/stem cells WCN19-0755 and gene therapy - Part II/training in neurology - Part II/child neurology and neuroimaging Poster shift 04 - Ataxia and cerebellar disorders/autoimmune disorders - Part II/CNS infections - Part II/dementia - Part II/ Effect of Fasudil on the synaptic protection of SH-SY5Y cells Epilepsy - Part II/functional disorders and behavioral neurology/ synapse injured by oxygen-glucose deprivation headache/metabolic and mitochondrial diseases/neuro-critical a a a a a a b care/neurogenetics/neuroophthalmology/neurophysiology/neu- W. Zhang , H.F. Zhang ,J.Ma, J. Zhao , Z.X. Zhou , M. Liu , Z. Chai ,Q. Wangb, J.Y. Chenga, L. Fenga, J.J. Hangc, Z.Q. Yuand, J.Z. Yue, B.G. Xiaof, rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells C.G. Mab and gene therapy - Part II/training in neurology - Part II/child aThe NO.1 Affiliated hospital of Shanxi Datong University, Institute of neurology and neuroimaging Brain Science- Shanxi Key Laboratory of Inflammatory Neurodegener- ative Diseases, Datong, China Brain functional connectivity in severe asymptomatic carotid bShanxi University of Chinese Medicine, The Key Research Laboratory of stenosis patients on the background of leeching course Benefiting Qi for Acting Blood Circulation Method to Treat Multiple Sclerosis of State Administration of Traditional Chinese Medicine- and M. Pospelovaa, T. Aleкseevab,A.Efimtsevc, N. Ivanovab, G. Trufanovc Scientific and Technological Innovation Team of Integrated Chinese and aAlmazov National Medical Research Centre, Neurology and Neuro- Western M, Jingzhong, China rehabilitation, St Petersburg, Russia cGeneral Hospital of Datong Coalmine Group, Neurosurgery, Datong, bAlmazov National Medical Research Centre, Neurology and Psychiatry, China St Petersburg, Russia dAcademy of Military Medical Sciences, Military Cognition and Brain cAlmazov National Medical Research Centre, Radiology, St Petersburg, Science Center, Beijing, China Russia eShanxi Datong University, Institute of Brain Science- Shanxi Key Laboratory of Inflammatory Neurodegenerative Diseases, Datong, China f Study the influence of the leeching course on brain functional Fudan University, Institute of Neurology- Huashan Hospital- Institutes connectivity in 10 patients (8 women and 2 man, average age 69 ± 5.4 of Brain Science and State Key Laboratory of Medical Neurobiology, years) with asymptomatic atherosclerotic one or both ICA stenosis in Shanghai, China the range of 65–75%. Magnetic resonance imaging (MRI), functional MRI at rest fMRI in 2 time points: before and after leeching course, Background which consisted of 10 sessions. Statistical processing and evaluation of Cerebral infarction (CI) is caused by occlusion of cerebral blood the results of neuroimaging studies of each patient and their group vessels, resulting in the neurological defects. Rho kinase (ROCK) (rest fMRI data) were carried out using the CONN v software inhibitors Fasudil has potential neuroprotective effects in CI. package.18, designed to determine the relationships between different parts of the brain, the structure of different rest networks and Objective functional networks of the brain. After a course of treatment (2-2.5 To observe the effect of Fasudil on change of synapse regenera- months), was identified the strengthening of positive functional tion and repair of SH-SY5Y cells injured by oxygen-glucose connections of the medial prefrontal cortex with the 10 zone of the deprivation (OGD). cerebellum (right hemisphere), the cerebellum worm. The strengthening of negative functional connections of the medial prefrontal Methods cortex with the left middle frontal gyrus and the right par- SH-SY5Y cells were divided into control group, oxygen-glucose ahyppocampal gyrus was determined. Selecting as the region of deprivation group (OGD group) and OGD with Fasudil group (OGD+ interest cerebellar rear of the working of the network is determined by Fasudil group). The morphology of synaptic injure and repair was the gain (including local) positive functional connections after observed by phase contrast microscope, and the expressions of hirudotherapy course with right and left hemisphere of the cerebellum ROCKII, phosphorylated myosin phosphatase 1(p-MYPT1), ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 387 postsynaptic density (PSD-95) and synaptophysin were evaluated by A. Mimenzaa, S. Aguilar Navarroa, L. Bracho Velaa, A. Zamudio Cruza, western blot assay. F. Hernandez Contrerasa, G. Jimeneza, J.A. Avila Funesb aInstituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Results Geriatric Neurology, México, Mexico The cell body and synapse of OGD group became smaller, bInstituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, synapses were retracted and broken, but control groups and OGD+ Geriatric, México, Mexico Fasudil group were similar. MTT of OGD group increased (p b 0.01), OGD+ Fasudil group increased (p b 0.05), but the cell viability of Background OGD+ Fasudil group decreased(p b 0.05), when compared with the The prevalence of Vit-D deficiency in older adults with cognitive OGD group. The expression level of ROCKIIand p-MYPT1 was impairment is approximately 70% to 90%. significantly higher in the OGD group (p b 0.05). The expression of Synaptophysin and PSD-95 was significantly inhibited in SH-SY5Y Primary objective cells injured by oxygen-glucose deprivation, but Fasudil could To determine the relationship between Vitamin D deficiency with effectively up regulate the expression of Synaptophysin and PSD-95 mild cognitive impairment and dementia in patients over 65 years of in OGD damaged SH-SY5Y cells. age. Secondary Objectives. Describe the clinical and sociodemographic characteristics of patients with vitamin D Conclusion alterations Fasudil protects and repairs neurons by inhibiting the expression of ROCK, p-MYPT1, and enhancing the expression of PSD-95 and Syn Method in OGD damaged SH-SY5Y cells. (NNSF of China 81471412, Cross-sectional study was carried out in the cognition clinic of the 81473577, Shanxi Science and Technology Key R&D Program Institute of Medical Sciences and Nutrition Salvador Zubirán. Patients 2016ZD0505, Shanxi Provincial Platform Base Special with a diagnosis of Mild Cognitive Impairment, dementia and Project201805D111009,201805D131005). patients without cognitive impairment (controls) were included. They will have a determination of 25-Hydroxy vitamin D. Three doi:10.1016/j.jns.2019.10.1542 categories were formed according to their level:N20 ng/ml normal, 12–20 ng/ml mild deficiency and b12 ng/ml severe deficiency.

Results WCN19-0786 200 patients were evaluated, of which 31 belonged to the control group, 85 to the group with MCI and 84 to the dementia group. In the three groups were more women than man, in the control group Poster shift 04 - Ataxia and cerebellar disorders/autoimmune showed statistically significant difference in mean age (p b 0.01), disorders - Part II/CNS infections - Part II/dementia - Part II/ year of education (p = 0.04) and functionality (p b 0.01). In the Epilepsy - Part II/functional disorders and behavioral neurology/ group of severe deficiency there was worse performance, in the headache/metabolic and mitochondrial diseases/neuro-critical executive functions and the evocation. The correlation for executive care/neurogenetics/neuroophthalmology/neurophysiology/neu- functions was 0.250, p = b0.001, Pearson's correlation for vitamin D b rorehabilitation/pain - Part II/sleep disorders - Part II/stem cells and total Mini-mental score was 0.203, p = 0.005 and gene therapy - Part II/training in neurology - Part II/child Conclusion neurology and neuroimaging There is a relationship between vitamin D deficiency and global cognitive functioning. Likewise, severe deficiency affects specific Relationship between the levels of vitamin D in elderly adults domains of memory (evocation and executive functions). with mild cognitive impairment and dementia in a tertiary care hospital doi:10.1016/j.jns.2019.10.1543