Article in Press

ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 119

WCN19-1291 WCN19-1293

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other outcomes/neuroepidemiology and environmental and other

Different outcome from different approach of therapy by Comparative assesment of prognosis and frequency of acute plasmapheresis and immunotherapy in GBS patients inﬂammatory demyelinating polyneuropathy in Turkish and Syrian patients between 2012 AND 2018 A. Wirathmawati, B. Munir, S. Nandar Faculty of Medicine, Brawijaya University, Neurology Department, E.E. Okuyucu, D. Selcuk Malang, Indonesia Mustafa Kemal University Hospital, Neurology, Hatay, Turkey

Objective Purpose To present different outcome of Guillain Barre Syndrome therapy To compare the frequencies and prognoses of the Turkish and between plasmapheresis and immunotherapy. Syrian patients diagnosed with, and treated for acute inflammatory demyelinating polyneuropathy (AIDP). Background Plasmapheresis and IVIG Therapy are standard therapy for GBS, Method but in Indonesia there is not any recorded data to show effectivity of The whole data of 40 patients with AIDP, 20 Turkish and 20 both plasmapheresis and IVIG. Syrian, who have been hospitalized between 2012–2018 in the Neurology Clinic of Mustafa Kemal University Medical Faculty, have Methods been evaluated retrospectively, and analyzed. Prospective serial case report of 50 GBS patient in Saiful Anwar General Hospital from January 2018 to January 2019, with EMG and Findings LCS analysis as tools to establish the GBS diagnose and GBS disability The average age were 43,65 ± 13,95. In 60% of the patients, upper score parameter for asses the improvement of therapy. Statistical respiratory infection has been detected as most common factor. The method using t paired test. admission time to hospital for Syrian patients was 11,3 ± 5,7 days, it was 6,5 ±4,0 days for the Turkish patients, and the difference was Results significant. The hospitalization and discharge MRC scores in Turkish 50 consecutive patients were included in the study and consist of patients were higher and the difference was statistically significant 32 men and 18 women. 1 patient was died at ER. Mean age was 40.5 (p b 0,001). While the Syrian patients with 3 or below AIDP disability years, mean of hospitalization was 15 days. Types of GBS were divide (Hughes) score was 30%, it was 100% in Turkish patients (p b 0,001). in 3 type; AMAN in 36%, AMSAN in 46% and SMF in 14%. Plasmapheresis as therapy was in 79% of patient, immunotherapy Result was 12% and the rest patient was refused to therapy and got Hyponatremia, low hospitalization MRC score, late admission to supportive therapy. Disability score were assessed at admission, hospital are determined as bad prognostic factors. In Syrian patients, discharge and 6 months follow up. Mean disability score from GBS hyponatremia was seen more respect to Turkish patients. The onset in hospital admission was 4.1. Plasmapheresis shown 60% hospitalization-discharge MRC scores of Syrian patients were lower, better outcome in our patients but IVIG only shown approximately and Hughes scores were higher respect to Turkish patients. In Syrian 30%. patients the need of intensive care and sequel ratio was higher, while the response to treatment was higher in Turkish patients. In Syrian Conclusion patients, AIDP progresses with worse prognosis than Turkish Plasmapheresis shown better outcome than IVIG in GBS patients, and their response to treatment is worse. We hypothesize treatment. that AIDP can be seen easier due to the suppression of the immunological picture during and after the war. Keyword: Guillain Barre Syndrome, Disability score, Plasmapheresis, Intravenous immunoglobulin doi:10.1016/j.jns.2019.10.992

References

[1] John R. Pikula, Guillan Barre Syndrome: a case report, J. Can. WCN19-1294 Chiropr. Assoc. 39 (2) (1995 Jun) 80–83. [2] Hugh J. Wilson, Bart C. Jacobs, Pieter A. Van Doom, Guillain Barrre Syndrome, Lancet 388 (2016) 717–727. Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.991 Epidemiological watch on epilepsy at the research and mental health laboratory (LNSM) Antananarivo, Madagascar: A 2 years study ARTICLE IN PRESS

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

F. Ravelosaona, R.S. Razafindrasata, N.F. Rasaholiarison, J. cUniversity of Bari Aldo Moro, Basic Medicine- Neuroscience- and Sense Razafimahefa, A.D. Tehindrazanarivelo Organs, Bari, Italy HU Befelatanana, Neurology, Antananarivo, Madagascar dPia Fondazione Cardinale G. Panico, Department of ClinicalResearch in Neurology, Tricase LE, Italy Introduction Epilepsy is the most frequently encountered neurological pathol- Background ogy in the world. In sub-Saharan Africa the prevalence varies We report a 82-year-old woman, who gradually started to between 2.2 and 58/1000 with an average of 15.83. present in 2015 cognitive impairment (forgetfulness, impaired attention) with fluctuating course and behavioural symptoms Objective (auditory and visual hallucinations, apathy). Since Spring 2016, To determine the epidemio-clinical profile of epilepsy in a reference progressive slowness of movements was observed. Since Autumn center that performs a profound clinical and paraclinic analysis such as 2016, she felt that another person was inside her and was telling the LNSM and comparing them to data from non-specificcenter. her what to do. Neurological examination showed ipomimia, akinesia and rigidity in all limbs, postural abnormalities, difficulties Methods in gait. A retrospective descriptive study was conducted between January 2013 and December 2014. Patient records for EEG with epilepsy Methods diagnosis and other sites : data from statistical directories of non- The patient underwent an extensive neuropsychological assess- specific centers in all regions of Madagascar, with direct standard- ment, MRI, Single Photon Emission Tomography (SPET) with ization of rates of prevalence and specific incidence of age of 2 dopamine transporter (DAT) scan. populations by a reference population. Results Results In July 2016, MRI scan showed cortical and subcortical atrophy Crude epilepsy prevalence rate: 572%o and 698%o/year in 2013 and small areas of increased signal intensity in centrum semiovalis of and 2014 seen at the LNSM. 15%o to non-specific center. Gross both cerebral hemispheres on FLAIR sequences. In September 2016, incidence in 2013 and 2014: 49988/100000 people/year and 50775/ neuropsychological testing documented cognitive impairment, 100,000 persons/year. Respectively 38/100000 persons/year and 88/ mainly characterised by deficits of executive functions and episodic 100000 persons/year at the level of non-specific center with the memory. In June 2017, about 2 years after disease onset, DAT-scan highest specific incidence in the 5 to 14 year age group diagnostic. did not show decreased DAT striatal uptake. So far, the patient The symptomatic form predominated. refused a repeat DAT-scan SPECT.

Discussion Discussion This result seen at LNSM are largely high compared to those of According to current diagnostic criteria, a clinical diagnosis of the non-specific center issued by the statistical directories and this probable Dementia with Lewy bodies can be made in this patient. difference persists despite the standardization with respect to age The absence of abnormalities on DAT-scan, reported in a low with change in the peak incidence of Epilepsy. Explained by the fact percentage of patients with neuropathologically confirmed diagnosis that in specific center like the LNSM the diagnosis of epilepsy is of DLB, may arise from a less marked neuronal loss in the substantia electroclinical and more thorough. nigra in such patients, who may show abnormal DAT-scan on follow- up. Conclusion Our study confirms the importance of a specific diagnostic center Conclusions to better handle epilepsy. This case supports the view that DLB in early stages may show different patterns of presentation from clinical and neuropatholog- doi:10.1016/j.jns.2019.10.993 ical viewpoints.

doi:10.1016/j.jns.2019.10.994

WCN19-1299

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - WCN19-1306 Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and Multiple personality and hallucinations in a patient with normal outcomes / neuroepidemiology and environmental and other dat-scan spect fulﬁlling diagnostic criteria for probable dementia with lewy bodies Comparative analysis of the psychoemotional and cognitive spectrum in various forms of parkinson's disease A. Danielea, G. Lacidognaa, C. Emanuelea, M. Orsinia, D. Seripab,G. Paronib, G. Logroscinoc, F. Panzad S. Djalilovaa, Z. Ibodullayevb, D. Abdullayevab aCatholic University of Sacred Hearth, Neurology, Rome, Italy aAssistent, Neurological diseases, Tashkent, Uzbekistan bFondazione IRCCS Casa Sollievo della Sofferenza, Complex Unit of bTashkent Medical Academy, Neurological diseases, Tashkent, Geriatrics, Department of Medical Sciences, San Giovanni Rotondo FG, Italy Uzbekistan ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 121

Purpose Computed tomography scan of the whole abdomen revealed a large To study the psychoemotional and cognitive spectrum in various retroperitoneal mass, suggestive of a large Liposarcoma. She under- forms of Parkinson's disease went an exploratory laparotomy, adhesiolysis, and radical excision of the retroperitoneal mass. Histopathologic gross examination of the Materials and methods specimen revealed that the mass weighed 800 grams, and measured 12 patients with Parkinson's disease 2, 3 degrees of severity were 441 X 331 X 120 cm. Microscopic examination showed a neoplasm included in the study. The average age varied within 47 ± 6.5. Of consisting of lobules of adipose cells, which were traversed by these, 8 women, 4 men. All patients were divided into 3 groups fibrocollagenous septae of varied thickness consistent with a well depending on the form of the disease. The first group included 4 differentiated Liposarcoma. patients with a tremor form of the PD; the second group - 3 patients Three years after surgey, she noted progression of bilateral ptosis with akinetic-rigit form; the third group consisted of 5 patients with now associated with facial weakness and generalized muscle fatigue mixed form of PD. The psycho-emotional spectrum was examined by that markedly improved with rest. She was admitted at a local Hospital Anxiety Depression Scale, the cognitive spectrum was hospital where Paraneoplastic Myasthenia Gravis with a Liposarcoma studied by neuropsychological study of Luria. was considered. Cranial and Chest CT scans were unremarkable. Pyridostigmine test was positive with improvement of ptosis, Results diplopia, and muscle weakness. Anti-acetylcholine receptor radioim- Considering the data of the HADS test, clinically significant munoassay was positive for AchR antibodies. She was treated with anxiety was observed in all 4 patients with a trembling form intravenous immunoglobulin (IVIG) and was discharged with (average score18 ± 5.7) and in 3 patients with a mixed form, but Pyridostigmine (Mestinon) as maintenance. with a lower intensity (average score14 ± 2.5). In comparison with the akinetic-rigit form, where all patients had a clinically pronounced doi:10.1016/j.jns.2019.10.996 depression (average score13 ± 3,7).P≤0,05 In the neuropsychological study, disturbances in concentration of attention, thinking, with less significant decrease in memory and frontal dysfunction were noted in patients with a trembling form (on WCN19-1319 average - 25.3 + 0.2 points); however, significant frontal dysfunctions were observed in patients with a mixed form. P≤0,05 Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Conclusion Part I / dementia - Part I /epilepsy - Part I / health economics and These results provide an opportunity for early prevention of outcomes / neuroepidemiology and environmental and other anxiety-depressive disorders and cognitive disorders, taking into account the form of PD. Retinal versus optic nerve predictors of early disability in ambulatory patients with multiple sclerosis, a comparative study doi:10.1016/j.jns.2019.10.995 M. Hamdoon Sohag University Hospital, Neurology, Sohag, Egypt

WCN19-1314 Background Multiple sclerosis (MS) is a common cause of neurological Poster shift 02 - Autoimmune disorders - Part I /CNS infections - disabilities in adults and commonly affects the visual pathway. Part I / dementia - Part I /epilepsy - Part I / health economics and Objective outcomes / neuroepidemiology and environmental and other To assess and compare the sensitivity of Visual Evoked Potentials (VEP) and Optical Coherence Tomography (OCT) as early correlates Paraneoplastic myasthenia gravis in a 62 year old Filipino woman of early disability in ambulatory patients with MS.METHODS: Forty- with a well-differentiated retroperitoneal liposarcoma: A case Four patients with MS with Expanded Disability Status Scale (EDSS) report of ≤ 4.5 (ambulatory patients) and 14 healthy controls participated in this study. Patients were classified into 3 groups according EDSS. a b R.J. Romero , J.A. Vatanagul Patients with EDSS of 0-1.5 formed the “No disability”, patients with a Perpetual Succour Hospital, Internal Medicine, Cebu, Philippines EDSS of 2-3 formed the “Minimal to mild disability”, and finally b Perpetual Succour Hospital, Internal Medicine- Neurology, Cebu, patients with EDSS of 3.5-4.5 formed the “Moderate to significant Philippines disability” groups. N75/P100 amplitude, P100 latency, Retinal Nerve Fiber Layer (RNFL) thickness, and Ganglion Cell Layer Complex V.G., is a 62 year old woman, housewife, known hypertensive and (GCLC) thickness were measured. asthmatic, from Cebu City admitted due to ptosis and an enlarging abdominal mass. Results Two years PTA, patient had onset of bilateral ptosis associated Patients showed significantly lower N75/P100 amplitude, higher with occasional diplopia. She just tolerated the condition and no P100 latency, lower RNFL, and GCLC thicknesses compared to controls. consult was done. However, there were non-significant changes in P100 latency, N75/ Eighteen months PTA, she noted a soft, palpable, non tender, P100 amplitude, and GCLC thickness among the 3 groups for both round mass on the right lower abdominal area. There were no patients with and without history of Optic Neuritis (ON). On contrary, associated changes in bowel and bladder pattern or weight loss. RNFL thickness was significantly different between the three groups Two weeks PTA, abdominal girth increased causing occasional for both patients with and without ON. Factorial ANOVA revealed non- exertional dyspnea. She sought consult with a gastroenterologist. significant disability x History of ON interaction. ARTICLE IN PRESS

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

Conclusions WCN19-1342 Compared to VEP parameters, RNFL thickness was a sensitive correlate with the degree of early disability in fully ambulatory patients with MS with or without history of ON. Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and doi:10.1016/j.jns.2019.10.997 outcomes / neuroepidemiology and environmental and other

Possible role of ghrelin in neuronal conduction in improved memory cognition WCN19-1338 V. kakoty, R. Kumar, R. Goyal Shoolini University- Solan H.P-173212- India, Department of Neurology, Poster shift 02 - Autoimmune disorders - Part I /CNS infections - School of Pharmaceutical Sciences, Solan, India Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other Background Ghrelin, an appetite stimulator, functions through IP3-DAG Preliminary results of cross – Sectional research for moderate signaling. It has been found to increase food related memory cognitive impairments among persons over 60 years in the retention in rodents. Kazakh population Objective S. Turuspekovaa, B. Demesinovaa, E. Nurgujaeva, E. Atantaevaa,R. The study aims to investigate the role of Ghrelin signaling in Tsoya, L. Bespalovab memory cognition and retention using intracerebroventricular aKazakh National Medical University, Department of Nervous Diseases streptozotocin (ICV-STZ) model in Wistar rats. with a Course of Neurosurgery, Almaty, Kazakhstan bKazakh National Medical University, Department of Psychiatry and Methods Narcology, Almaty, Kazakhstan Male Wistar rats (250-280 g) were employed into the study with sample size n=6. Streptozotocin (STZ) 3 mg/kg was administered on Today, about two million elderly people live in Kazakhstan, which day one through intra-cerebroventricular route. n-Octanoic acid, a is more than 10% of the population, thus crossing the seven percent Ghrelin activator was given in two doses 50 and 100uL for 21 daysby ++ threshold for the definition of an “aging” country in the world. Data dissolving in polyethylene glycol 600whereas Nifedipine, Ca on the prevalence of mild cognitive impairment (MCI) and dementia channel blocker was given at 10 mg/kg, i.p. to one group underwent are absent in Central Asia, particularly in Kazakhstan. The Global to administration of n-octanoic acid high dose.Impairments in prevalence pattern of dementia depends on several factors, from life cognition, memory consolidation and retention were assessed using fi expectancy to the health status, last but not least from the particular Morris water maze, Y maze, balance beam, open eld test and environment. It is very difficult to determine a particular factor for it. photoactometer test. The biochemical estimations for oxidative stress i.e. lipid peroxidation, glutathione, and acetylcholinesterase Purpose activity were done in rat brain homogenate. Statistical analysis was To study the prevalence of cognitive impairment in persons of the carried out using graph pad prism 5. Kazakh population over 60 years old in Almaty Results fi Materials and methods ICV-STZ treated animals exhibited memory de cits in Morris 150 respondents aged from 60 to 84 years (Mean age 67.6) took water maze, Y maze, balance beam test. Administration of fi part in the screening. The material was collected using the Champ low and high doses of n-Octanoic acid produced signi cant Clinic Questionnaire Questionnaire and the MOCA test (cut point restoration of memory retention. However, nifedipine abolished b26) on the basis of the Almaty city polyclinic. the memory improvement produced by n-octanoic acid. The level of oxidative stress and AChE activity observed in rat brain was Results also reversed. Mild cognitive impairment was detected - 39.9%, light cognitive impairment - 33,4%, normal cognitive function - 26.7%. Expected risk Conclusion fi factors for MCI: arterial hypertension – 86.89%, Cardiac ischemia, The nding may reveal that ghrelin plays pivotal role in angina pectoris – 70.21%, pathology of the thyroid gland and pancreas improving cognition, retentionand working memory possible (Diabetes, hyperthyroidism and hypothyroidism) – 29.50%, atheroscle- through Ca signaling. rotic vascular disease – 29.9%, brain trauma – 18.22%, level of education – 4.24%, depression – 0.6%, respectively. Keywords: Alzheimer’s disease, Ghrelin, Streptozotocin, n-Octanoic acid (OA), Amyloid beta, Nifedipine Conclusions Kazakhstan is a middle-income country with a growing tendency of an aging population, and we have an urgent need to continue doi:10.1016/j.jns.2019.10.999 screening the population for the identification of moderate cognitive impairment in individuals in the Kazakh population. doi:10.1016/j.jns.2019.10.998 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 123

WCN19-1350

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other

Mitoptosis is a non-return point in formation dark degenerative neurons during acute experimental endotoxemia

E. Gasimov Azerbaijan Medical University, Histology- Embryology and Cytology, Baku, Azerbaijan

Background Recent studies have described the signiﬁcant role of inﬂammation in progressive nature of neurodegeneration. However, there is no unanimous opinion on the mechanism and forms of ultrastructural changes in the neurons of the cerebral cortex.

Methods Acute experimental endotoxemia was induced by intravenous injection of puriﬁed LPS from E. coli (serotype 0111:B4) at a dose of 1,0 mg/kg dissolved in saline (0,5 ml). Under deep anesthesia the cerebral cortex was removed and then processed using standard EM. Ultra thin sections from the cerebral cortex were examined by using TEM.

Results In the state of endotoxemia, in one visual ﬁeld of the cerebral cortex of the white rat, dark deformed neurons are seen in various stages of degeneration along with bright neurons. In the initial stage of formation of dark neurons, along with strong dehydration,

destruction of the nuclear pore complex, expansion of the perinuclear spaces and the lumen of cisterns of the endoplasmic reticulum are observed. Between the latter there are numerous free ribosomes and mitochondria without visible ultrastructural changes (Fig. 1A). Later, if the outer membrane is preserved, the lysis of the mitochondrial cristae occurs (Fig. 1B). From this moment, except some secondary lysosomes and residues of condensed nucleoli, other organelles and cytoskeleton elements are not detected in the cytoplasm of dark neurons (Fig. 1C).

Conclusions Internal mitoptosis - starting with the destruction of the integrity of the mitochondrial cristae is the point of no return during the formation of degenerative dark neurons without ultrastructural signs of apoptosis.

doi:10.1016/j.jns.2019.10.1000 ARTICLE IN PRESS

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

WCN19-1352 A. Subir, F. Ghafoor, K. Nc, M. Rafeeque M.E.S Medical College, Neurology, Perinthalmanna, India

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Aim Part I / dementia - Part I /epilepsy - Part I / health economics and To present an interesting case of isolated INTRACRANIAL ROSAI outcomes / neuroepidemiology and environmental and other DORFMAN disease with headache,vision loss and pachymeningitis

Progressive multifocal leukoencephalopathy (PML) presenting as Method focal seizures in a ﬁlipino male with aids a case report Case report with photos of MRI picture of Half of “Eiffel in night “ appearance, Optic fundus photo, tissue histopathology showing A. Conopio, J. Vatanagul emperipolesis and post treatment imaging pictures(resolution of Perpetual Succour Hospital, Internal Medicine, Cebu, Philippines enhancement).

Case Case A 38-year old male, health care worker presented one month 56 year old blind female with 4 year prior history of insidious PTA with myoclonic jerks of the left upper extremity lasting onset progressively worsening diplopia leading to vision loss and approximately one minute associated with left hemiparesis. He associated severe headaches. was initially managed as cerebrovascular disease (CVD) infarct at a She was extensively worked up at different institutes for 4 local hospital. Brain plain CT scan and MRI were both unremark- years with prior MRI brain showing pachymeningeal thickening able. Patient was discharged with noted improvement of his motor thought to be due to a post lumbar puncture change and had strength. negative vasculitic and auto immune work up. On examination, A week after he developed intermittent fever and recurrence of she was blind with severe olfactory dysfunction. Her new MRI myoclonic seizures. MRI of the brain plain and contrast showed a showed a pachymeningeal thickening -half of “Eiffel in night” non-specific and non - enhancing T2W and FLAIR hyperintensities in appearance. the right and left parietal white matter due to small vessel ischemic Her work up for pachymeningitis was negative. A meningeal change or foci of demyelination and temporal lobe volume loss biopsy was done. Initially misdiagnosed as IgG4 disease and later (Fig. 1). Rosai Dorfman disease confirmed with Immuno histochemistry His social history revealed MSM (male having sex with male). HIV being positive for CD 68,S100. Thus the final diagnosis was ELISA was positive and CD4 T-cell count was of 22 cells/ul. Blood isolated INTRACRANIAL ROSAI DORFMAN DISEASE without any Cultures, serum electrolytes and Chest X-ray taken were all other systemic manifestations or lymphadenopathy. She was unremarkable. Patient was started on combination antiretroviral started on Methotrexate and steroids following which her drug therapy, Azithromycin and Cotrimoxazole and Levetiracetam headache and pachymeningitis subsided but vision loss persisted. and discharged. He developed recurrent fever, headache, left sided hemiparesis, Conclusion blurring of vision, focal seizures and progressive deterioration of Although rare, intracranial Rosai Dorfman disease should be sensorium and was readmitted. Empiric Ceftriaxone, IV Valproic considered in the differentials for pachymeningitis and to Acid and Levetiracetam were started.CSF samples were sent for be carefully distinguished from IgG4 related diseases by immuno- cultures and film array ®assay for meningitis and encephalitis, histochemistry. An earlier diagnosis and treatment could have India ink and TB gene Expert were all negative (Table 1). CSF averted vision loss due to optochiasmatic involvement as in our Polymerase Chain Reaction (PCR) for JC virus was positive. He case. deteriorated, became comatose and eventually succumbed on the 3rdweek of admission. doi:10.1016/j.jns.2019.10.1001 doi:10.1016/j.jns.2019.10.1002

WCN19-1354 WCN19-1374

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other outcomes / neuroepidemiology and environmental and other

An interesting case of intracranial rosai dorfman disease mim- A case report: Medical management of brain abscess from a self- icking as IgG4 related pachymeningitis extracted impaled nail ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 125

D.K. Ferrer disorders in the main group have changed dramatically 7.5 times. In Quirino Memorial Medical Center, Department of Adult Neurology, patients with psychopharmacotherapy in the 2A group psy- Center for Neurological Sciences, Quezon City, Philippines choemotional condition was improved 3.5 times. Compare with 2B group - only 1.5 times. Management of nail impalement to the brain is usually done in a hospital setting to prevent further injury to the brain. This aims to Conclusions report a case of a 41-year-old male who was accidentally hit on his According the study anxiety-depressive syndrome are wide- left fronto-parietal area by a falling wooden plank with a protruding spread among patients with the systemic lupus erythematosus, three-inch nail. He pulled out the nail by himself. Few days later, he which need complex treatment with Psychotherapy and presented with focal motor seizures, dysphasia, right-sided weak- psychopharmacotherapy. ness and numbness and was admitted. Cranial MRI revealed a ring- like contrast enhancing lesion in the Left mid-posterior frontal cortex doi:10.1016/j.jns.2019.10.1004 and subcortex containing air and metallic artefact which may represent a hematoma or an abscess. The patient was managed as a case of cerebral abscess secondary to a penetrating injury and was given with IV antibiotics alone. Rehabilitation therapy was instituted WCN19-1381 and was discharged with minimal deﬁcit. This case report describes the management done in a case wherein the eloquent area was hit Poster shift 02 - Autoimmune disorders - Part I /CNS infections - and the patient has recovered with minimal deﬁcit despite injudicious self-removal of a penetrating object. Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1003 Cognitive disorders and their rehabilitation in parkinson’s diseases

WCN19-1377 N. Maxamatjanova Assistent, Neurological diseases, Tashkent, Uzbekistan

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Purpose Part I / dementia - Part I /epilepsy - Part I / health economics and Early identiﬁcation and eradication of cognitive impairment outcomes / neuroepidemiology and environmental and other observed in Parkinson’s diseases.

Evaluation of the effectiveness of psychopharmacotherapy and Material and methods psychotherapy in the complex treatment of systemic lupus The study involved 27 patients with Parkinson’s diseases at 1-2 erythematosus degree with cognition disorders, 9 of them women and 18 men. The average age of patients 62 ± 7.5 years. The patients were divided N. Maxamatjanova into two groups: Main group: 15 patients who received Assistent, Neurological diseases, Tashkent, Uzbekistan асatinolmemantine with Various gymnastic exercises with neuropsychological trainings to stimulate cognitive functions; the control Purpose group of 12 patients who received only aсatinolmemantine. MoCA To evaluate the effectiveness of psychotherapy(PT) and psycho- scale was used to determine deeper cognitive impairments. For the pharmacotherapy(PPhT) in the complex treatment of systemic lupus purpose of neuropsychological training to improvement abstraction erythematosus and descanting we assigned modeling of plasticine, classiﬁcation of objects; for memory and language stimulation - autobiogrophic Materials and methods diary, make a book; to stimulate tactile gnosis - guess what is in the The study was performed in 48 patients. Of these, 44 (91.7%) bag. Together with these trainings we used gymnastic exercises (for women and 4 males (8.3%). The average age of the subjects was 32 ± hand, foot, head) to maximize stimulation of both sides of brain. 8.2 years. Patients were divided into 2 groups: 1) Main group - 25 The results shown, that different levels of cognitive disorders has (52%) patients which treated with PPhT and PT; 2) Control group been identiﬁed in both groups. According MоCA scale in 88% of which separated into two group: A - 12 (25%) patients which cured patients in both groups attention and memory disorders, 75% of with PPhT and basis therapy; B – 11 (23%) were treatment limited by speech abnormalities, 85% of abstract thinking disorders, and 35% of basic therapy. For evaluation psycho-emotional status, anxiety, and gnosis disorders. depression disorders we used Hospital Anxiety and Depression Scale After the Various gymnastic exercises with neuropsychological (HADS). trainings data of MoCA scale were as follows: cognitive functions in main group improved by 2 times in compare with control group (p Results b0.01). According to the HADS scale, clinical manifestations of anxiety- depressive syndrome (16 ± 5 points) in main group were at 21 Conclusion patients (84%), at 8 (66.7%) in group 2A and at 7 (63.6%) in group 2B. The treatment of cognitive impairments in Parkinson's disease, Clinically expressed anxiety (16 ± 4.7 points) and subclinical combined with neuropsychological training and various exercises, depression (9 ± 1.1 points) in main group at 3 (12%), at 3 (25%) in improves the quality of life of patients and prevents early disability 2A and 2B 27.3% were observed.[r b0,03].After treatment, data were analyzing in 2 months by HADS, showed that psychoemotional doi:10.1016/j.jns.2019.10.1005 ARTICLE IN PRESS

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

WCN19-1384 with long standing cerebellar syndrome and sensory neuropathy, presenting as status epilepticus where chronic lead toxicity was conﬁrmed to be the cause.

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Case report Part I / dementia - Part I /epilepsy - Part I / health economics and A 35 year male presented with status epilepticus. Over a period of 7 outcomes / neuroepidemiology and environmental and other years he suffered from cerebellar ataxia and sensory neuropathy. He was also hypertensive. MRI brain showed bilateral cerebellar and thalamic ﬁ Chronic lead encephalopathy presenting as status epilepticus hyperintensities (Fig 1). Latest MRI also shows signi cant cerebellar atrophy. CSF showed albumin-cytological dissociation (ﬁg2). The serum lead level was 33 micromol/litre (Normal less than 5). No history of R. Sudath, I. Rajapakse, M. Aravinthan, B. Senanyake direct ‘occupational’ exposure noted. He lived adjacent to a main road National Hospital of Sri Lanka, Institute of Neurology, Colombo, Sri within the central city limits. Given the clinical presentation and Lanka biochemistry chelation with peniclillamine was initiated. Key words: Lead toxicity, Cerebellar syndrome, Status epilepticus

Discussion Introduction Chronic lead toxicity causes encephalopathy and sensory predom- Chronic lead poisoning, commoner in low and middle income inant peripheral neuropathy as seen here.Hypertension is known to be countries can cause neurological disorders. We present such a case associated. Albumin-cytological dissociation in CSF is a rare but ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 127 consistent ﬁnding. MRI typically shows hyperintensities in basal WCN19-1391 ganglia, thalami and cerebellum. Serum lead levels remain the best marker of toxicity. However in chronic disease the levels may not correlate with the outcome. Chelating agents used are either EDTA, Poster shift 02 - Autoimmune disorders - Part I /CNS infections - DMSA, or penicillamine. Seizures were controlled with anti-epileptics. Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other Conclusion Lead toxicity should be considered a possibility in ataxia due to Pattern of muscle involvement in dermatomyositis – A retrospec- combined sensory neuropathy & cerebellar dysfunction. Presentation tive analysis using STIR MRI imaging with status epilepticus was unusual. It is a reversible condition with chelation. S. Bhattacharjeea, B. Maramattom Varkeyb, J. Thomasc aAster Medcity- Kochi, Internal Medicine, Kochi, India doi:10.1016/j.jns.2019.10.1006 bAster Medcity- Kochi, Neurology, Kochi, India cAster Medcity- Kochi, Rheumatology, Kochi, India

All patients with suspected Idiopathic Inflammatory Myopathy, WCN19-1389 over a 4 year period - from August 2014 to August 2018, seen in the departments of Neurology and Rheumatology of Aster Medcity, Kochi, India, were included in the retrospective analysis. 28 patients Poster shift 02 - Autoimmune disorders - Part I /CNS infections - with DM were selected, who fulfilled the EULAR/ACR classification Part I / dementia - Part I /epilepsy - Part I / health economics and criteria. Of the 28 patients, 17 patients who had undergone fat outcomes / neuroepidemiology and environmental and other suppression sequence, short tau inversion recovery (STIR) MRI muscle imaging were selected. The MRI images were collated and Cerebrospinal fluid CXCL10 as a surrogate marker of therapy- analysed by a single examiner who has over 4 years of experience response and therapy-predict for HTLV-1-Associated myelopathy/ with muscle MRI. Of the 17 patients with STIR imaging, 12 tropical spastic paraparesis underwent a whole-body MRI and 5 underwent thigh muscle MRI only. K. Tamakia, T. Satob, J. Tsugawaa, S. Fujiokaa, N. Yagishitab, N. Arayab, We found that 76% patients were women, with a mean age of 48 J. Yamauchib, M. Nagasakac, S. Tsutsumic, Y. Yamanob, Y. Tsuboia yrs. aFukuoka University, Neurology, Fukuoka, Japan 13 were adult DM, 3 were amyopathic DM and 1 case was a bSt. Marianna University School of Medicine, Rare Diseases Research, juvenile DM (9 years). Institute of Medical Science, Kawasaki, Japan The mean disease duration was 10.8 months, and the mean time cSt. Marianna University Graduate School of Medicine, Advanced to MRI from diagnosis and initiation of treatment was 4.7 months Medical Innovation, Kawasaki, Japan except in 3 cases who presented very late (1-2 years after onset). Most muscle changes were patchy, involving muscles Since HTLV-1-associated myelopathy/tropical spastic paraparesis asymmetrically. (HAM/TSP) is a debilitating, progressive disease with no effective 70% cases showed involvement of lower limb muscles. And 30% treatments, there is a dire need to develop a novel therapy that truly showed upper limb and trunk muscle involvement. improves the long-term functional prognosis. However, this true Paraspinal muscles and shoulder girdles were involved in 50% endpoint is virtually impossible to consider as a primary endpoint in patients in whom whole-body MRI was performed. clinical trials, as it takes decades. Therefore, development of Interestingly, the muscles involved the most were the thigh surrogate markers that enable to assess the effectiveness of new (anterior and posterior compartment muscles), calf muscles, the interventions more rapidly with greater accuracy is essential. glutei and pelvic muscles. The pelvic muscles were frequently Currently, there are several candidate surrogate markers for HAM/ involved (47%), especially obturator internus and externus. TSP. Cerebrospinal fluid (CSF) CXCL10 in particular is involved in the pathogenesis and correlates with disease progression. Changes in doi:10.1016/j.jns.2019.10.1008 surrogate marker levels should be observed in response to treatment and they should correlate with prognosis. Here, we investigated these aspects of several markers, including CSF CXCL10. Patient information, motor function evaluation results, and CSF WCN19-1393 examination data from 13 patients with HAM/TSP who received steroid pulse therapy were collected retrospectively. We compared Poster shift 02 - Autoimmune disorders - Part I /CNS infections - the levels of CXCL10 and neopterin, total protein, cell counts, anti- HTLV-1 antibody titer (all CSF markers), and Osame motor disability Part I / dementia - Part I /epilepsy - Part I / health economics and scores (OMDS) before and after pulse therapy. outcomes / neuroepidemiology and environmental and other In conclusion, CXCL10 and neopterin levels in CSF declined in response to steroid pulse therapy and correlated with changes in An unusual survivor of status epilepticus: A case report prognosis based on continuation of treatment. In addition, patients with HAM/TSP who benefited from steroid pulse therapy had high A. Jadhava, A. Guptaa, G. Dashb CSF CXCL10 levels. Therefore, the CSF CXCL10 level would be useful aDiplomate of National Board-Mazumdar Shaw Medical Centre- as a surrogate marker of therapy-response and therapy-predict in Narayana Health-Bangalore-India, Neurology, Bangalore, India patients with HAM/TSP. bMazumdar Shaw Medical Centre-Narayana Health-Bangalore-India, Neurology, Bangalore, India doi:10.1016/j.jns.2019.10.1007 ARTICLE IN PRESS

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

Introduction (60-69 points); and 18 patients (47.4%) had high degree of We report an unusual case of status epilepticus (SE) depression (70 points) (p b0.01). According to the Spielberger State-Trait Anxiety Inventory the Case report level of personal anxiety equals to 31-45 score was present in 9 33 years old male underwent urology procedure for stone (23.4%) patients; 46 and higher scores marked 29 (76.3%) patients (p removal. 4 hours after extubation, he developed headache, b0.01). desaturation, unconsciousness and seizures. His mother and daugh- In connection with this category, patients need along with ter had history of epilepsy. On examination, he was on ventilator, antiretroviral therapy and psychotherapy, namely, social and psy- comatose. His CT and MRI brain were normal. EEG showed chological counseling, cognetive behavioral therapy, which were generalized spike wave discharges (GSW). Initial Routine Blood held in the course of 10 sessions, in conjunction with pharmaco- investigations were normal. He was on inj. Levetiracetam 1.5 gm IV therapy which was lasted for 6 months. Twice a day, Inj. Sodium Valproate 1 gm IV Twice a day, Inj. After the therapy data of psychometric tests were as follows: Lacosamide 200mg IV twice a day. Midazolam was titrated upto 300 63.2% in patients with severe and moderate depression observed mg/hr. At this time, his EEG showed persistent GSW with mild depression; in 78.4% of patients with severe anxiety showed intermittent clinical seizures. He was started on Ketamine and improvement on mild stage of anxiety. titrated upto 170mg/hr. His seizure continued but further dose could not be escalated in view of sepsis and hypotension. CSF showed Conclusion elevation of protein levels and other parameters were normal. After This study showed that using psychotherapy to patients with 2 days of sepsis management, 2nd cycle of Midazolam started. There AIDS in the presence of psycho-emotional disorders, facilitates the was no response to higher dose of Midazolam. So, infusion of severity of depression and anxiety, which leads to a relative thiopentone was started and titrated.Burst suppression achieved at improvement in the quality of life of patients. dose of 300mg/hr. Anesthesia recycling was done. Midazolam : 6 cycles (21days), Ketamine 2 cycles (5days), Thiopentone: 3 cycles doi:10.1016/j.jns.2019.10.1010 (15 days).MRI brain (Day 15) suggestive of sequelae of hypoxic ischaemic injury. He was discharged with Tab Levetiracetam 3 gram/ day, Tab sodium valproate 1000mg/day and clonazepam 8mg/day.At the time of discharge his modiﬁed rankin score was 5. WCN19-1397

Conclusion We postulate that aggressive management of SE would result in Poster shift 02 - Autoimmune disorders - Part I /CNS infections - good outcome. Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1009 Primary progressive aphasias: Phenotypical and etiological variability

WCN19-1396 A. Gharbia, A. Nasriab, S. Mrabetab, I. Kacemab, A. Gargouriab, M. Ben Jebaraab, R. Gouiderab aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia Poster shift 02 - Autoimmune disorders - Part I /CNS infections - bUniversity of Tunis El Manar, Faculty of Medicine of Tunis, Tunis, Part I / dementia - Part I /epilepsy - Part I / health economics and Tunisia outcomes / neuroepidemiology and environmental and other Background Principles of medical and psychological care of patients with the Primary Progressive Aphasias (PPA) are defined as an early and fi acquired immune de ciency syndrome relatively isolated progressive language impairment. This syndrome recognizes several phenotypes and different etiologies. N. Maxamatjanova Neurological diseases, Tashkent, Uzbekistan Objective To describe the clinical and radiological features of different Purpose forms of PPA in a Tunisian cohort. Explore the principles of providing medical and psychological care of patients with the acquired immune deficiency syndrome Patients and methods (AIDS) A 10 years retrospective study (2009-2019) including patients diagnosed with PPA according to Gorno-Tempini Criteria(2011) was Materials and methods conducted, at the Neurology Department of Razi Hospital. Demo- We examined 38 patients with AIDS, including 17 women, 21 graphic, clinical, neuropsychological and brain imaging features were men. The average age of patients was 30,2 ± 8,3. To verify the analyzed. diagnosis was used standard laboratory tests to determine the HIV infection, objective status. To investigate the psycho-emotional Results spectrum we used Tsung scale to determine depression and Weincluded 42 patients with sex-ratio=1.3. Age of onset of Spielberger State-Trait Anxiety Inventory. language impairment was 66.9 years. A memory complaint was present in 76.2% with a mean time from the language complaint of Results 2.0 years. Mean MMSE and FAB scores were respectively 14.6 and According to the Tsung scale 5 (13.1%) patients had mild 7.7. Episodic memory impairment was noted in 61.9% (hippocampal depression (50-59 points); 15 patients (39.5%) the average level profile in 28.6%). The logopenic variant (lvPPA) was the most ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 129 common (31.0%), followed by the semantic variant (svPPA=23.8%) and the nonfluent/agrammatic variant (nfvPPA=19.0%). Aphasia was unclassifiable in 26.2%. Two patients had a secondary etiology (toxic and normal pressure hydrocephalus). Among the neurodegenerative group, Alzheimer's disease (AD) was predominant (42.9%); lvPPA being the most common form (13/18) (nfvPPA=1/18, svPPA=1/17, unclassifiable=3/18). Fronto temporal lobar degeneration (FTLD) accounted for 26,2% of etiologies. Unlike AD, svPPA was most frequently found (8/11)(nfvAPP=3/11,lvAPP=0/11).

Conclusion The lvPPA was the most common phenotype in our series and AD the leading cause. NfvAPP and especially svAPP were predomiant in FTLD. Considering the absence of clinicopathological correspondance, the development of reliable biomarkers will specify the neuropathological diagnoses and avoid inappropriate therapeutic approach. doi:10.1016/j.jns.2019.10.1011

12yr old boy rt.handed presented with left partial seizures in WCN19-1406 clusters followed with left sided weakness.He had episode of right partial status epilepticus 6 months back and 3 months back. On examination he was drowsy with power 0 in left UL & LL with Poster shift 02 - Autoimmune disorders - Part I /CNS infections - increased salivation, neck stiffness. Skin examination showed Part I / dementia - Part I /epilepsy - Part I / health economics and giant melanocytic naevi on limbs, face, torso and back. MRI Brain outcomes / neuroepidemiology and environmental and other showed parafalcine hypointense meninges on T2, hyperintense meninges on T1 images with nodular leptomeningeal enhance- Neurocutaneous melanosis ment with hydrocephalus. CSF study showed 1 lymphocyte, proteins 14mg/dl, sugar 56mg/dl. CSF cytology showed abnormal A. Shetty cells which were stained with May Grunwald Giemsa showing Criticare Hospital, Neurology, Mumbai, India prominent nucleoli. CT chest and abdomen was normal.EEG showed generalised slowing with no epileptiform activity. His seizures were controlled Neurocutaneous melanosis is a rare fatal neurological ailment with LVT, LAC, OXZ, CLZ. He was also started on diamox, mannitol for characterised by cutaneous nevi with leptomeningeal melanosis. hydrocephalus. He had intermittent fever which was controlled with Though there is no effective treatment but able to diagnose and take antibiotics. He had residual left hemiplegia and had to be fed through care of symptoms and complications will help in the palliative care of rhyles tube. He did continue to have intermittent seizures for which these patients and would further help in counselling the relatives as antiepileptic drugs were adjusted. Our subject had all the risk factors per the prognosis of this disease. ARTICLE IN PRESS

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

for NCM reported as presence of a GCMN, male sex, satellite nevi or Discussion multiple CMN, and head, neck, or posterior midline location. Tuberculous cerebrovascular disease is a serious complication of CNS TB. Most TBM associated brain infarcts are multiple, bilateral, symmetric, located in the deep gray matter. Inﬁltrative, doi:10.1016/j.jns.2019.10.1012 proliferative and necrotising vessel pathologies have been described. There is some evidence that vasospasm may mediate stroke early in the course of the disease and proliferative intimal disease in later. WCN19-1408 Conclusion CNS TB should be considered a cause in young stroke patients Poster shift 02 - Autoimmune disorders - Part I/CNS infections - with unexplained cranial neuropathy and constitutional symptoms. Part I/dementia - Part I/epilepsy - Part I/health economics and A delay in diagnosis can lead to serious complications. outcomes/neuroepidemiology and environmental and other

An uncommon cause of young stroke

R. Sudath, I. Rajapakse, M. Aravinthan, B. Senanayake National Hospital of Sri Lanka, Institute of Neurology, Colombo, Sri Lanka

Introduction/Background Central nervous system tuberculosis (CNS TB) which accounts for 1-10% of all cases of TB can rarely present with cerebrovascular disease which is a serious complication. We present a young patient with an acute stroke as the ﬁrst manifestation of CNS tuberculosis.

Case report 15 year boy presented with sudden left hemiplegia. He was previously well apart from a few days of loss of appetite. He additionally had unexplained neurology of a dilated, reactive pupil on the left. His CSF was infected with a lymphocytic pleocytosis, high protein and a signiﬁcant sugar drop. In spite of empirical anti – meningitis therapy he deteriorated. MRI brain revealed a right basal ganglia T2 Hyper intensities which later became bilateral on T2/Flair with diffusion restriction on DWI and patchy contrast enhancement. [Fig 1and 2] He also developed autonomic instability. Empirical treatment with anti TB drugs and corticosteroids was commenced. doi:10.1016/j.jns.2019.10.1013 PCR Gene-X pert subsequently conﬁrmed the diagnosis of CNS TB. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 131

aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia, bUniversity of Tunis El Manar, Faculty of medicine, Tunis, Tunisia

Background Alzheimer's disease (AD) patients who develop symptoms before the age of 65 are classiﬁed as early onset (EOAD). The ɛ4 variant allele of apolipoprotein E (APOE ɛ4) is known to be the major genetic risk factor for Alzheimer's disease (AD). Several studies suggested opposite effects of APOE ɛ4inEOADandlate onset AD.

Objective Our aim was to explore the role of APOE ɛ4 variant in a Tunisian cohort of EOAD.

Methods We conducted a descriptive and retrospective cross-sectional study including patients with deﬁnite AD according to the recommendations of 2011 of the National Institute on Aging and Alzheimer's Association who had APOE genotyping with PCR–RFLP method, recruited from the department of Neurology of Razi University Hospital in Tunisia. We analyzed clinical and neuropsychological data.

Results We included 140 EOAD patients. One hundred patients (71.4%) were APOE ɛ4 carriers. Sex-ratio was 0,67 with no significant differences between APOE ɛ4 carriers and non carriers. Mean age was 76 years and mean age of onset = 56.9 years [41–64] and were both significantly older in APOE ɛ4 carriers (respectively p = 0,046 and p 〈10−3). Mean disease course was 6,14 years and was significantly shorter in ɛ4 carriers. There were no differences in dementia or executive dysfunction severity in APOE ɛ4 carriers and non carriers (mean MMSE = 14,2 and 16,8(p = 0,083); mean FAB = 7,4 and 8,6 respectively (p = 0,287)).

Conclusion APOE ɛ4 was common in EOAD associated with older age at onset and shorter disease course in line with previous studies.

doi:10.1016/j.jns.2019.10.1014

WCN19-1416

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

Auto immune limbic encephalitis; clinical proﬁle and treatment response – a single center experience WCN19-1412 ^ P. Sarathchandran, S. AlRukn, A. AlMadani Rashid Hospital, Neurology, Dubai, United Arab Emirates Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and Background outcomes/neuroepidemiology and environmental and other Auto immune limbic encephalitis are usually antibody mediated and may be a paraneoplastic manifestation. Early onset APOE Ɛ4-positive Alzheimer's disease patients: Are they different? Results We report a case series of thirteen patients were admitted with A. Nasriab, I. Sghaiera, A. Gharbia, S. Mrabetab, A. Gargouriab, M. Ben features suggestive of auto-immune encephalitis from January 2014 – Jebaraab, I. Kacemb, R. Gouiderab and March 2019. Age range was 18 37, with male: female ratio of ARTICLE IN PRESS

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

1:4.5. All of the patients had prodromal respiratory catarrh a week or two preceding onset of neurological symptoms. Psychosis was a prominent initial symptom in all patients. Extrapyramidal symptoms were seen in six. Seizures occurred in eleven patients, three of whom were refractory. Three had associated autonomic instability. Antibody testing was positive for VGKC antibodies in ﬁve, anti NMDA antibodies in six and two patients were seronegative for any antibodies. MRI was normal in anti NMDA encephalitis. It showed mesial temporal swelling/hyper intensities in patients with anti VGKC encephalitis, two of whom developed mesial temporal sclerosis at one year follow up. Antibody titers did not correlate with clinical manifestations. Paraneoplastic work up was positive for ovarian teratoma in one patient with anti NMDA encephalitis who had refractory encephalopathy despite tumor removal and aggressive immunotherapy. Most patients had near complete recovery with treatment (methyl prednisolone, immunoglobulins and/or PLEX). Most patients with anti VGKC encephalitis required long term anti epileptic medications.

Conclusion Prominent psychosis, dysautonomia and extrapyramidal symptoms after a prodrome of respiratory catarrh warrant early consideration of autoimmune encephalitis since early treatment initiation determines the outcome. Paraneoplastic etiology portends a poor prognosis. doi:10.1016/j.jns.2019.10.1015

WCN19-1422

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

Silent intracerebral hydatid cyst presenting with atypical neurologic deﬁcits

R.J. Ketarena, J. Simcab, V. Hartoyob aSiloam Hospitals Lippo Village, Neurology, Tangerang, Indonesia; bFaculty of Medicine- Universitas Pelita Harapan, Neurology, Tangerang, Indonesia

Introduction Hydatid disease is a scarce parasitic infection seen in 2% of all cases. Considered as one of the most geographically widespread zoonoses in the world, this disease is highly endemic in eastern countries. Intracerebral hydatid cyst has been identified as one of the right frontal lobe until corona radiate (fig. 1). MR spectroscopy done most common manifestations encountered with patients usually after that showed both Lactate and Succinate peak with no elevated come with symptoms indicating increased intracerebral pressure. Cholin or Cholin/Certain ratio, suggesting a hydatid cyst (fig. 2 and Surgical therapy remains as first line of therapy. 3). She denied any contact with possible infection source.

Objective Results To report a case of silent intracerebral hydatid cyst with atypical Due to the nature of her symptoms, she was opted for fi conservative treatment with close monitoring of her disease's neurologic de cits^ progression. She was given gabapentin 100 mg three times daily Case and eperisone 50 mg twice daily. Currently she has minimal A 65 year-old female admitted to outpatient department with symptoms and function well. chief complaint of chronic neck and right arm pain since 6 months prior. She denied headache, weakness, any visual field nor sight Conclusion disturbance. Neurological examinations were unremarkable. During Frontal brain cysts may exhibit very subtle neurological deficits, routine brain CT however, a mass was found which expanded within as such physicians need to be meticulous. Surgical removal of the ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 133 cyst still remains as mainstay treatment; nevertheless, conservative remain unknown (Bell's palsy). The most often cause of iatrogenic treatment may be considered if the latter is yet possible to be done. nerve damage is parotid gland surgery (facial nerve runs through the gland). The surgery is the treatment of choice in both benign and doi:10.1016/j.jns.2019.10.1016 malignant tumors. Finding the biomarker of those tumors might inﬂuence the treatment decision and preserve the function of facial nerve.

WCN19-1423 Aim ^ In present study we investigated the serum levels of selected adipocytokines in patients with parotid gland tumors (benign: 36 Poster shift 02 - Autoimmune disorders - Part I/CNS infections - patients with pleomorphic adenoma (PA group), 25 patients with Part I/dementia - Part I/epilepsy - Part I/health economics and Warthin's tumor (WT group) and 11 with malignant tumors). The outcomes/neuroepidemiology and environmental and other control group included 30 healthy individuals.

An interesting case of VZV encephalitis with intraparenchymal Results hemorrhage The mean adiponectin concentration was significantly higher in benign tumor patients compared to controls in both genders in PA N. Surya, M. Desai and WT groups. Total serum leptin levels in all of our patients (males Bombay Hospital and Research Centre, Neurology, Mumbai, India and females) did not differ between groups. Serum visfatin concentrations were similar in all tumor patients in PA, WT and malignant groups and were significantly higher compared to We present a case of Varicella zoster encephalitis in a 62 year controls. adult male, presented with complaints of fever, headache, vesicular rash, altered sensorium. On examination his vitals were normal, Conclusions neurological examination showed drowsy but arousable state, brisk In summary we showed that adiponectin plays an “anti- reflexes and extensor planter response. Routine blood investigations malignant” role in benign tumors. Furthermore, leptin and visfatin were within normal limits. Csf studies showed Wbc counts 2000 seem to play an important role in the pathology of salivary gland (lymphocytic), 8350 Rbc, protein 265 and Glucose 45. Csf PCR for tumors, although in both genders in a different manner. Our findings varicella zoster virus was positive. Mri findings showed features of may have a potential impact on decision of treatment and therefore meningo-encephalitis with cerebellar hemorrhage. Pt was treated the quality of life related with facial nerve function. with Iv antivirals, anti-biotics and other supportive treatment. Gradually pt. improved and was shifted out of Icu in 5 days and doi:10.1016/j.jns.2019.10.1018 discharged 15 days later. Varicella zoster virus is an exclusively human neurotrophic virus. VZV can involve virtually every part of the CNS and PNS. The primary infection with the virus causes varicella. The virus remains latent in nervous tissue and upon secondary activation causes a variety of syndromes involving the WCN19-^1433 central nervous system (CNS) including meningoencephalitis and “ ” cerebellitis. Zoster encephalitis can occur in both immunocompe- Poster shift 02 - Autoimmune disorders - Part I/CNS infections - tent and immunocompromised hosts. VZV encephalitis is in fact a Part I/dementia - Part I/epilepsy - Part I/health economics and vasculopathy rather than true encephalitis. outcomes/neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1017 The contribution of brain dysfunction in the formation of depressive disorders in epilepsy

a b c b a WCN19-1428 L. Lipatova , T. Kapustina , T. Dakukina , A. Roshka , T. Alexeeva ^ aAlmazov National Medical Research Centre- Saint-Petersburg- Russia, Neurology and Psychiatry, St. Petersburg, Russia; Poster shift 02 - Autoimmune disorders - Part I/CNS infections - bBekhterev National Medical Research Centre of Psychiatry and Part I/dementia - Part I/epilepsy - Part I/health economics and Neurology, Epilepsy, Saint-Petersburg, Russia; c outcomes/neuroepidemiology and environmental and other The state institution «Republican Research and Practice Center for Mental Health»- Minsk- Belarus, Psychiatry, Minsk, Belarus Parotid gland surgery as iatrogenic cause of facial nerve palsy – The role of adipocytokines in the pathology of parotid gland The aim of the study is to investigate the metabolism of tumors ﬂudeoxyglucose (F18-FDG) in the brain of patients with epilepsy and associated depression (PED) in interictal period by the method of P. Sowaa, M. Misioleka, M. Adamczyk-Sowab positron emission tomography (PET) and EEG. In particular, such as: aMedical University of Silesia, Department of Otorhinolaryngology and structural abnormalities, monoamine pathways, cerebral glucose Laryngological Oncology in Zabrze, Katowice, Poland; metabolism, all play a role in the common pathogenesis of these bMedical University of Silesia, Department Neurology in Zabrze, conditions. We examined 50 patients (PED), all PED using MRI and Katowice, Poland. F18-FDG PET of the brain, EEG. EEG results were processed using coherent analysis (CA). All subjects were divided into 3 groups: astheno-depressive (ADS) 40%, hypochondriac (HDS) 28%, depressive- Background dysphoric (DDS) 32%. According to the results of CA in all three groups Facial nerve paresis is an important implicator of human quality disintegration between intrahemispheric and interhemispheric of life. The are many known causes of facial nerve palsy but some still ARTICLE IN PRESS

134 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx interactions was observed, more pronounced changes were revealed Objective in PED group with astheno-depressive symptoms. In groups PED with To determine the various aetiologies of pachymeningitis, the HDS and DDS revealed decreased metabolism throughout the cortex, clinical proﬁle, the various treatment strategies, patient outcome. in both anterior and posterior insula regions left as well as right hippocampal parahippocampal regions, activity in the cerebellum. Material and methods However, in the group with severe ADS revealed increased activity in 27 patients with clinical & radiological diagnosis of the right parahippocampal and left anterior cingulate regions, and pachymeningitis were included in the study from Jan 2014 – April decreased activity in the left fusiform gyrus, left superior temporal, left 2019. They were followed up over 1-2 yrs. Thorough work up for angular gyrus, and left insula. Thus, on the basis of the data obtained, secondary causes was done. Treatment of underlying cause was done we suggest that affective disorders may be one of the signs of the and immunosuppression was given. Outcome was analysed on formation of connective and the network pathology. follow up. . doi:10.1016/j.jns.2019.10.1019 Images MRI of a patient with idiopathic hypertrophic pachymeningits with 8,9,10,11,12 cranial nerve palsy. WCN19-1436 Meningeal biopsy showing multiple non-caseating granulomas in ^ a patient with neurosarcoid.

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Discussion Part I/dementia - Part I/epilepsy - Part I/health economics and Idiopathic pachymeningitis is diagnosis of exclusion. Hence outcomes/neuroepidemiology and environmental and other thorough work up required to rule out secondary causes. Highly responsive to steroids. Hypertrophic pachymeningitis: Is it really idiopathic? Conclusion P. Barvalia, S. Patil, K. Jagiasi, P. Ojha, R. Singh, G. Soni, V. Aglave, N. In our study headache was the universal symptom, responded to Kadam immunotherapy. We found secondary causes in 13 cases. Etiological Grant Medical College and Sir J J Group of hospitals, Neurology, Mumbai, workup is of utmost importance as treatment changes. Recurrence is India. high, long term immunotherapy is required. The disease is treatable. I have obtained patient and/or Institutional Review Board (IRB) approval, as necessary. An Institutional Review Board (IRB) and/or Background Animal Use Committee have waived the requirement for their formal Hypertrophic pachymeningitis is a disorder characterized by approval of the study. localized or diffuse enlargement of the dura mater. doi:10.1016/j.jns.2019.10.1020 Aim To elucidate the various characteristics of patients presenting with hypertrophic pachymeningitis. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 135

WCN19-1445 Background ^ Stereoelectroencephalography (SEEG) is shown to be a valuable tool for the anatomoelectroclinical definition of the epileptogenic Poster shift 02 - Autoimmune disorders - Part I/CNS infections - zone in patients with drug refractory epilepsy (DRE) considered for Part I/dementia - Part I/epilepsy - Part I/health economics and surgery especially useful in patients who are MRI negative or have outcomes/neuroepidemiology and environmental and other lesions in eloquent or deep seated brain areas. In India, many patients are not offered this diagnostic procedure for reasons such as Features of the structure of epileptic syndrome and cognitive cost and limited technical resources. disorders in patients with cerebral arteriovenous malformation Objective a b c d L. Lipatova , T. Kapustina , T. Dakukina , T. Alexeeva To document the initial experience with the effectiveness^, safety aAlmazov National Medical Research Centre- Saint-Petersburg- Russia, and outcomes of surgeries following SEEG procedure at our centre. Neurology and Psychiatry, Saint-Petersburg, Russia; bNational Medical Research Centre of Psychiatry and Neurology named Methods – after V.M. Bekhterev, Epilepsy, Saint-Petersburg, Russia; 5 patients (age range 23 ^45 years) underwent SEEG monitoring c The Republican Research and Practical Center for Epidemiology and over the last eighteen months. Three of these were MRI negative^, Microbiology- Minsk- Belarus, Psychiatry, Minsk, Belarus; Additional non invasive evaluation viz. PET and MEG was done in all dNeurology and Psychiatry, Saint-Petersburg, Russia these cases. MEG was useful in planning the surgical strategy in one of the three patients. 2 had subtle MRI lesions with corresponding Objective PET hypometabolism. The SEEG helped limit the area of resection in To assess the frequency, structure of epileptic syndrome and both and avoid eloquent cortex in one. cognitive impairment in patients with cerebral AVM (PAVM). The main group consisted of 78 PAVM: average age of 36.2. The results of ^Results the study:1 subgroup- a right-hemisphere localization - 41(53%); 2 All underwent surgery, 2 insular, one frontal, one temporal and subgroup - a left-hemisphere localization – 37(47%). Of these, 33% one frontal opercula, of which two were done in the same admission. frontal lobe; 12% the temporal lobe;17% area central convolutions; Two had acute post operative seizures, and of these one underwent a 28% the parietal and the occipital lobe;10% - subcortical structures. In redo surgery for incomplete resection. None developed post fi all cases evaluated, the frequency of epileptic seizures: frequent 11%; operative sensorimotor de cits. All patients have an ENGEL class IA – moderate 50%; rare 28%; seizures were not observed in 11% cases. outcome over the follow up period (^1 ^18 months). The structure of epileptic syndrome was presented in the following seizures: partial sensorimotor in 29%; vegetative-visceral - 40%; Pathologies observed were FCD^ mixed: complex partial; secondary generalized - 31%. Changes in EEG SEEG is a safe useful and reliable option for invasive monitoring in 14% cases, changes were not observed; moderate diffuse changes in patients with DRE, providing means for seizure localization. at the level of diencephalic structures –23%; paroxysmal activity of ^ doi:10.1016/j.jns.2019.10.1022 malformations with an interest in diencephalic structures of the – brain-41%;the formation of a mirror focus ^10%, moderate irritative – changes of EEG ^7%; the presence of the pattern of epileptiform activity-5%. Cognitive impairment of varying severity degree were identified in PAVM: mild cognitive disorders – 21%; mild cognitive WCN19-1479 impairment – 64%; dementia from mild to severe, severity – 13%. The duration and frequency of the epileptic syndrome of the complex Poster shift 02 - Autoimmune disorders - Part I/CNS infections - structure aggravate the course of cognitive impairment, thereby Part I/dementia - Part I/epilepsy - Part I/health economics and reducing quality of life of PAVM. A comprehensive assessment of cognitive disorders, structure and frequency of epileptic syndrome outcomes/neuroepidemiology and environmental and other can be a diagnostic criterion of the results of the immediate outcomes of endovascular treatment of AVM. Fatal ameobic meningo-enchephalitis in immunocompetent host in resource limited setting in India doi:10.1016/j.jns.2019.10.1021 S. Chavanda Chavanda Hospital, Medicine, Latur, India

WCN19-1446 We present a rare case of N.fowleri living amoeba meningitis in immunocompetent person who used soiled expired eye drops for symptomatic relief for itching which was prescribed by some general Poster shift 02 - Autoimmune disorders - Part I/CNS infections - practitioner long back patient had fulminant course of disease Part I/dementia - Part I/epilepsy - Part I/health economics and expired within almost 4 days of onset of disease, diagnosis was outcomes/neuroepidemiology and environmental and other made on basis of clinical signs of meningism, csf examination revealed neutrophilic predominance meningitis with simple wet Early experience with stereo EEG from a quaternary hospital in mount light microscopy showed amoebic motility, PCR studies was India not possible due to resource limited setting. While N. fowleri infection appears to be quite rare compared to other diseases, the D.S. Iyer, J. Mani, A. Kumar, D. Sanghavi, M. Munshi, C. Millar clinical manifestations of primary amoebic meningoencephalitis are Kokilaben Dhirubhai Ambani Hospital, Neurology, Mumbai, India devastating and nearly always fatal. Due to the rarity of N. ARTICLE IN PRESS

136 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx fowleriinfections in humans, there are no clinical trials to date that F. Oukhai, M. Mesellmani, A. Ouerdiene, M. Mansour, J. Zaouali, R. assess the efficacy of one treatment regimen over another. Most of Mrissa the information regarding medication efficacy is based on either case Military Hospital for Instruction of Tunis, Neurology, Tunis, Tunisia reports or in vitro studies. Secondly simple test of wet mount in light microscopy has diagnostic values in resource limited settings. Background Adie’s tonic pupil is a neurological disorder more common in doi:10.1016/j.jns.2019.10.1023 women. It is characterized by persistent unilateral mydriasis. Tonic pupil constricts poorly to light but reacts better to accommodation. It is caused by parasympathetic ciliary ganglion damage and is characterized by hypersensitivity to muscarinic receptor agonists WCN19-1484 (pilocarpine). Most cases are idiopathic, though it can be caused by autoimmune diseases, tumors, trauma, and surgery. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and Case report outcomes/neuroepidemiology and environmental and other We report a case of a 39-year-old woman with a past medical history for migrainous headache, left knee and bilateral femoral head Paraneoplastic neuropathies. Antibodies spectrum osteonecrosis, anemia and arthritis. She was diagnosed with sjogren syndrome based on symptoms of mouth and eyes dryness, positive D. Labunskiy serum anti-RO/SSA and ANA antibodies and labial salivary gland N.P. Ogarev Mordovia State University N.P., Neurology and Psychiatry, biopsy showing lymphocytic sialadenitis. She presented with pro- Saransk, Russia gressive vision loss in the right eye. The initial examination showed anisocoria, an afferent pupillary Small-cell lung cancer (SCLC) sometimes is associated with defect and a sector pupil palsy of the right eye. Ophthalmoscopic fi paraneoplastic neurological autoimmunity. Different antibodies have evaluation, visual acuity and eld testing were normal. Visual- fl been used for diagnosis of this condition and for diagnosis and signs evoked potentials (VEPs) were normal. Ankle jerk re exes were of therapy results. Therefore various types of antibodies play a absent. ’ fi pathogenic role in mechanisms of autoimmune processes in The diagnosis of Adie s tonic pupil was con rmed with rapid paraneoplastic neuropathies (PN). miotic response of the affected pupil to 0.125% pilocarpine drop. The Under our observation were 59 patients with different type of PN, patient was treated with Pilocarpine drops and had slightly 37 from them suffered from SCLC with paraneoplastic neuropathies, improved symptoms. and 11 from Gullian-Barre syndrome. Control group consists of 246 neurologically healthy donors. Cerebrospinal fluid (CSF) was ob- Conclusions ’ tained from urological patients during spinal anesthesia and Adie s tonic pupil is a benign, mostly idiopathic syndrome caused consequent surgeries. by parasympathetic denervation. However, it can be associated with fl We used ELISA and Western Blot analysis for detection of anti- infectious, in ammatory, malignant, or autoimmune pathologies. myelin-associated glycoprotein antibodies (MAG), anti-ganglioside Systemic conditions associated with tonic pupils are rare. This is, as fi antibodies. which are principally associated with autoimmune far as we know, the rst case reported of a sjogren syndrome peripheral neuropathies and anti-glutamic acid decarboxylase anti- associated with tonic pupil. bodies in serum and CSF of all patients and control group. Besides this AB against urinergic receptor channel P2X purinoceptor 7 doi:10.1016/j.jns.2019.10.1025

(P2X7), the voltage-gated potassium channel KV1.3 and the voltage-gated sodium channel NaV1.7 as examples of targeting ion channels were studied. It was revealed that in 78 per cent of PN all AB were increased WCN19-1486 two and three-fold. Ion channels AB were especially raised. In conclusion, we can state that antibody response plays a key Poster shift 02 - Autoimmune disorders - Part I/CNS infections - role in pathogenesis of all autoimmune neuropathies. Paraneoplastic Part I/dementia - Part I/epilepsy - Part I/health economics and pathology in this regard is essential. Future research has to be done consider ion channel involvement and autoimmune reactivity outcomes/neuroepidemiology and environmental and other toward them. Age and education effect on verbal ﬂuency in Saudi Arabia doi:10.1016/j.jns.2019.10.1024 T. AlMuayqil, R. AlShathri, H. AlNasser, L. AlFawzan, A. AlQarni King Saud University, Medicine, Riyadh, Saudi Arabia

WCN19-1485 Objectives • Measuring normative performance of verbal fluency across different age and education groups. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - • Determining which fluency tasks are not influenced by age and Part I/dementia - Part I/epilepsy - Part I/health economics and education. outcomes/neuroepidemiology and environmental and other • Looking at performance on the test according to letter and category properties. Adie’s tonic pupil in sjogren's syndrome ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 137

Methods molecular weight of FITC-Dextrans and the intensity of fluorescent A cross sectional study with an estimated sample size of 246, the intensity was focused in the cerebral cortex. The antibodies were not age range is from (18-100) years from both genders. Participants enough to neutralize the concurrent RABV to prevent mice death, were recruited from the community and through convenient while the permeability of BBB was efficient at day 5th of post- recruitment in Riyadh city, Saudi Arabia. The study was performed immunization. The FITC-Dextran is deduced as an important on Arabic speaking population. We used two types of verbal fluency fluorescent tagged drug that can reveal the pathogenicity of central tasks, semantic and phonemic. Each task was limited to 1 minute. nervous system based viral infectious diseases. The semantic fluency task consists of four categories: four legged animals, boys names, girls names and countries. Whereas in doi:10.1016/j.jns.2019.10.1027 phonemic fluency, the letters (Ain, Sheen, Qaf) were used. Data was analyzed using IBM SPSS system (21.0).

Results WCN19-1499 Comparing males with females, males performed better in ^ countries, the letter “Sheen” and “Qaf”. Whereas females performed better in girls names, and no difference was noted in boys names, Poster shift 02 - Autoimmune disorders - Part I/CNS infections - four-legged animals and the letter “Ain”. Both semantic and Part I/dementia - Part I/epilepsy - Part I/health economics and phonemic fluency were significantly affected by increase in age, outcomes/neuroepidemiology and environmental and other except for the letter “Sheen”. Increase in the levels of education has been shown to significantly affect both semantic and phonemic Status epilepticus in intracranial hypotension, rare complication fluency, with the lowest performance for people with 6 years of of CSF diversion education or less. P. Sarathchandran, R. AlSuwaidi, J. Inshasi, A. AlMadani Conclusion Rashid Hospital, Neurology, Dubai, United Arab Emirates Semantic and phonemic fluency were significantly affected by age ^ and education, while the effect of gender was noticed according to Back ground the category. A relationship between seizures and intracranial pressure (ICP) has been proposed, but not clearly identified. doi:10.1016/j.jns.2019.10.1026 ^ Materials and methods We report two cases of status epilepticus on patients shunted for hydrocephalus. WCN19-1492 Case 1: Twenty nine year old Pakistani gentleman, presented with history of headache, diplopia and unsteadiness of gait. CT brain Poster shift 02 - Autoimmune disorders - Part I/CNS infections - showed communicating hydrocephalus. He underwent VP shunt, which improved his symptoms. CSF analysis was normal. Two weeks Part I/dementia - Part I/epilepsy - Part I/health economics and later he presented with parkinsonism, bulbar dysfunction and outcomes/neuroepidemiology and environmental and other encephalopathy followed by generalized seizures. Repeat CT showed diffuse cerebral edema, sagging brainstem and downward drooping fl Permeability patterns of uorescent isothiocyanate dextranin splenium of the corpus callosum suggesting intracranial hypoten- blood-brain barrier of micemodel sion. He was treated with anti epileptic drugs (AED), but his EEG continued to show electrographic seizures. His VP shunt was a b b W. Ahmad , A. Ahmad , Q. Akram programmed to a higher resistance level, reducing the CSF diversion a KBCMA College of Veterinary and Animal Sciences- Narowal- Pakistan, rate and thus improving the intracranial hypotension. His symptoms Department of Clinical Sciences, Narowal, Pakistan; improved and he became seizure free. EEG showed improvement in b KBCMA College of Veterinary and Animal Sciences- Narowal- Pakistan, parameters. Department of Pathobiology, Narowal, Pakistan Case 2: Thirty one year old Nigerian gentleman presented with fever and altered sensorium. CT showed communicating hydroceph- Rabies is considered as a deadly zoonotic disease especially in alus. He was diagnosed to have tuberculous meningitis based on CSF lower socioeconomic states. The predilection site of rabies virus analysis including culture. He underwent VP shunt and was started (RABV) is brain tissue where it develops severe encephalitis on antituberculous therapy and steroids. Two months later he predominantly through neuronal dysfunction. The present study started having seizures refractory to multiple AEDs. CT showed has demonstrated the variations in permeability patterns and features of intracranial hypotension and EEG showed electrographic corresponding Blood Brain Barrier (BBB) damage in experimental seizures.^ rabid lab models through use of fluorescent isothiocyanatedextrans (FITC-Dextrans) in three different weights (70kDa, 150kDa and Conclusion 200kDa).The FITC-Dextran has been extensively used in the study of Intracranial hypotension can present with varying manifestations. microcirculation and capillary permeability assessment in the field of Seizures in a patient who has a shunt procedure should warrant a pharmacokinetics. The rabies infected mice models were selected in consideration of intracranial hypotension since they respond to this study. These animals were post-immunized through antibodies changing CSF pressures only. that could decrease the death ratio of mice by neutralizing the virus particles. In order to observe these changes, the brain tissues of the doi:10.1016/j.jns.2019.10.1028 mice were processed through immunofluorescence. It was revealed that BBB was considerably been crossed by 70kDa and 150kDa ARTICLE IN PRESS

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

WCN19-1510 ischemic preconditioning (IPC) in hyperlipidemic (Hpl) rat heart. ^ Experimental Hpl was produced by feeding high fat diet to rats for a period of 28 days. Isolated langendorff's perfused normal and Hpl rat Poster shift 02 - Autoimmune disorders - Part I/CNS infections - hearts were subjected to global ischemia for 30 min followed by Part I/dementia - Part I/epilepsy - Part I/health economics and reperfusion for 120 min. The myocardial infarct size was assessed outcomes/neuroepidemiology and environmental and other macroscopically using triphenyltetrazolium chloride staining. Coro- nary effluent was analyzed for lactate dehydrogenase (LDH) and Role of fibrates in attenuated cardioprotective effect of ischemic creatine kinase-MB release to assess the extent of cardiac injury. preconditioning in hyperlipidemic rat heart Moreover, the oxidative stress in heart was assessed by measuring thiobarbituric acid reactive substance, superoxide anion generation S. Mehtaab, G. Singhab and reduced form of glutathione. Moreover, I/R produced myocardial a injury, which was assessed in terms of increase in myocardial infarct Rayat-Bahra University- Kharar,^140104, SAS Nagar, Punjab, India bDepartment of Pharmacology, University School of Pharmaceutical size, LDH and CK-MB release in coronary effluent and decrease in coronary flow rate. Four episodes of IPC (5 min each) afforded Sciences, SAS, Nagar, India^ cardioprotection against I/R-induced myocardial injury in normal rat The present study has been designed to investigate the beneficial heart. On the other hand, IPC mediated myocardial protection against role of Fenofibrate in attenuated the cardioprotective effect of I/R-injury was abolished in Hpl rat heart. However, Treatment with ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 139

Fenoﬁbrate (100 mg/kg/day, i.p.) as agonists of PPAR-α have not Understanding of extent and nature of PPS could improve manage- affected the cardioprotective effect of IPC in normal rat heart, but its ment of epileptic patients.^ treatment markedly restored the cardioprotective potentials of IPC in Hpl rat heart. It is noted that the high degree of oxidative stress doi:10.1016/j.jns.2019.10.1030 produced in Hpl rat heart during reperfusion and consequent down regulation of PPAR-α may be responsible to abolish the cardioprotectivepotentials of IPC. WCN19-1514 doi:10.1016/j.jns.2019.10.1029 ^

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and WCN19-1511 outcomes/neuroepidemiology and environmental and other

Reproductive endocrine functions in women with epilepsy Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and B. Menona, K. Ramalingamb outcomes/neuroepidemiology and environmental and other aApollo Speciality Hospitals, Neurology, Nellore, India bNarayana Medical College and Superspeciality Hospital, Biochemistry, Occurence of postictal psychiatric manifestations in epileptic Nellore, India patients Women with epilepsy (WWE) have higher incidence of hormonal a ab a ab ab R. Jouini , S. Mrabet , F. Ellouze , A. Nasri , M. Djebara , A. disturbance in comparison to the general female population. We ab ab ab Gargouri-Berrechid , I. Kacem , R. Gouider analyzed the reproductive endocrine functions in patients with a Razi Hospital, Neurology, LR18SP03 Mannouba, Tunisia idiopathic generalized epilepsy and compared with controls. bUniversity of Tunis El Manar, Faculty of Medicine of Tunis, Tunis, Tunisia^ Materials & methods The study was conducted in the outpatient department of Background^ Neurology at a superspeciality hospital from south India. Women of Postictal psychiatric symptoms (PPS) are defined by occurrence, reproductive age with epilepsy were recruited for the study. Patients one week after a seizure, of depression, anxiety or psychosis besides in post menopausal state, pregnancy, who have undergone oophorec- any other medical condition. Our aims were to asses PPS and to tomy, use of oral contraceptive pills, patients with systemic illness, investigate relationships^between PPS and the characteristics of the long term medication for other diseases or progressive or degenerative illness.^ diseases were excluded. Five ml of venous blood was collected from 3rd day of follicular phase in fasting condition from the women study Methods ^ subjects. Free T3(fT3), Free T4(fT4) and Thyroid Stimulating Hormone It is a cross sectional study conducted between January and May (TSH), Follicular Stimulating Hormone(FSH). Luteinizing Hormone 2019 including patients diagnosed with epilepsy, and who consulted (LH), Prolactin, Testosterone and Estrodiol were measured. Hormones within 7 ^days from a seizure occurrence at the neurology depart- like serum Androstenedione, dehyroepiandrosterone(DHEA) and ment of Razi hospital. Assessment was performed through the serum17-OH Progesterone were measured. Modified Mini Screen (MMS) identifying patients with mood disorder, anxiety disorder or psychotic symptoms. Results Mean age of patients (N = 100) 22.78 + 7.08 and controls (N = Results^ 75) was 24.45+ 4.52. BMI was higher in patients (23.87 + 5.18) fi fi Fourty ve patients were assessed within 7 ^days from the last than in controls (21.87 + 2.11) p = .05. There was no signi cant seizure. Sex ratio was 1. Mean age was 38.6^years +^14. Mean duration difference in thyroid hormone levels and prolactin. There was a of illness was 15.5^years +^11.1. About 71% of patients had idiopathic lower level of progesterone(0.54 + 0.27) vs. 1.01 + 0.34 ng/ml), p epilepsy. Seizures were generalized in 49% and focal in 51%. Using = .00; higher levels of dehydroepiandrostenedione (0.69 + 0.25vs. MMS, 53% of the patients had a global score less than 6 points resulting 0.54 + 0.38μg/mL), p = .05 and luteinizing hormone-LH (6.01 + in a low likelihood of mental disorder, 30% had a moderate likelihood 3.90mIU/mL vs. 2.85 + 1.36mIU/mL), p = .00, higher LH/FSH ration of having mental disorder and 18% had a threshold score over 9 which (0.87 + 0.50 vs 0.32 + 0.16, p = .00). AED'S were Phenytoin (70%), is a high likelihood of having mental disorder. Identified PPS were Carbamazepine(26%), Valproate (4%). There was no significant mood symptoms (84%), anxiety (71%) and psychosis (24%). PPS was difference between individual AED's. correlated with a positive family history of psychiatric illness and electroencephalographic abnormalities.^ Conclusion This study demonstrates that WWE have reproductive endocrine Conclusion abnormalities. The role of epilepsy versus EIAEDS needs further Our results showed a high prevalence of PPS. The lack of evaluation. standardized diagnostic tools could explain these disparities. doi:10.1016/j.jns.2019.10.1031 ARTICLE IN PRESS

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

WCN19-1517 Background ^ Literature review show an association between chorea and antiphospholipid (aPL) antibodies in systemic lupus erythematosus Poster shift 02 - autoimmune disorders - Part I^/CNS infections - (SLE) patients. Chorea is recognized as one neurologic syndrome of Part I^/^dementia - Part I^/epilepsy - Part I^/^health economics and SLE. outcomes^/^neuroepidemiology and environmental and other Objectives MRI-etiology parallels study of medial and lateral temporal lobe We described a case with chorea as the ﬁrst manifestation of SLE without aPL criteria in the following. epilepsy in Uzbekistan^ ^

A. Sattarova, G. Rakhimbaeva, D. Sattarova Methods Tashkent Medical Academy, Department of Neurology, Tashkent, A22^years old man with medical history of focal epilepsy diagnosed six months previously and family history of SLE develop- Uzbekistan^ ^ ment unilateral left body choreic movements of four weeks of Objective evolution. Thus, we made an approach to patients with chorea MRI-etiology dedicated study on Temporal lobe epilepsy (TLE) within surround context of mainly etiology in Mexico (Syndenhann has not previously conducted in Uzbekistan. We study MRI etiology Chorea, Medications, Hungtinton disease, HIV).^ factors and structural pathology in lateral and medial temporal Serum liver enzymes in normal values. The Folstein test was 28/ epilepsy (LTLE and MTLE). 30 points.^ Anti-streptolysin O titer: 120 UI (N200 UI). Antinuclear antibod- b b Methods ies: 1:640 UI ( 1:160 UI by IFI), anti DNAds antibodies: 23 UI ( 10 N The results based on of examination of 48 patients with MTLE and UI), anticardiolipin IgG antibody 83 UI ( 20 UI).^ LTLE, who were on a stationary examination in the department of Cranial IRM showed a hyperintensity in right caudatus body fi neurology of the first clinic of Tashkent Medical Academy from 2016 (FLAIR sequence) as mainly nding. to 2018 years. The study was performed on an MRI-tomography with The patient started symptomatic treatment with risperidone in a magnetic field induction of 1.5 T. All patients underwent standard high dose and inmunosupressor therapy by methylprednisolone protocol programs. bolus plus cyclofosfamide pulse with better improvement in movement disorder. He was classified as SLE (SLEDAI 2012 criteria) Results without systemic involvement. During the following he didn't have The most common etiologic factor in the group of patients with positive aPL antibodies, and aPL syndrome criteria. MTLE was sclerosis of the hippocampus (78.2%, p = .02), while in the group of patients with LTLE with the same frequency, the Conclusions main etiological factor was tumors and craniocerebral traumas of Conclusions: This a rare case suggested that other antibodies are 36.4% and 31.6% respectively, p = .02. The tumor factor in the involved in neurological features of SLE and aPL syndrome or MTLE group was only 8.4%, p = .02. Malformation of the cortex vasculitis secondary didn't the only mechanisms that development and infectious diseases of central nervous system (CNS) of movement disorders in SLE. were found to be the same in both groups at 8.4% accordingly, doi:10.1016/j.jns.2019.10.1033 p = .02%.

Conclusion To sum up, pathological ﬁndings of damage in the medial temporal lobe, resulting from not only the hippocampus and the WCN19-1519 amygdala, but also the tumor factors and infectious diseases of CNS. In most cases craniocerebral trauma and tumors of temporal lobe Poster shift 02 - Autoimmune disorders - Part I/CNS infections - results in LTLE. Part I/dementia - Part I/epilepsy - Part I/health economics and doi:10.1016/j.jns.2019.10.1032 outcomes/neuroepidemiology and environmental and other

Lateralization parallels study in Uzbekistan: Comparison of changes between lateral and medial temporal lobe epilepsy WCN19-1518 ^ A. Sattarova, G. Rakhimbaeva Tashkent Medical Academy, Department of Neurology, Tashkent, Poster shift 02 - autoimmune disorders - Part I/CNS infections - Uzbekistan^ Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Objective We aimed to study the comparative changes between medial and Chorea, a rare manifestation of systemic lupus erythematosus. lateral temporal lobe epilepsy (MTLE and LTLE). Case report Method D. Rebolledo Garciaa, P.Ó. González Vargasb, D.L. Uriostegui Corderoa The study was based on the results of examination of 48 patients aHospital General Dr. Nicolas San Juan, Internal Medicine, Toluca, with medial and lateral temporal epilepsy, who were on a stationary Mexico examination in the department of neurology of the ﬁrst TMA clinic bHospital Materno Perinatal Monica Pretelini ISEM, Neurology Service, from 2016 to 2018. During Video-EEG-monitoring we recorded 114 seizures of various types. Toluca, Mexico^ ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 141

Results amplitude of the P300 component and signs of desynchronization The most signiﬁcant lateralizing features in this form of epilepsy of excitation in the visual cortex. For men, the average latent period were one-sided wrist automatism, a dystonic brushes and an of P100 was 117.9 ± 7.7 ms, for women - 111.2 ± 7.0 ms (normative unbalanced turn of the head. The most valuable lateralizing sings of data: 102.3 ± 3.2 ms). the epileptogenic focus for this form of epilepsy are the dystonic setting of the wrist (85.7%, p = .02), the inverting turn of the head Conclusions (88.9%, p = .02) and after-seizure aphasia (100%). Also for this The obtained results indicate that the presence of diffuse changes, group, the prevalence of localization of pathological disturbances in which were characterized mainly by local irritative and epileptiform the region of the anterior temporal electrode was found (75,0%, p = changes, can serve as electrophysiological criteria for HIV infection. .02 in comparison with the LTLE group), and in cases of presence of additional zones of localization - their registration in the contralat- doi:10.1016/j.jns.2019.10.1035 eral temporal lobe (63,6%, p = .02 in comparison with the LTLE group).

Conclusion WCN19-1529 Taking into account the revealed features of seizures, differential ^ diagnosis between lateral and medial forms of temporal epilepsy can be based on the lateralizing sings. Poster shift 02 - autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and doi:10.1016/j.jns.2019.10.1034 outcomes/neuroepidemiology and environmental and other

New features of the cerebral hemodynamics of brain arteries in patients with HIV-infection in Uzbekistan WCN19-^1527 A. Sabirova, G. Rakhimbaeva, S. Kuranbaeva Tashkent Medical Academy, Department of Neurology, Tashkent, Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Uzbekistan Part I/dementia - Part I/epilepsy - Part I/health economics and ^ outcomes/neuroepidemiology and environmental and other Objective We aim to study the cerebral hemodynamic indices of the brain New characteristics of neurophysiological changes in arteries in HIV-infected patients. neurocognitive disorders in HIV-infected patients in Uzbekistan Materials and methods A. Sabirova, S. Kuranbaeva, G. Rakhimbaeva 56 HIV-infected patients were examined, with veriﬁed serological Tashkent Medical Academy, Department of Neurology, Tashkent, and clinical diagnosis, whose average age was 35.7 ± 1.3 years, Uzbekistan ^ among which there were 31 (55.4%) men and 25 (44.6^%) women. The state of cerebral hemodynamics and vascular reactivity of the Objectives cerebral arteries was studied using standard techniques using We aim to study the features of electroencephalographic indices sensors of 2, 4, 8 MHz on the Spectromed-300 device (Russia). taking into account Р300 in the formation of early neurocognitive disorders. Results and discussion Cerebral hemodynamic disorders in patients according to the Methods Doppler study showed a decrease in blood ﬂow velocity in the ICA by EEG registration was provided out in 20 patients (11 women and 24.5 and 33.9%, in the MCA by 25.4 and 34.5%, in the PA - by 24.3 and 9 men) at rest and with functional tests, the cognitive evoked 44.7%, in OA - by 21.7 and 32.6% (groups 1 and 4, respectively) in potentials of the brain P300 upon presentation of visual and auditory relation to the indicators in the control group. There were also signs stimuli, measured the time of complex sensory-motor responses of an increase in vascular tone in all investigated vessels, according upon presentation of visual and auditory stimuli, were recorded to an increase in the pulsation index (Pi) and circulatory resistance visual evoked potentials during pattern stimulation. The average age (Ri), on average, 1.5 and 2.3 times in group 1; 1.8 and 2.75 times in 4 of patients was 35.2 ± 8.8 years. The number of CD4 lymphocytes in groups. blood plasma ranged from 230 to 859 cells / μl, the average was 451 ± 55 cells/μl. Conclusion An increase in vascular tone suggests the inadequate functioning Results of dilatatory mechanisms at the capillary bed level, which indicates Of the 18 patients in whom local changes on the EEG were stress in the mechanisms of adaptation of cerebral vessels, which, if recorded, involvement of the temporal areas was noted in 94% of not corrected, can lead to their breakdown and development of cases, the frontal-central areas - in 66% of cases, parietal-occipital - in dyscirculatory processes in the CNS in HIV-infected patients already 50% of cases. When registering visual evoked potentials in 75% of at the preclinical stages of the disease. cases, an increase in the latent period of the P300 component was observed on average from 10 to 30% with a decrease in the doi:10.1016/j.jns.2019.10.1036 ARTICLE IN PRESS

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

WCN19-1533 A. Khodabakhsh, S. Abdi ^ Iranian Center of Neurological Research- Shariati Hospital- Tehran University of Medical Sciences, Neurology, Tehran, Iran. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and Background outcomes/neuroepidemiology and environmental and other IncobotulinumtoxinA or Xeomin® is a new formulation of botulinum neurotoxin type A is commonly used as a therapeutic as Determining risk factors of developing adverse effects of antiep- well as aesthetic medication. This drug is recommended to be used ileptic drugs in epileptic patients immediately after reconstruction but may be applied with a delay due to economic justifications, however the efficacy of these two S. Mrabetab, K. Moallaa, A. Nasriab, I. Kacemab, M. Djebaraab, S. Aidlic, therapeutic options remains unknown. The present trial aimed to A. Gargouri-Berrechidab, R. Gouiderb assess the stability of Xeomin® and comparing baseline efficacy with aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia two week stored reconstructed drug. bUniversity of Tunis El Manar, Faculty of Medicine, Tunis, Tunisia cUniversity of Tunis El Manar, Faculty of Medicine- Department of Methods Pharmacology, Tunis, Tunisia This rater-blinded randomized clinical trial was performed on 20 healthy volunteers. Xeomin® (2.5 units) was randomly injected into Background and objectives EDB muscle of the foot. The remainder of the drug was kept in a Adverse effects (AE) of antiepileptic drugs (AEDs) are common in refrigerator at 4 °^C for 2 weeks and was injected in into the EDB daily neurological practice. Ensuring that an AE is attributable to an muscle of the opposite foot two weeks later. The compound muscle AE and predicting its occurrence remain a challenge. Our aim was to action potential Amplitude of extensor digitrum brevis (CMAP-EDB) investigate the predictors of AE of AEDs in epileptic patients. muscle was assessed at baseline as well as in second week, first month, and third month after injection and compared between the Materials and methods foots intervened. We conducted a cross-sectional study at the department of Neurology at Razi hospital (Tunisia), from January 2016 to June 2016. Results All epileptic patients on AEDs for at least 6 months during the study There was no difference in mean baseline CMAP amplitude and period were screened for inclusion. Demographic, clinical character- area between foots injected by fresh and refrigerated Xeomin® at istics, AED regimen and associated AE were recorded. Risk factors for the three time points of second week, first month, and third month developing AE were assessed. after injection. The Repeated measure ANOVA test also indicated no difference in the trend of the change in amplitude of CMAP (p = Results 189) and area (p = 390) across the two intervention protocols. We included 54 epileptic patients with AEDs-related AE were included. Sex ratio was 1.34. Mean age was 36.5 years and mean Conclusion disease duration was 19 years. About 46% were on monotherapy. The refrigeration time of two weeks seems not to reduce the Drug Valproate (72%) and Cabamazepine (44%) were the most frequently efficacy. prescribed AED regardless of regimen. Acute, dose-dependent reactions (neurologic (28.84%) and gastrointestinal symptoms (10.84%)) doi:10.1016/j.jns.2019.10.1038 were the most common AE, followed by hematologic idiosyncratic reactions (14.43%). Increased duration of the disease (p = .014), high frequency of initial seizures (p = .001), patients with interictal EEG abnormalities (P = .005), polytherapy (p = .009), and those receiving WCN19-1549 carbamazepine (p = .007) and high doses of valproate intake (p = .023) were found to be at higher risk of experiencing AE. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Conclusions Part I/dementia - Part I/epilepsy - Part I/health economics and Both disease characteristics and AED patterns were found to be outcomes/neuroepidemiology and environmental and other determinants in AE development. Clinicians should be aware of these factors when monitoring patients to minimize the risk and to Cerebral venous thrombosis and solid tumors improve compliance. F. Oukhai, M. Messelmani, A. Ouerdiane, M. Mansour, J. Zaouali, R. doi:10.1016/j.jns.2019.10.1037 Mrissa Military hospital for instruction of Tunis, Neurology, Tunis, Tunisia

Background and aims Cerebral venous thrombosis (CVT) associated with cancer are WCN19-^1534 rare. They are mainly associated with hematologic malignancies, and exceptionally with solid tumors. We aim to determine clinical Poster shift 02 - Autoimmune disorders - Part I/CNS infections - presentations, etiologies and outcomes of cancer-associated CVT in Part I/dementia - Part I/epilepsy - Part I/health economics and a single center in Tunisia. outcomes/neuroepidemiology and environmental and other Methods Assessing stability of Xeomin botulinum neurotoxin type A and A retrospective analysis was conducted, selecting patients comparing baseline efﬁcacy with two week stored reconstructed drug diagnosed with CVT and solid tumors at the neurology department ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 143 of the Military hospital of Tunis. Clinical characteristics, treatments, Patients with the early-onset seizures had signiﬁcant higher score and outcomes were reviewed. in m Rankin scale compared with late-onset seizures. Front- temporo-parietal location was the most common for generalized Results tonic-clonic seizures and focal seizures with impaired awareness. We selected 4 patients with solid tumors and CVT, representing Carbamazepine and valproid acid were the most common drugs used 8% of those diagnosed with CVT. The group comprised two men and in treatment of postroke epilepsy. two women, with a mean age of 55 years. Three patients presented with headache, one patient with status epilepticus. Three patients Conclusion were followed for cancer. One had a small-cell lung carcinoma with 1. Poststroke seizures most often have generalized tonic-clonic cerebellar metastasis and left transverse sinus compression. The character second had a breast cancer with a meningeal carcinomatosis. The 2. Early seizures are associated with a higher disability on the third had keratinizing squamous cell carcinoma of the vulva. These modiﬁed Rankin scale patients received chemotherapy and radiotherapy. Thrombosis of the 3. The fronto-temporo-parietal location of stroke increases the risk of upper superior sagittal sinus and right transverse sinus led to the focal seizure with impaired awareness and generalized tonic- diagnosis of small cell lung carcinoma with metastases in the fourth clonic seizures patient. All patients received anticoagulant treatment, with good neurological outcome in three patients. doi:10.1016/j.jns.2019.10.1040

Conclusion Solid tumors are a rare etiology of CVT. The underlying mechanisms can be direct or indirect. It is appropriate to look for WCN19-1573 cancer in case of CVT as an early diagnosis is essential to ensure optimal care. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - doi:10.1016/j.jns.2019.10.1039 Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

Asymptomatic varicella zoster virus infection presenting as WCN19-1564 Guillain-Barre Syndrome

M. Xifaras, D. Chousos, G. Limpitaki, A. Ziakka, E. Kerezoudi Poster shift 02 - Autoimmune disorders - Part I/CNS infections - "Pammakaristos" General Hospital, Neurology, Athens, Greece Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Guillain-Barre Syndrome (GBS) is a rare, serious, non-seasonal neurological disorder in which the immune system affects the Poststroke epilepsy-retrospective analysis peripheral nervous system, in most of the cases following a gastrointestinal or upper respiratory infection. Campylobacter jejuni, Myco- B. Paradowski, J. Chojdak-Łukasiewicz, E. Dziadkowiak plasma pneumonia, CMV, EBV, or HIV infections have all been associated Medical University Wrocław, Department of Neurology, Wrocław, with GBS. Varicella zoster virus (VZV) is a rare cause of GBS. We are Poland reporting a case of GBS following an asymptomatic VZV infection. A 64 year old male with history of hypertension presented with Introduction progressive limb weakness for 5 days. He had difficulty walking or The cerebrovascular disease is an important cause of epilepsy. The lifting his hands. He had no history of respiratory, gastrointestinal or incidence may significantly vary (from 2.3% to 43%). Post stroke other infection for the past month. He was afebrile, without blisters seizures occur within two weeks of stroke onset (as early onset or shingles on his trunk and neither had he exhibited them before. seizures) or after two weeks of stroke (as late-onset seizures). The The neurological examination revealed absence of tendon reflexes aim of this study was to evaluate clinical characteristics, including and flexor plantar reflex bilaterally. The patient had weakness in seizure type, radiological range of changes, modified Rankin scale upper and lower limbs (2/5). Sense of pinprick was impaired. Brain and treatment of patients with post stroke seizures. MRI scan showed chronic microvascular ischemic disease. Cervical/ thoracic MRI scan was normal. Nerve conduction studies were Methods compatible with acute inflammatory demyelinating polyneuropathy. We retrospectively analysed medical history of 164 adult patients CSF examination revealed elevated protein levels (96 mg/dL) and 5 with diagnosed post-stroke epilepsy hospitalized in Department of cells/mm3 (albumino cytological dissociation). Laboratory tests Neurology from 2012 to 2018. including ESR, CRP, C3, C4 etc. were negative. PCR for CMV, EBV, HSV, Mycoplasma pneumonia, Legionella etc. on CSF was negative. Results CSF PCR for VZV was positive. 87% of patients (143/164) were diagnosed with ischemic stroke, VZV is the cause of chickenpox and herpes zoster and may be 13% (21/164) had hemorrhagic stroke. The mean age of patients was severe for immunocompromised patients. Usually, GBS follows the 69,8 years. 86 (52%) patients were men. 101 patients developed reactivation of VZV, but the pathogenesis is poorly understood. generalized tonic-clonic seizures, 63 focal onset seizures (including 44 patients with impaired awareness). 17 patients had early onset doi:10.1016/j.jns.2019.10.1041 seizures, including 12 patients with ischemic stroke. ARTICLE IN PRESS

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

WCN19-1597 Introduction Dementia is a neurological disorder witch generally due to structural brain damage. It is often progressive and relatively Poster shift 02 - Autoimmune disorders - Part I/CNS infections - irreversible. Part I/dementia - Part I/epilepsy - Part I/health economics and Each year, 4.6 million new cases of dementia were predicted, with outcomes/neuroepidemiology and environmental and other numbers affected nearly doubling every 20 years to reach 81.1 million by 2040. These estimates were described as “provisional,” Subarachnoid neurocysticercosis, a severe presentation of the given that prevalence data were lacking in many world regions, and disease patchy in others.

I. Gonzalesa, J. Bustosb, S. Sánchezb, H. Saavedraa Aims aInstituto Nacional de Ciencias Neurológicas, Unidad de Cisticercosis, To assess the prevalence of dementia in the southern region of Lima, Peru; Tunisia. bUniversidad Peruana Cayetano Heredia, Unidad de Cisticercosis, Lima, Peru Methods We conducted a cross-sectional, descriptive study. This survey Introduction took place at the psychiatric department of the Regional Hospital of Neurocysticercosis (NCC) is a chronic parasitic disease of the Gabes. We included all patients, over 65 years old, who consulted for fi human nervous system, highly endemic in Latin America and other the rst time at the psychiatric ward of Gabes from 1 January 2011 to developing regions. A complicate extraparenchymal presentation is 31 December 2018. We used a pre-established form that explored subarachnoid NCC (SANCC), associated with late diagnosis and sociodemographic and clinical patients data. We used the Diagnostic difficult management. We report a retrospective study aimed to and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) to describe the characteristics of SANCC patients attended in our classify diagnostics. Results:We included 148 patients. Patients were institution between years 2008 and 2013 and its evolution up to men in 56.8% of cases with mean age of 76.4 +/- 8.6 years. The 2017. diagnosis of dementia was retained in 16% of cases including 13.5% Alzheimer-type dementia and 2.5% dementia secondary to vitamin fi Materials and methods B12 de ciency.Memory disorder was the most frequent reason for We identified 53 patients discharged with a diagnosis of SANCC consultation (30.2%), followed by agitation (17.3%), delirious and and who had at least one neuroimaging exam after discharge. They hallucinatory syndrome (13%) and sleep disorder (8.6%). were 27 females (51%) and 26 males, with a mean age of 42 years, ranging from 19 to 66 years. Their mean length with symptoms was Conclusion 40 months (standard deviation 53). The most frequent manifesta- Dementia is a disease that is becoming increasingly prevalent. tions were headache (48/49, 98%), nausea/vomiting (44/49, 90%9), Screening for its symptoms allows early diagnosis and can generalized seizures (31/49, 63%), and confusion (25/49, 51%). improvedquality of care. Serology was strongly positive in all cases and strongly reactive doi:10.1016/j.jns.2019.10.1043 (seven antibody bands) in 46 of 53 cases (87%). The most frequent locations were the suprasellar cistern (42/53, 79%), peri- mesencephalic (35/53, 66%) and posterior fossa (22/53, 42%). Parenchymal lesions were also present in 47/53 (89%). WCN19-1603 Conclusion SANCC is a severe form of NCC associated with a chronic course Poster shift 02 - Autoimmune disorders - Part I /CNS infections - and clinically manifesting with headache, intracranial hypertension Part I / dementia - Part I /epilepsy - Part I / health economics and and seizures. Complete resolution is obtained in a low proportion of cases, and mortality is still very high. outcomes / neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1042 Supra-nuclear dementia revealed by bipolar disorder: case report

A. Wafa, E. Emna, C. Bessem, A. Mouna, E.B. Maryem, G. Latifa aRegional Hospital of Gabes, Psychiatric Department, Tunisia b WCN19-1598 Regional Hospital of Gabes, Department of Neurology, tunisia, Tunisia

Introduction Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Bipolar disease is associated with a cognitive deﬁcit. The Part I / dementia - Part I /epilepsy - Part I / health economics and evolution of bipolar diseases towards a dementia syndrome is outcomes / neuroepidemiology and environmental and other possible and is known in the old terminology of "vesanic dementia". In this work we present the observation of a woman followed for Prevalence of dementia in a clinical population followed in resistant bipolar disorder and who subsequently develops dementia. psychiatry in the Southern of Tunisia Observation A. Mounaa, A. Wafaa, F. Dhouhaa, C. Bessema, E.B. Maryema, E. Emnab, This was a 48-year-old woman, with a personal history of G. Latifaa diabetes and vitiligo. Since 2015, she has started presenting thymic aRegional Hospital of Gabes, Psychiatric department, Tunisia episodes, frequent and resistant to treatment. The diagnosis of bRegional Hospital of Gabes, Department of neurology, Tunisia bipolar disorder was retained.The evolution was marked by the non- improvement and the appearance of several traumatic falls of its ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 145 own height. At the resumption of the interrogation, she reported the Conclusions notion of slurred speech and abnormal movements of the eyes Determination of the level of DHEA, as well as the reaction of Fe2 starting with the onset of psychiatric symptomatology. At the + can be taken as one of the biochemical markers for diagnosing psychiatric interview, she was in euthymia with sleep disorders, early forms of asthma, which can be recommended for screening without delirious activity. Neurologically, she had an unstable walk diagnostics of early forms of asthma. with a tendency to retropulsion, a fixed gaze with difficulty in mobilizing the eyes in the vertical and horizontal direction and an doi:10.1016/j.jns.2019.10.1045 extrapyramidal syndrome. The evolution was marked by the appearance of short-term memory disorders. Cerebral MRI showed atrophy of the midbrain and some puncthal signals in T2 and Flair of the non-specific cortico-subcortical white matter. The diagnosis of WCN19-1616 supra-nuclear dementia was retained.

Conclusion Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Recognition of cognitive impairment in the evolution of bipolar Part I / dementia - Part I /epilepsy - Part I / health economics and illness is important so as not to neglect these signs by calling them outcomes / neuroepidemiology and environmental and other hypochondriacal or phobic traits. Dementia is to be mentioned, especially in case of resistance to treatment. New web-approach epidemiology study of drug-resistant temporal lobe epilepsy (TLE) in Uzbekistan doi:10.1016/j.jns.2019.10.1044 A. Umarov, G. Rakhimbaeva Tashkent Medical Academy, Department of Neurology, Tashkent, Uzbekistan WCN19-1614 Objective Poster shift 02 - Autoimmune disorders - Part I /CNS infections - We reviewed data on the epidemiology of epilepsy in Uzbekistan and estimated the prevalence and incidence of drug-resistant Part I / dementia - Part I /epilepsy - Part I / health economics and temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) outcomes / neuroepidemiology and environmental and other based on extrapolation from the available data drawn from the literature. The significance of dehydroepiandrosterone sulfate (DHEA-S) in the diagnosis of early forms of alzheimer's disease in Uzbekistan Method We searched the electronic database PubMed, using the following A. Umarov, G. Rakhimbaeva, D. Tolibov, F. Yunusov search terms in the title: “epilepsy” OR “temporal lobe” OR Tashkent Medical Academy, Department of Neurology, Tashkent, “hippocampal sclerosis” AND “epidemiology” OR “prevalence” OR Uzbekistan “incidence.” Relevant original studies were included. We also reviewed several previous systematic reviews, meta-analyses, and Objectives their references. For hormones that affect the state of cognitive activity, the researchers include dehydroepiandrosterone (DHEA) and its Results sulfonated form - dehydroepiandrosterone sulfate (DHEA-s). Of We concluded that the estimated current Uzbek prevalence of particular interest is the relationship between DHEA-s and drug-resistant HS-TLE is 0.47–0.63 cases per 1000 people, and the Alzheimer's disease (AD). We aimed to establish the relationship of estimated Uzbek incidence is 2.8–3.0 cases per 100,000 people per indicators of the cognitive sphere with the level of DHEA-s in the year. Based on a Uzbek population of 33 million, we estimate that as early form of AD. many as 13900–17800 Uzbek patients still suffer from drug-resistant HS-TLE and are in need of surgery or other therapeutic options. Material and methods 50 patients (28 men and 32 women) under the age of 65 (mean Conclusion age 62.3±5.7 years) with the presence of cognitive impairment and Although full scale epidemiologic studies on drug-resistant HS- 5 healthy individuals were examined. The study was carried out TLE are needed, several observational studies allow adequate using enzyme immunoassay with the determination of the level of estimates of the range for the incidence and prevalence of this DHEA-c before and after oxidation with the catalyst Fe2+. condition. Given the morbidity and mortality associated with poorly controlled seizures, this relatively large and growing patient Results population is a matter of concern. Considering the variety of The patients with AD of the main group was characterized by the treatment options that are available or in the pipeline to treat presence of an organic neurological deficit and a progressive decline drug-resistant epilepsy, future efforts should focus on advocating for in cognitive functions. Neuropsychological studies on the MMSE early referral of patients with drug-resistant HS-TLE for more scale indicate the results of cognitive functions: 20.8±4.05 in the comprehensive epilepsy management. group of patients with AD, 27.1±0.8 in practically healthy individuals. Serum oxidation led to a sharp increase in DHEA levels in the doi:10.1016/j.jns.2019.10.1046 control group (to 5.2±0.5; after 7.1±0.5), while in the serum of patients with BA, the increase in level was either not observed or was insignificant ( up to 2.2±0.5; after 2.5±0.3). ARTICLE IN PRESS

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

WCN19-1621 S. Kuranbaeva Tashkent Medical Academy, Department of Neurology, Tashkent, Uzbekistan Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and Objective outcomes / neuroepidemiology and environmental and other To establish the role of natural AAT class IgG to nerve tissue proteins (S-100, GFAP, NF-200, and MBP) in the pathogenesis of Semantic dementia, an autopsy case with a clinical course of 17 damage to the nervous system in HIV. years Materials and methods M. Yamazaki, R. Sengoku, S. Murayama The level of autoantibodies to neurotropic proteins was deter- Tokyo Metropolitan Geriatric Hospital and Institution of Gerontology, mined in the serum of 99 patients with HIV infection. In the course of Neurology, Tokyo, Japan the study, four groups of patients were identified: HC lesions, conditionally not related to HIV, but caused by stress, toxic-allergic Introduction effects, the presence of somatic pathology, and other factors (48 Semantic dementia (SD) is a syndrome of progressive deteriora- patients, 48.5%; group 1); with primary lesion of the nervous system tion in semantic memory (knowledge of objects, people, concepts of HIV without significant immunodeficiency (primary neuro-AIDS - and words). SD is typically associated with frontotemporal lobar 9.1% (9 patients); group 2 - with secondary damage to the nervous degeneration with TDP-43 (FTLD-TDP) type C pathology. system and severe immunodeficiency (secondary neuro-AIDS - 22.2% (22 patients); Clinical summary A 72-year-old right-handed male retired from trading company, Results of the study with 16 years of education, came to neurological clinic complaining In clinically healthy individuals (control group), the determined of a 6-year history of difficulty understanding words and phrases. No parameters were distributed as follows: AAT S100 - 77.5 ± 7.6 cu, behavioural changes were reported. There was no relevant family AAT GFAP - 57.9 ± 5.7 cu, AAT NF- 200 - 72.9 ± 7.1 USD, AAT MBP - history. Neurological examination was normal. An initial MR brain 58.8 ± 5.5 USD scan showed marked anterior temporal lobe atrophy with left-sided The highest among the studied AAT in all groups was the level of predominance. The clinical picture suggested SD. Over the following AAT to S100, while in patients with HC lesions against HIV, this 9 years, he showed a gradual decline in semantic memory. At the age figure exceeded the standard values by an average of 1.9 times (P of 82, he was unable to speak anything. He died at the age of 83, 17 b0.01), and in patients without HC damage - 1.6 times (P b0.05). years after symptom onset. Informed consent was given for brain tissue examination before he died. Conclusion Thus, the clinical and immunological analysis revealed a clear Pathological summary pattern in the content of antibodies to neurotropic proteins The brain weighed 1415 g and appeared asymmetrical. There was depending on the presence of NS damage in HIV. marked temporal lobe atrophy with left-sided predominance, especially anterior inferior temporal gyrus. Frequent TDP-43 immu- doi:10.1016/j.jns.2019.10.1048 noreactive inclusions were present in the hippocampus, amygdala, entorhinal cortex and cerebral neocortex, with a lot of long dystrophic neurites. Pathological diagnosis was FTLD-TDP type C, which was also confirmed by the band pattern of C-terminal WCN19-1633 fragments of TDP-43 on western blotting of sarkosyl-insoluble fractions extracted from the frozen brain. The corticospinal tract Poster shift 02 - Autoimmune disorders - Part I /CNS infections - did not show degeneration. Part I / dementia - Part I /epilepsy - Part I / health economics and Conclusion outcomes / neuroepidemiology and environmental and other In the present case, clinical diagnosis was SD and pathological diagnosis was FTLD-TDP type C without corticospinal tract Quantitative content of neurospecific protein S-100 in neuro-aids degeneration. in Uzbekistan

S. Kuranbaeva doi:10.1016/j.jns.2019.10.1047 Tashkent Medical Academy, Department of Neurology, Tashkent, Uzbekistan

Objective WCN19-1631 To establish the role of neurospeciﬁc protein S-100 in HIV.

Materials and methods Poster shift 02 - Autoimmune disorders - Part I /CNS infections - The level of autoantibodies to neurotropic proteins was deter- Part I / dementia - Part I /epilepsy - Part I / health economics and mined in the serum of 99 patients with HIV infection. In the course of outcomes / neuroepidemiology and environmental and other the study, four groups of patients were identiﬁed: HC lesions, conditionally not related to HIV, but caused by stress, toxic-allergic The role of natural autoantibodies to the nervous system proteins effects, the presence of somatic pathology, and other factors (48 in neuro-aids in uzbekistan patients, 48.5%; group 1); with primary lesion of the nervous system ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 147 of HIV without significant immunodeficiency (primary neuro-AIDS - WCN19-1638 9.1% (9 patients); group 2 - with secondary damage to the nervous system and severe immunodeficiency (secondary neuro-AIDS - 22.2% (22 patients); group 3 - patients without damage to the nervous Poster shift 02 - Autoimmune disorders - Part I /CNS infections - system (20 patient, 20.2%; group 4). Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other Results of the study When evaluating the results of an immunological study, it was Evaluation of higher cortical functions of Alzheimer's disease in established that all groups of patients differed from the control Uzbekistan group, both in the level and in the degree of scatter of the neurotropic protein S100. In patients with HC lesions on the D. Tolibov, G. Rakhimbaeva background of HIV, this indicator exceeded the standard values by Tashkent Medical Academy, Department of Neurology, Tashkent, an average of 1.9 times (Pb0.01), and in patients without HC damage Uzbekistan — by 1.6 times (Pb0.05). Purpose Conclusion Evaluate the effectiveness of diagnostic disorders of higher Considering the fact that soluble Ca-binding protein of the cortical functions in patients with Alzheimer's disease using scales nervous tissue S100 is a trophic factor for serotonergic neurons, GDR (Global Deterioration Rating) and MMSE (Mini Mental Scale regulates the permeability of ion channels, the detection of Examination). autoantibodies to this protein is of great clinical importance in neurological practice and can be used as a marker of brain tissue Material and methods damage in patients with HIV. Study involved 82 patients (35 men and 47 women) aged 65 to 78 years (mean age 71,8 ± 3,3 years) divided into 2 groups: group 1 doi:10.1016/j.jns.2019.10.1049 (basic group) - Alzheimer's disease 45 patients, group 2 (comparison group) - 37 patients with chronic cerebral ischemia (II- IIIst.) with mild cognitive impairment. GDR and MMSE were used for assessing the severity of cognitive impairment. WCN19-1636 Results These neuropsychological studies indicate the results of cognitive Poster shift 02 - Autoimmune disorders - Part I /CNS infections - functions: in group I - GDR 5,0 ± 0,5 scores, MMSE 21,8 ± 4,05 Part I / dementia - Part I /epilepsy - Part I / health economics and scores. In group II - GDR 2,0 ± 0,5 scores, MMSE 27,0 ± 0,5 scores. outcomes / neuroepidemiology and environmental and other Senile dementia develops in commonly characterized by a relatively sparse confabulation products. Confabulation shifted to a more or Dementia risk and hypertension: A literature review less distant past ideas about the environmental situation and the self (amnestic confabulation). At the stage of mild dementia clearly G. Campbell, A. Jha identified the most features of amnestic aphasia, amnestic disorder Texila American University, College of Medicine, East Bank Demerara, component of praxis, and in some cases, signs of constructive Guyana dyspraxia. There is a long preservation of motor component of praxis. One of the main risk factors for brain vascular diseases in developing countries is hypertension. Moreover, a positive corre- Conclusions lation between cognitive decrease or dementia and the level of The total scores on the MMSE and the GDR is a sensitive indicator blood pressure was found in several epidemiological studies. The of cognitive deficits and higher cortical functions of mild to moderate results of most longitudinal studies have shown that the cognitive Alzheimer's disease before, is effective in determining therapeutic function often corresponds inversely to blood pressure values approaches and tactics of early prevention in patients with measured 15 to 20 years earlier. In the pathogenesis of vascular Alzheimer's disease. dementia cerebral infarction, lacuna, and change in the white matter are involved, but may also contribute to developing doi:10.1016/j.jns.2019.10.1051 Alzheimer's disease. The deteriorating cognitive function of hypertensive subjects has also been explicitly explained by microcirculation disorders and endothelial dysfunction. Recent therapeutic studies have paved the way for antihypertensive therapy to WCN19-1642 prevent dementia (vascular or Alzheimer type) and must be confirmed by other studies. This review paper focuses on the role of hypertension as a risk factor for cognitive impairment and Poster shift 02 - Autoimmune disorders - Part I /CNS infections - summarizes current knowledge on the relationships between mid- Part I / dementia - Part I /epilepsy - Part I / health economics and life hypertension and late-life dementia. outcomes / neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1050 Isolated neurosarcoidosis presenting with reduced consciousness: A case report ARTICLE IN PRESS

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

I. Saeed ResearchGate with no limitation of period until 2018. The terms of John Radcliffe Hospital, Medicine, Oxford, United Kingdom research were “epilepsy”“Cameroon”“epileptic seizures”“convul- sions” and regions of the countries. The patient had a background of myelodysplasia, atrial fibrillation and a posterior circulation infarct with acute bilateral Results vestibulocochlear failure 8 months prior to presentation with 2 We reviewed 20 publications but only 15 were used for this month decline manifesting as severe headaches then confusion review. The prevalence of epilepsy in Cameroon varies from 35.4 to proceeding to reduced GCS of 8 (E-1, V-2, M-5). 134.5‰. Children are mostly affected. There is a light male LP showed a lymphocytosis, a raised protein and low glucose. predominance, that could be explained by risk behavior in young Cytology showed increased lymphoid cells. No abnormal cells were male. Incidence of epilepsy varies from 98 to 222.7 per 100000 PY seen on cytospin. HSV PCR and the rest of the viral screen was especially in village closed to the Sanaga river which are endemic negative as well as TB Elispot and mycoplasma serology. zone of onchocerciasis. This Incidence is highly associated to Provisional diagnosis was acute disseminated encephalomyelitis. microfilaria density. This leads to a new entity called Onchocerciasis She received a 3 day course of Methyl prednisolone. associated epilepsy. Many studies reported etiologies of epilepsy: Serial brain imaging showed recurrent and progressive multifocal, poor pregnancy follow-up, CNS infections, stroke and head trauma. deep white matter and grey matter T2 hyperintensities with restricted The relative risk of mortality in 6.2 times for people with epilepsy diffusion, continuing to accumulate despite high dose steroids. compare to people without epilepsy due mainly to: status epilepticus Imaging was principally consistent with either a small vessel and SUDEP. Consequences of epilepsy include: stigmatization, vasculitis or an intravascular lymphoma, but was not diagnostic of exclusion, socioprofessional disabilities, burns and neuropsychiatric either or reliably discriminatory. disorders. Brain biopsy showed a florid non-necrotising granulomatous inflammation centred on the leptomeninges with focal parenchymal Conclusion involvement. The biopsy results suggested a diagnosis of neuro- Epilepsy is a major public health problem. Many causes and risk sarcoidosis. She was then commenced on Infliximab as well as factors could be identified and prevented. That is why we should further courses of steroids. improve awareness and advocacy. Since then, improvement has been modest but she has become more interactive and alert. Furthermore, a repeat MRI showed doi:10.1016/j.jns.2019.10.1053 significant reduction in contrast enhancement suggesting a response to treatment. Conclusion: isolated neurosarcoidosis is a challenging diagnosis and can mimic many conditions. Treatment with steroids and WCN19-1661 infliximab is associated with favourable outcomes. doi:10.1016/j.jns.2019.10.1052 Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other

WCN19-1646 The academic teleneurology evolution: A comparison of telestroke and general neurology inpatient consultations

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - M. Matiello, K. Mahler, J. Estrada, C. Whitney, A. Viswanathan, L. Part I / dementia - Part I /epilepsy - Part I / health economics and Schwamm outcomes / neuroepidemiology and environmental and other Harvard Medical School - Mass General Hospital, Neurology, Boston, USA Epidemiology of epilepsy in Cameroon: A review Introduction D. Gams Massia, M. Njankouo Yacoubaa, D. Jacquesb, K. Carolinea,K. The rising demand for access to emergency and inpatient Callixtec neurological expertise is explained by increased prevalence of aDouala General Hospital / University of Douala, Internal Medicine, neurological diseases and improved diagnostic and therapeutic Douala, Cameroon approaches. While the initial developments of teleneurology were bDouala Laquintinie Hospital / University of Douala, Neurology, Douala, focused on acute stroke care, consultations for other neurological Cameroon diseases further improve access and value. cDouala Laquintinie Hospital / University of Yaoundé 1, Neurology, Douala, Cameroon Goal To compare telestroke (TS) and routine-and-urgent general Background teleneurology (RUTN) consultations in terms of volume, modes of More than 10 million people with epilepsy live in Africa. Many communication and neurologist satisfaction. patients in Sub-Saharan Africa do not received appropriate treatment due to socio-economic and cultural factors. Cameroon has one of the Methods highest prevalence in the world. Records of consults (n=3657) provided by the Massachusetts General Hospital Teleneurology Service from January-December Methods 2018 were obtained from our teleneurology database. We compared We systematically reviewed research articles, original papers, the rate of consults to each service line, frequency of video consults abstract on PubMed, Google Scholars, African Journals Online (AJOL), and neurologist's satisfaction. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 149

Results dominated CNS involvement in 9 cases and PNS involvement in 2 TS consultations were provided to 21 hospitals, 8 of them are also cases. contracted to receive RUTN consults. A total of 1646 consults were Discussion: done through TS (incidence rate 102.9) and 2011 through RUTN The wide range in the prevalence of neurological manifestations (incidence rate 251.4). The incidence rate difference between the in connective tissue diseases reveals the necessity of clear decision two services was 148.5 (95% CI 138.1-158.9, pb0.0001). The algorithms for the association of neurological symptoms with a proportion of video consults in telestroke was 27% and in RUTN systemic autoimmune disease b 10% (p 0.0001). In a 5-point Likert scale, neurologist's satisfaction Conclusion: mean score was 4.67 in TS vs 4.69 in RUTN (p=0.39). Recent studies identiﬁed potential paraclinical markers for the diagnosis of nervous system involvement in connective tissue diseases Conclusion Out study suggests that there is a large demand for inpatient doi:10.1016/j.jns.2019.10.1055 general teleneurology consultations through an academic-based teleneurology program. Use of video consults are more frequent in telestroke and the neurologist satisfaction with the TS and RUTN was similar. The combination of teleneurology and telestroke meet an important and ever-growing need of access to neurology specialty WCN19-1668 care. Poster shift 02 - Autoimmune disorders - Part I /CNS infections - doi:10.1016/j.jns.2019.10.1054 Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other

An update of neurological involvement in gougerot sjogren WCN19-1664 syndrom: diagnosis challenge and moroccan team experience

Poster shift 02 - autoimmune disorders - part i /cns infections - S. Sabiry, S. Louzi, S. Bellakhdar, B. El Moutawakil, H. El Otmani, M.A. part i / dementia - part i /epilepsy - part i / health economics and Rafai Ibn Rochd University hospital, Neurology-Neurophysiology, Casablanca, outcomes / neuroepidemiology and environmental and other Morocco

Neurological manifestations revealing connective tissue diseases, Introduction from central to peripheral nervous system: diagnosis challenge Neurological manifestations in Gougerot-Sjogren syndrome (GSS) are valued differenly. This is essentially the achievement of the S. Sabiry1, R. amzil1, S. bellakhdar1, H. el otmani1, B. el moutawakil1, peripheral nervous system. The frequency of neurological manifes- M.A. rafai1 tations varies according to the studies (10-70%) but is on average 1Ibn Rochd University hospital, Neurology-Neurophysiology, CASA- between 15 and 25%. BLANCA, Morocco Patients and methods Introduction: We report a series of 21 cases of neurological manifestation Connective tissue diseases (CTD) can present with a wide revealing a GSS collected over a period of 12 years.GSS diagnosis was spectrum of neurological symptoms, involving both the central retained according to Americano-European group criteria consensus (CNS) and peripheral nervous system (PNS) revealing the disease. on 2002. The aim of this study is to determine the frequency the clinicoradiological, biological and the outcome of nervous system involve- Results ment in CTD 95.2% of cases were female with a sex ratio of 0.05 with an Patients and Methods: average age of 42.3 years. Neurological manifestations was revealing A retrospective study of patients collected over a period of 12 in 95.2% of cases. Central involvement was noted in 9 cases with years (January 2005-December 2016). All of this patients were encephalic involvement in 6 cases, there was encephalic involve- evaluated based on currently used criteria. ment associated with myelopathy in 2 cases and cranial nerve Results: involvement associated with encephalic involvement in one case. 104 cases neurological manifestation revealing CTD were found Peripheral nervous involvement was noted in 7 cases, with The average age was 38,4 years with a female predominance (sex sensitivomotor polyneuropathy in 3 cases, the sensitive neuropathy ratio= 0,22) in one case, isolated involvement of the cranial nerves in 2 cases and Neurological signs revealed sarcoidosis in 28 patients, dominated multiplex mononeuropathy in 1 case. Peripheral nervous involve- by brain localization in 20 patients followed by medullary involvement associated with central involvement was noted in one case.An ment, cranial nerves, muscle and peripheral nerves. Neuro-Behect’s association with other neurological conditions was noted in 4 cases disease was reported in 26 cases dominated by Rhombencephalitis and cerebral venous thrombosis with 44.7% and 23.7% respectively. Discussion Neurological manifestations revealed Gougerot-Sjogren syndrome In our study we found a concordance of the proﬁle of the in 21 cases dominated by CNS involvement in 9 cases and patients, with the literature.Central neurological involvement was PNS involvement in 7 cases. Neurological manifestations of predominant compared to peripheral nervous involvement, con- antiphospholipid syndrome was reported in 18 cases dominated by trary to the literature. In terms of peripheral involvement, cerebrovascular events in 7 cases followed by PNS and SEP like sensitivo-motor polyneuropathy was the most frequent in our manifestations. Neurolupus diagnosis was retained in 11 cases series. ARTICLE IN PRESS

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

Conclusion Key words: Collaboration, Epilepsy, Neurologist, General The neurological manifestations related to GSS are varied and Practitionner, Burkina Faso difﬁcult to diagnose because they often occur before the diagnosis of GSS doi:10.1016/j.jns.2019.10.1057 doi:10.1016/j.jns.2019.10.1056

WCN19-1675

WCN19-1673 Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and Poster shift 02 - Autoimmune disorders - Part I /CNS infections - outcomes / neuroepidemiology and environmental and other Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other Cognitive impairment in ankylosing spondylitis patients: A case- control study Collaboration between neurologists and general practitioners concerning epilepsy care in Burkina Faso E. Suriano, A. Beatriz Pereira de Souza, B. Kusznir Vitturi, D. Yukito Torigoe Santa Casa de São Paulo Medical School, Department of Rheumatology, Y.T.M. Lenganea, A. Millogob, N. Kissanic, P.B. Somed, M.A.J. Kyelema, São Paulo, Brazil C. Napone, J. Kaborea aUniversity teaching hospital Yalgado Ouedraogo, Neurology, Ouaga- dougou, Burkina Faso Background fl bUniversity teaching hospital Souro Sanou, Internal medicine, Bobo Ankylosing spondylitis (AS) is a chronic in ammatory disease Dioulasso, Burkina Faso that affects vertebrae and sacroiliac joints. Studies sugest that cUniversity hospital Mohamed VI, Neurology, Marrakesh, Morocco neurological manifestations might be more frequent than expected dUniversité saint Thomas d'Aquin, Public Health, Ouagadougou, Burkina in autoimmune rheumatological diseases. Faso eUniversity teaching hospital of Bogodogo, Neurology, Ouagadougou, Objectives Burkina Faso Assess frequency and clinical predictors of cognitive impairment in AS patients. Background and purpose Methods Collaboration between practitioners, especially between Cross-sectional and case-control study, including consecutive AS neurologists and general practitioners in the management of patients seen in tertiary hospital. Control group included 33 healthy epilepsy in Burkina is few documented. The quality of this subjects. We registered clinical and demographic data. Functional collaboration is important for a proper management. Our aim was capacity was assessed using HAQ. Neurological appraisal was made to describe the general practitioner / neurologist relationship in with standardised questionnaires: BCSB, MoCA, CDR and HAD. epilepsy care. Analysis of the data was performed using qui-square, t-tests and multivariate analysis (SPSS 22.0). Significance level was set as b0.05. Methods 9 neurologist and 31 GP were assessed, from December 21th 2018 Results to January 31th 2019 in Burkina Faso. It was a trans-sectional We included 35 patients (17 female) with a mean age of 53.1 qualitative study by performing auto-questionnaires. (±13.9) years; almost all were under biologic therapy. Mean age of AS diagnosis was 41.9 (±13.9) years. In univariate and multivariate Results analysis, patients with AS presented significant lower BCSB, MoCA We surveyed 40 doctors including nine neurologists and 31 GPs. and CDR scores (pb0.05). Adjusting for level of education, just 12 and According to the GPs, the frameworks of concertation exist in 77.42% 8 patients presented normal BCSB and MoCA scores (pb0.01), of the cases. They are often quarterly and the question of respectively. Most affected domains were attention, delayed recall collaboration was sometimes mentioned. The country's reference / and executive functions. Cognitive decline was associated with counter-referral system was unknown from 09.63% of GPs and 2/9 higher HAQ scores (functional outcome due to AS) (pb0.05) but neurologists. GPs never received any counter-referral from neurol- not with prolonged time of disease. No correlation was found ogists in 90.32% (n = 28) of cases. Among neurologists 7/9 think that between sex, disease-modifying antirheumatic drugs, HLA-B27, C- GPs can diagnose epilepsy. Half of the GPs find that neurologists are reactive protein levels and the neurological impairment. Mean HAD not available. The attitude of neurologists is perceived as fraternal by score was 12.4 (±8.7) and anxiety and depression were more the majority of the GPs. According to them, the collaboration is quite prevalent in AS patients than in control group (pb0.01). good and moderately appreciated.

Conclusion Conclusions Clinicians must be aware that patients with AS may have neurological Concerning epilepsy care in Burkina Faso, GPs perceive the manifestations of the disease. Further studies are encouraged. collaboration with neurologists as average. According to the majority of generalists the counter-referral from neurologists are never doi:10.1016/j.jns.2019.10.1058 received. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 151

WCN19-1684 aZambrano Hellion Health Center- Tecnológico de Monterrey-NL- México, Neurology and Neurosurgery Institute, Monterrey, Mexico bUniversity Hospital- Dr. Jose Eleuterio Gonzalez, Neurology, Monterrey, Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Mexico Part I / dementia - Part I /epilepsy - Part I / health economics and cChristus Muguerza Hospital, Internal Medicine, Monterrey, Mexico outcomes / neuroepidemiology and environmental and other dUniversity Hospital- Dr. Jose Eleuterio Gonzalez, Infectology, Monterrey, Mexico Limbic encephalitis as the presenting feature of sjögren syndrome Case 1 24-year-old female,healthy,nurse,arrives with 72 hrs of an J.I. Castro Macias intense headache,fever 40 ºC,photophobia,drowsiness,vomiting. Hospital Regional de Alta Especialiad del Bajio, Department of Physical examination:Tachycardia,neck stiffness,meningism signs. neurosciences, Leon- Gto., Mexico Lumbar puncture was performed reporting glucose 54mg/dL,Proteins 44mg/dl, Leukocytes 462mm3,PMN 60%.Lactate 9.7,negative Chinese Background ink in CSF.Treatment with ceftriaxone/vancomycin was started.On rd Autoimmune encephalitis associated with serum and intrathecal the 3 day Neisseria meningitidis was isolated in a blood culture. antibodies against intracellular and surface neuronal antigens of the Transcranial Doppler ultrasound (TCD) was performed on the third limbic system neuropiles, and several neuropsychiatric and day of treatment, documenting peak systolic velocity (PSV) of 148 neurocognitive disorders have shared mechanisms of origin. Sicca cm/sec in left MCA and pulsatility index(PI) 1.22,dexamethasone was th syndrome is present in only 50% of patients with neurologic started at 30 mg/day. 5 day PSV 120.6cm/sec/ PI 2.24 in left MCA, th involvement. However, limbic encephalitis (LE) has been recognized PSV120 cm/sec,PI of 2.15 in right MCA. 7 day with dexamethasone, on occasion as a feature of Sjogren’s syndrome (SS). PSV of both MCAs were normal. Patient received antibiotic therapy for 14 days. Case A 40-years-old male patient presented with sudden development Case 2 of headache, episodic memory failure, focal cognitive epileptic and 63-year-old female, with no past medical history arrived with 48 generalized seizures, fever, complex visual hallucinations, fluctua- hrs of evolution with intense headache,fever 39 ºC,stupor,neck tions in awareness. Neurological examination showed drowsiness, as stiffness and right hemiplegia,conjugate gaze deviation to the left, well as high freceuncy generalized tremor at rest. MRI showed an babinski and chadodck signs bilateral presents. MRI shows left area of increased signal on fluid-attenuated inversion recovery frontal hyperintensity in T2 Flair.Lumbar puncture was performed (FLAIR) sequence bilaterally in the temporal lobe. CSF analysis reporting Glucose 31mg/dL,Proteins 178mg /dl,Leukocytes 3575 showed a high protein level; viral screening and bacterial cultures, mm3,PMN 99%.PCR in CSF Neisseria-meningitidis is detected. Treat- paraneoplastic antibody panel, anti NMDA were negative, EEG ment with ceftriaxone/vancomycin is started. TCD was performed on showed bilateral temporal focal slowing, PET scan was negative for the third day of treatment, documenting PSV of 140 cm/sec in left th malignancy, Anti Ro / SSA and anti La / SSB antibodies were elevated, MCA and PI 2.06, initiating dexamethasone at 30 mg/day. 5 day PSV Schirmer test was positive for reduced tear production and minor 177 cm/sec/ PI 2.11 in left MCA, PSV 187 cm/sec,PI of 1.49 in right th salivary gland biopsy demonstrated focal lymphocytic sialadenitis. MCA. 8 day of treatment with dexamethasone, PSV of both MCAs Treatment with azathioprine and prednisone was started as well as were normalized. Antibiotic was given for 21 days. rituximab every 6 months with marked improvement shown by lack of seizure recurrence; However, episodic memory failure remained. Conclusion This report aims to document the usefulness of TCD as a follow- Conclusion up method in the decision to use steroids at high doses. Recognizing the association between MRI temporal lobe lesions and LE should prompt further evaluation for paraneoplastic and doi:10.1016/j.jns.2019.10.1060 autoantibody-related entities, including SS. This is the first case where the association of neuropsychiatric symptoms is described between LE and SS. WCN19-1711 doi:10.1016/j.jns.2019.10.1059

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and WCN19-1686 outcomes / neuroepidemiology and environmental and other

Serial case study and literature reviewed for best management of Poster shift 02 - Autoimmune disorders - Part I /CNS infections - cerebral amyloid angiopathy (CAA) in regard: antiepileptic and Part I / dementia - Part I /epilepsy - Part I / health economics and cognation related outcomes / neuroepidemiology and environmental and other A. Elrekaby, E. Dakneviciute Monitoring of cerebral vasculopathy by transcranial doppler Stroke, Medicne, Colchester, United Kingdom ultrasonography as a guide for the therapeutic use of steroids in neisseria-meningitis series of cases in the hispanic population Aim We evaluated the best practice in management of cerebral L. Olguina, J.E. Garcia-Berlangaa, F. Flores-Alfarob, E. Puron-Gonzalezc, amyloid angiopath (CAA) in regard: best to treat seizure and the H. Martineza, F. Gongora-Riveraa, A. Camacho-Ortizd relation between cognition decline and CAA. ARTICLE IN PRESS

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

Method Results We presented serial of 4 interesting cases who presented to out There was no change in all cases of OMDS before and after stroke service/ unit in the last two years. We screened each abstract treatment. In Modified Aschworth Scale (MAS), 5 out of 10 patients and full-text article for inclusion, abstracted data, and applicability, with spasticity of lower extremities showed 1 stage of improvement and graded evidence. Data sources. We searched PubMed and the within 1 week. In 10m walking test. There was no obvious Cochrane Database published up to August, 2018. improvement in the number of the steps, but the time became shorter by 10% or more in 5 patients. There is no specific trend in Result OABSS. The value of neopterin of CSF decreased 12.1 pmol/ml from We reviewed 8 studies, including 117 cases to understand better 15.2 pmol/ml. seizure management in CAA. Most studies researched steroid effect on CAA-ri, and in suspicion of CAA or CAA-ri patients seem to Conclusion improve on steroids not even in their seizure activity, but also in The effectiveness of oral administration of L-arginine was speech problems, confusion, general fitness, and radiological suggested in walking disorder and dysuria in the chronic phase of changes.We could not find any evidance of specific antiepileptic HAM/TSP. medication in those cases. To better understand the connection between dementia and CAA doi:10.1016/j.jns.2019.10.1062 we reviewed 24 articles, including 5342 cases. We find that the cognitive decline more steep in patients who have more than 2 microbleeds and another study noted impairment only significant if more than 5 microbleeds WCN19-1718

Conclusion We find finding in regard antiepileptic, there was no clear Poster shift 02 - Autoimmune disorders - Part I /CNS infections - superiority or specific agent that should complete benefit, neverthe- Part I / dementia - Part I /epilepsy - Part I / health economics and less, steroid can be considered. There is reasonable risk to develop outcomes / neuroepidemiology and environmental and other cognitive impairment and dementia in CAA. The survey of autoimmune paraneoplastic neurological syn- doi:10.1016/j.jns.2019.10.1061 dromes in the Southern Moravia region of the Czech Republic during 2005-2018 period

P. Stouraca, J. Bednarovab WCN19-1717 aUniversity Hospital Brno, Neurology, Brno, Czech Republic bUniversity Hospital Brno, Microbiology, Brno, Czech Republic Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Objective Part I / dementia - Part I /epilepsy - Part I / health economics and The survey of autoimmune paraneoplastic neurological syn- outcomes / neuroepidemiology and environmental and other dromes (PNS) analyzing the changing profile of antibodies and syndromes in the context of improving laboratory diagnostics and fi A clinical trial evaluating the safety and ef cacy of oral L-arginine clinical suspicious index in the Southern Moravia region of the Czech therapy in patients with HTLV-1-associated myelopathy/tropical Republic during the period of 2005-2018 is presented. spastic paraparesis Background E. Matsuura Autoimmune PNS belong to the group of rare diseases with still Kagoshima University Graduate School of Medical and Dental Sciences, limited knowledge about their prevalence and profile in different Department of Neurology and Geriatrics, Kagoshima, Japan countries.

Background Methods HAM/TSP is a chronic inflammatory neurological disease caused Forty one patients were diagnosed for antibodies using immuno- by HTLV-1 infection. Safe and cheap medicines are required for the blot (Ravo Diagnostics Germany) and IIFT Autoimmune Encephalitis lifelong treatment of HAM/TSP. We studied about the safety and Mosaic 1 (Euroimmun,Germany) efficacy of oral L-arginine administration. Results Methods We diagnosed 15 patients positive for onconeural anti - Hu Among the patients with HAM/TSP who satisfied the Japanese antibody; 11 patients for anti-Yo and 2 patients for anti - Ri. Anti - diagnostic guidelines, patients with a degree of Osame’s Motor Hu positive patients presented with sensory neuronopathy, Disability Score (OMDS) of 6 or less and taking steroid drugs not brainstem encephalitis, limbic encephalitis, encephalomyelitis and more than 10 mg/day in terms of prednisolone are included. cerebellar syndrome. Anti -Yo positive patients had cerebellar Eleven subjects are participated in this study and the average syndrome with additional mild signs. Two anti - Ri positive patients age is 70.0 years. L-Arginine hydrochloride granules were orally had brainstem encephalitis. Two of anti-CRMP5 positive patients had administered for 20 days at 7 g / day. Clinical assessment cerebellar ataxia and polyneuropathy and 1 patient with anti- Ma2 including 10 m walking test, 3 m Time up and go test, Modified antibodies presented encephalitis. Seven patient with anti-NMDAR Ashworth Scale (MAS) and overactive bladder questionnaire antibodies presented predominantly psychiatric symptoms and (OABSS) and blood test were conducted before and after the dysautonomia.. Two patients were diagnosed with Zic 4 antibodies. administration. The profile of tumors was reviewed. One atypical case of limbic ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 153 encephalitis without onconeural antibodies and chronic lymphatic R. Fajar leukemia is reported Yogyakarta State University, Mathematics, Yogyakarta, Indonesia

Conclusions Alzheimer's disease is indeed difﬁcult to detect at the beginning Results of this survey characterize autoimmune PNS in Southern of an attack because it resembles ordinary senility, but gradually Moravia of the Czech Republic emphasizing the prevalence,long - the ability to think and the motoric abilities of sufferers decreases. term course and treatment outcomes. These results can serve as a The peak, sufferers can no longer think normally. In the end the reference for diverse population studies worldwide. sufferer can experience death after several years because his motoric ability is not functioning. As a solution, technology doi:10.1016/j.jns.2019.10.1063 can help in solving existing problems as early as possible. By using technology information and computers, doctors and paramedics are expected to be able to detect the presence of Alzheimer's disease earlier quickly and easily. One technology that can be used is the WCN19-1721 Expert System. This expert system will collect the necessary information, then the existing information is processed in a predetermined rule and then draw conclusions from the data Poster shift 02 - Autoimmune disorders - Part I /CNS infections - obtained. An expert system prototype for the early diagnosis of Part I / dementia - Part I /epilepsy - Part I / health economics and Alzheimer's disease, which the author developed is a model to outcomes / neuroepidemiology and environmental and other support diagnosis decision making. By using Fuzzy Tsukamoto calculations the results of accuracy are high so that it can help Biometry of brain and corpus callosum from MID sagittal MRI doctors make decisions for patients. The author has tested 25 images in patients with history of epilepsy patients diagnosed samples obtained an accuracy of 0.92. This shows that Fuzzy Tsukamoto has a high level of accuracy to F. Al-Marria, M.F. Rezab,M.Aﬁq Bin Mahayidinb, T. Begumb, Z. Idrisb diagnose this disease. As conclution, Fuzzy Tsukamoto's calculation aKing Faisal University, Neuroscience, Al-Ahsa, Saudi Arabia technique is proven to be able to solve the problem of diagnosing bUniversiti Sains Malaysia, Neurosciences, Kota Bharu, Malaysia Alzheimer's disease quickly and accurately.

The previous studies demonstrated that the epilepsy patients doi:10.1016/j.jns.2019.10.1065 showed higher mid-callosal width, smaller corpus-callosal area and volumetric reduction of corpus-callosum, but this study focused on any changes in the callosal-to-brain ratio of subjects with epilepsy when compared to control. The sample population are patients that had their WCN19-1736 mid sagittal MRI image of their brain taken during their visit to Hospital Universiti Sains Malaysia (HUSM). Using the medical history available Poster shift 02 - Autoimmune disorders - Part I /CNS infections - from the database, sample for patients with history of epilepsy (n=68) Part I / dementia - Part I /epilepsy - Part I / health economics and were selected. For the control group (n=68), they are age and gender matched, nominated from those who have radiologically normal MRI outcomes / neuroepidemiology and environmental and other images in the database but without the history of epilepsy. Three individual ratters were measured the anterior-posterior (AP) length of Neuroleptic malignant syndrome in anti-nmdar encephalitis – A the brain and the corpus-callosum followed by a callosal-to-brain (CB) phenomenon of delayed recognition and psychotropic drug ratio from their mid sagittal MRI images. Each group consists of 30 males resistance and 38 females with a mean age of 25.6±15.3 years. One ratter did not show significant differences in the AP brain length, the AP callosal length M.M. Señga, J.P.B. Reyes and the CB ratio between epilepsy and non-epilepsy group. But other The Medical City, Adult Neurology, Pasig City, Philippines two ratters verified that the AP length of the corpus-callosum and CB ratio are significantly decreased in the epilepsy group compare to non- epilepsy group. Consistency analysis using two-way mixed model, the Introduction average measures of Intraclass Correlation Coefficients (ICC) for inter Anti-N-Methyl-D-Aspartate receptor (Anti-NMDAr) encephali- rater reliability was 89.5% (p=0.000). The results suggest that the tis is an autoimmune disease that is often confused for callosal-brain ratio may be a potential additive biomarker for epilepsy psychiatric disorders resulting in late recognition. It presents with prediction which is essential to elucidate further. a constellation of psychiatric/behavioral changes, seizures, autonomic instability. It may be associated with the finding of an doi:10.1016/j.jns.2019.10.1064 ovarian teratoma. When unrecognized, patients are treated with antipsychotics due to the initial presentation of psychiatric complaints.

Patient WCN19-1723 We report a 43-year old nulliparous female whose presentation at the ER was suggestive of Neuroleptic Malignant Syndrome (NMS) Poster shift 02 - Autoimmune disorders - Part I /CNS infections - –fever, rigidity and elevated creatine kinase in the background of Part I / dementia - Part I /epilepsy - Part I / health economics and neuroleptic medication intake. However, it was preceded by a outcomes / neuroepidemiology and environmental and other subacute course behavioral changes, mutism and seizures which cannot be accounted for by NMS alone. Work up excluded infectious and metabolic causes. Imaging was unremarkable. CSF studies Applied artiﬁcial intelligence as expert system for diagnosis of revealed antibodies to NMDAr. Alzheimer's disease using fuzzy sukamoto ARTICLE IN PRESS

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

Discussion WCN19-1747 Anti-NMDAr encephalitis presents with a subacute course of psychiatric complaints and are often treated with antipsychotics. Later in the course, seizures, decreasing sensorium and, dyskine- Poster shift 02 - Autoimmune disorders - Part I /CNS infections - sias arise. In rare instances, patients develop adverse reaction to Part I / dementia - Part I /epilepsy - Part I / health economics and antipsychotics and becomes a cause of emergency department outcomes / neuroepidemiology and environmental and other consult. Anti-NMDAr Encephalitis may be confused with NMS. However, it may also co-exist at the same time. With timely Immune haemolytic anaemia a rare but important complication recognition, complications may be prevented and immunotherapy associated with intravenous immunoglobulin therapy initiated. Caution must be taken when giving antipsychotics. A. Sonia, N. Sonib Keywords: Anti-NMDAr, Anti-NMDAr encephalitis, Autoimmune aNeurology, Aurangabad, India encephalitis, Neuroleptic malignant syndrome, NMS bMGM Medical college and hospital, General medicine, Aurangabad, India doi:10.1016/j.jns.2019.10.1066

Intravenous Immunoglobulin (IVIG) is used frequently in treatment of AIDP and CIDP. The incidence of IVIG associated haemolysis is 1/1000 IVIG WCN19-1746 treatment episodes. The IVIG haemolysis pharmacovigilance group defines IVIG Poster shift 02 - Autoimmune disorders - Part I /CNS infections - associated haemolysis if episodes occur within 10 days of adminis- Part I / dementia - Part I /epilepsy - Part I / health economics and tration of IVIG. outcomes / Neuroepidemiology and environmental and other We report a case of 27 year male, who developed subacute weakness of lower limb followed by upper limbs and neck muscles with areflexia and truncal weakness.Clinically and electrophysiolog- High incidence of severe neurologic manifestations and mortality ically was diagnosed as GB syndrome (Acute motor axonal in adults with listeria monocytogenes meningitis in Taiwan: A polyneuropathy). In view of neurological deterioration and worsen- hospital-based study ing of limb weakness was started on IVIG therapy 0.4g/kg/day for 5 days. S.Y. Chen, W.N. Chang After fifth day of treatment patient was found to be icteric Kaohsiung Chang Gung Memorial Hospital, Neurology, Kaohsiung City, clinically and pale but neurologically was static. His lab revealed T.bil Taiwan, ROC of 5 and indirect 3.6. Liver enzymes were mild deranged. His LDH was high and ICT/DCT was negative, viral markers were negative and Background USG abdomen was normal. Hb dropped and output decreased. He The clinical characteristics of Listeria (L.) monocytogenes menin- was started on hydration and B12 and folic acid supplements and gitis are uncommonly reported solely in the literature. serial monitoring of LFT and Haemoglobin under guidance of Haematologist. His Hb dropped from 15 to 7 but bilirubin improved Methods returned to normal. Based on investigation and haematology opinion The clinical and laboratory features of patients with adult and with review of literature possibility of ivig induced haemolytic fi bacterial meningitis (ABM) identi ed in the study period of 2000- anaemia was considered. 2018 were collected and those with L. monocytogenes meningitis Conclusion –IVIG induced haemolytic anaemia is rare but were enrolled for study. important complication of IVIG therapy. 1)It depends on Blood group of IVIG recipient, (A,B and AB) are more likely to develop Results haemolytic transfusion syndrome. 2) We should Monitor Totally, 11 adults with L. monocytogenes meningitis were enrolled Haemoglobin post IVIG therapy. 3) Haemolysis also depends on rate fi and three of them were identi ed in 2000-2010 and the other eight, of transfusion. 2011-2018. They were 5 men and 6 women, aged 47-76 years. None of them had a postneurosurgical condition as the preceding event, doi:10.1016/j.jns.2019.10.1068 and 8 of them belonged to community-acquired infection. Of these 11 patients, only three of them had the diagnosis of meningitis b 24 hours of symptom onset. The major clinical manifestations of these 11 patients included fever (11), altered consciousness (8), seizure (8) WCN19-1750 and hydrocephalus (5). Using adequate dosage of penicillin G or ampicillin as the empiric antibiotic was found in three patients only. The therapeutic result showed a mortality rate of 72.7% (8/11) and 2/ Poster shift 02 - Autoimmune disorders - Part I /CNS infections - 3 (66.7%) of the survivors had severe neurologic deficits. Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other Conclusion This study revealed an increased incidence of L. monocytogenes Mathematical model and numerical simulations of chemotaxis in infection in ABM in recent years, and the involved victims had severe Alzheimer disease neurologic manifestations and poor therapeutic outcome. R. Fajar, P. Jupri doi:10.1016/j.jns.2019.10.1067 Yogyakarta State University, Mathematics, Yogyakarta, Indonesia ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 155

Alzheimer's dementia is a disease caused by senile plaques as WCN19-1758 results of accumulation of β-myloid protein which are production too high. This process also causes the activation of microglia cells or inflammatory, microglia consequently will move toward the source Poster shift 02 - Autoimmune disorders - Part I /CNS infections - of β-amyloid, this event is called chemotaxis. Chemotaxis events Part I / dementia - Part I /epilepsy - Part I / health economics and have been formulated into a mathematical model by Keller-Segel in outcomes / neuroepidemiology and environmental and other 1970 and later developed in the case of Alzheimer's disease by M. Luca in 2001. In this research the model is developed and numerical Neurosarcoidosis IN a filipino-chinese with recurrent solutions were analyzed by the finite difference method. The results craniopathies and peripheral neuropathies responding to ste- obtained indicate that the concentration of β-amyloid and microglia roids: A case report will be homogeneous for a long time. The concentration or density of microglia cells in nerve cells that experience Alzheimer's disease will L.G. Garcia tend to converge to low concentrations for a long time. The University of Santo Tomas Hospital, Department of Neurology and concentration of β-Amyloid proteins in nerve cells that suffer from Psychiatry, Manila, Philippines diseases such as Alzheimer's will increase exponentially and will converge to very high concentrations for a long time. This condition Neurosarcoidosis in its rarity is an often overlooked diagnosis β causes an accumulation of -Amyloid concentration and causes especially in cases of recurring craniopathies as focal neurologic senile plaques to form which is the main cause of Alzheimer's deficits. A 26-year-old Filipino-Chinese male consulted for evaluation disease. of his recurrent bilateral facial neuropathy accompanied by intermittent bilateral blurring of vision. At times, there was also right doi:10.1016/j.jns.2019.10.1069 finger flexor weakness. Administration of intravenous and oral methylprednisolone controlled his symptoms. With tapering and discontinuation of steroids, his symptoms would recur. Appearance of eyelid nodules prompted biopsy which disclosed a noncaseating WCN19-1751 granuloma. The diagnosis of sarcoidosis was supported more with findings of lymphadenopathies on chest Computed Tomography scan. Neuroimaging and Cerebrospinal fluid analysis were non- Poster shift 02 - Autoimmune disorders - Part I /CNS infections - contributory. He was the first Filipino-Chinese patient documented Part I / dementia - Part I /epilepsy - Part I / health economics and with neurosarcoidosis with craniopathies and peripheral neuropa- outcomes / neuroepidemiology and environmental and other thies to our knowledge to date.

Eosinophilic granulomatosis with polyangitis masquerading as doi:10.1016/j.jns.2019.10.1071 acute demyelinating inﬂammatory polyradiculoneuropathy: A case report

J. Datu, A. Baroque, J.A.K. Torres WCN19-1769 University of Santo Tomas Hospital, Neurology and Psychiatry, Manila, Philippines Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Eosinophilic granulomatosis with polyangitis (EGPA) is a sys- Part I / dementia - Part I /epilepsy - Part I / health economics and temic small vasculitic disease involving the skin, lungs, kidneys and outcomes / neuroepidemiology and environmental and other peripheral nerves. In this report, we documented a case of a 47-year old Filipino female with EGPA initially mimicking an acute inflam- Distinct prion-like seeding properties of α-synuclein strains and matory demyelinating polyradiculoneuropathy, who presented with assessment of inactivation methods paraesthesia’s of bilateral lower extremities followed by ascending paralysis. A. Tarutaniab, T. Araic, S. Murayamad, S.I. Hisanagae, T. Tomitaa,M. With the history of asthma, confirmatory by biochemical workups Hasegawab and diagnostics: a positive perinuclear anti-neutrophilic cytoplasmic aThe University of Tokyo, Laboratory of Neuropathology and Neurosci- antibodies (p-anca), eosinophilia of more than 10% in peripheral ences- Graduate School of Pharmaceutical Science, Tokyo, Japan blood, Para nasal sinusitis in facial x-ray and polyneuropathy in bTokyo Metropolitan Institute of Medical Science, Department of EMG-NCV revealed that the patient fulfilled the criteria of the Dementia and Higher Brain Function, Tokyo, Japan American College of Rheumatology for EGPA. cUniversity of Tsukuba, Department of Neuropsychiatry- Division of We treated her with high dose glucocorticoid combined with Clinical Medicine- Faculty of Medicine, Ibaraki, Japan immunosuppressant, oral cyclophosphamide which provided induc- dTokyo Metropolitan Geriatric Hospital and Institute of Gerontology, tion of remission and significant improvement on her motor strength Department of Neuropathology, Tokyo, Japan by Manual Muscle Testing (MMT) and resolution of sensory deficits eTokyo Metropolitan University, Department of Biological Science, on all modalities of her upper extremities. Tokyo, Japan It is therefore necessary to consider EGPA in management of ’ patients presenting with bilateral limb weakness, paraesthesia s and Amyloid-like intracellular abnormal protein deposits are the systemic symptoms. We have obtained patient and/or Institutional defining neuropathological feature of many neurodegenerative Review Board (IRB) approval, as necessary before accomplishing this diseases. A growing body of evidence strongly suggests that these study. abnormal proteins have prion-like properties, and induce self- templated amplification and cell-to-cell transmission. However, it is doi:10.1016/j.jns.2019.10.1070 not yet clear whether exposure to exogenous prion-like proteins can potentially cause these diseases in humans. Here, we examined this ARTICLE IN PRESS

156 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx question by characterizing the seeding activities of synthetic α- in 54 patients(p) studied for possible neurodegenerative deterioration synuclein (α-syn) fibrils and abnormal α-syn extracted from brains and its correlation by groups with the results of anatomical (NMR), of patients with multiple system atrophy (MSA) and dementia with functional-SPECT- and biochemical tests (TAU-t/TAU-p & β-amyloid). Lewy bodies (DLB). Exposure to synthetic α-syn fibrils at concentra- 18F-FDG-PET/CT acquired at 30-45' injection intravenous of 180MBq tions above 100 pg/mL caused seeded aggregation of α-syn in SH- (18F-FDG), by brain PET located of 15 'duration. SY5Y cells, and seeded aggregation was also observed in C57BL/6J mice after intracerebral injection of at least 0.1 μg/animal. α-Syn Results aggregates extracted from MSA brains showed higher seeding 54p with 18F-FDG-PET/CT, 57%♂, 43%♀, age: 63a [29-83]. Reason activity than those extracted from DLB brains, and their potency for consultation: mild cognitive impairment-MCI-(7p), FTD(11p), AD was similar to that of synthetic α-syn fibrils. We also investigated (8p), subjective memory complaints-SMC-(4p). In 51(94%)patients the effects of various methods that have been reported to inactivate 18F-FDG-scans was pathological result: 68.5% anterior pattern and abnormal prion proteins (PrPSc), including autoclaving at various 22% posterior pattern. temperatures, exposure to sodium dodecyl sulfate (SDS), and -CSF-biomarkers and 18-FDG-PET/TC MATCH in 85.2% (3p normal combined treatments. The combination of autoclaving and 1% SDS pattern,37p-FTD, 6-AD) and 8p (14.8%) with not coincidence. substantially reduced the seeding activities of synthetic α-syn fibrils -Only 44p(81%) with MRI, 11% (6p) with hippocampal atrophy and α-syn aggregates extracted from MSA brains. However, single concordant with hipometabolism at 18F-FDG-PET/CT. treatment with 1% SDS or generally used sterilization conditions -36p(67%) had SPECT 75% (27p) was pathological and coincident proved insufficient to prevent accumulation of pathological α-syn. In with 18F-FDG-PET/CT 55%(20p). conclusion, α-syn aggregates derived from MSA patients showed a In no case was there a triple coincidence 18F-FDG-PET/TC-MR potent prion-like seeding activity, which could be efficiently reduced and CSF, possibly due to the poor findings of the MRI, influenced by by combined use of SDS and autoclaving. acquisition with a 1.5T device. doi:10.1016/j.jns.2019.10.1072 Conclusion Anterior pattern was the most frequent, porbably related to the bias generated from the Neurology consultation. We found a good correlation between our results and the CSF biomarker, like the WCN19-1770 tendency to rely more on the clinical, neuropsicological and PET tests, reserving the CSF for difficult diagnosis. Better correlation with MRI, can get with better hippocampal volumetry (how we are Poster shift 02 - Autoimmune disorders - Part I /CNS infections - observing with MR-3T). Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1073

Establish the correlation between the functional, anatomic and CSF-biomarkers, in the neurodegenerative deterioration of referred patients to the pet/unit WCN19-1774

J.M. Nogueiras Alonsoa, A. Renda Alcaldea, B. Nunez De De Oliveirab, Poster shift 02 - Autoimmune disorders - Part I /CNS infections - C.D.V. Martinez Ramosb, V.D.V. Rodríguez Moralesb Part I / dementia - Part I /epilepsy - Part I / health economics and aSpecialist, Nuclear Medicine, Vigo, Spain bResident, nuclear medicine, Vigo, Spain outcomes / neuroepidemiology and environmental and other

ﬁ Aim Quanti cation of medial temporal lobe atrophy score in patients -Asses the correlation between the results of 18F-FDG-PET/CT, with vascular dementia SPECT, brain-MR and CSF in the diagnosis of neurodegenerative a b a a b dementia. P. Siongco , M.Z. Cabreros , S. Marasigan , J. Navarro , P.D. Lagamayo aUniversity of Santo Tomas Hospital, Department of Neurology and Material and methods Psychiatry, Metro Manila, Philippines b Retrospective review (March-November 2018) of cerebral 18F- University of Santo Tomas Hospital, Department of Radiological FDG-PET/CT scans by two double-blind nuclear physicians, conducted Sciences, Metro Manila, Philippines ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 157

Background Results Vascular dementia (VD) is the second most common cause of The mean age of subjects in the study was 73.54 years old (mean dementia in older people. It is a chronic progressive disease of the SD + 8.63), mostly females and completed tertiary education. Linear brain bringing about cognitive impairment. The loss of brain regression analysis was used to measure the influence of medial parenchyma is caused by cerebrovascular diseases. Dementia rates temporal lobe atrophy in MMSE-P and CDT scores. Results showed are growing at alarming proportion in several regions of the world inverse relation for MTA on both MMSE-P and CDT scores. Hence, for and is related to population aging. every 1-unit increase in MTA, there is a 0.58-unit decrease in CDT and 0.66-unit decrease in MMSE-P scores. Objective This study aimed to determine the influence of medial temporal lobe atrophy on the Mini-Mental State Examination-Philippines Conclusion (MMSE-P) and Clock Drawing Test (CDT) scores in patients MTA significantly decreased MMSE-P and CDT scores in patients diagnosed with VD. with VD. So, as the medial temporal lobe atrophy score is higher, a corresponding decrease in both the MMSE-P and CDT scores. Design This study utilized a cross-sectional design. We included elderly doi:10.1016/j.jns.2019.10.1074 Filipinos N 60 years old diagnosed with VD from 2013 – 2018. Subjects were derived from in- and out-patient referrals in a tertiary hospital in Metro Manila. Schelten’s visual scale was utilized to evaluate medial temporal lobe atrophy (MTA). ARTICLE IN PRESS

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

WCN19-1775 bNational Institute of Mental Health and Neurosciences-NIMHANS, Neuropathology, Bengaluru, India cNational Institute of Mental Health and Neurosciences-NIMHANS, Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Neuromicrobiology, Bengaluru, India Part I / dementia - Part I /epilepsy - Part I / health economics and dNational Institute of Mental Health and Neurosciences-NIMHANS, outcomes / neuroepidemiology and environmental and other Neuroimaging and Interventional Radiology, Bengaluru, India eNational Institute of Mental Health and Neurosciences-NIMHANS, Estimating seizure burden in Korea by internet searching volume Neurosurgery, Bengaluru, India

K.H. JI Introduction Busan Paik Hospital- Inje University College of Medicine, Department of Cerebral phaeohyphomycosis is a rare cause of central nervous Neurology, Busan, Republic of Korea system infection. Even immunocompetent hosts may be affected.

Introduction Objective Google Trends and Naver DataLab are open tools that provide To describe the clinical course and challenges in the diagnosis of a relative Internet search volume on specific queries and allow us to patient with cerebral phaeohyphomycosis who was initially mis- estimate indirectly public interest in search terms. Both provide the taken to have tubercular meningitis. comparative averages of the search volumes that numbers are representing search interest proportional to the highest point in Case the chart for the time. As Naver is a dominant Internet search engine A 48-year-old farmer from India presented with chronic headache in Korea, Korean interest can be estimated more precisely with Naver and intermittent fever. Serial MRIs of brain showed enhancing Datalab than with Google Trends. Korean seizure burden was exudates in basal cisterns, sella, perichiasmatic and cerebellopontine estimated using queries on syncope and seizure by Naver DataLab angle region. Cerebrospinal fluid (CSF) study showed lymphocytic and compared with those by Google Trends in Western countries. pleocytosis (102 to 1000 cells/cu.mm) and elevated protein (47 to 338 mg/dl). Anti-nuclear antibody, anti-neutrophil cytoplasmic Methods antibody and angiotensin converting enzyme levels were normal. The relative search volume is referred to as the Naver Trend Index GAD65 antibody was inconsistently positive in serum. Cysticercal (NTI) and Google Trend Index (GTI) respectively. NTI and GTI with antibody was detected in CSF. Based on these observations, the queries for the syncope-related terms (syncope, faint, fainting, differential diagnoses of tubercular vs cysticercal meningitis were collapse) and seizure-related terms (seizures, convulsion, convulsive considered. He was treated with anti-tubercular therapy, albe- movement, epilepsy, epileptic seizure) were downloaded from Dec.1. ndazole and steroids. His symptoms persisted despite good compli- 2018 to May. 1. 2019. The proportions of seizure over syncope, ance. Dural biopsy after two years of illness revealed necrotising granulomas with numerous slender, pauci-septate pigmented fungal NTIseizure/syncope and GTIseizure/syncope were computed. hyphae. Dural culture grew Cladophialophora species. ATT and Results steroids were discontinued and treatment with amphotericin B and The seizure-related terms were more searched than syncope- voriconazole was initiated. related terms in Korea (mean NTIseizure/syncope 3.84±0.91) but less in Discussion western countries (mean GTIseizure/syncope United States 0.91±0.12, United Kingdom 0.80±0.15, Canada 0.80±0.22). Cladophialophora species, the most frequently isolated species in cerebral phaeohyphomycosis, causes life-threatening brain Conclusion abscess, meningitis, encephalitis, myelitis or arachnoiditis. The These findings suggest high public interest and fear of seizure in salient feature in our patient is chronic indolent course despite both Koreans and westerns considering ten times the high incidence long-term steroid intake without anti-fungal therapy for two of syncope over seizure in a lifetime. It is probable that Koreans have years. more fear and seizure burden than westerners. Conclusion doi:10.1016/j.jns.2019.10.1075 Diagnosis of cerebral phaeohyphomycosis requires high index of suspicion. CSF and MRI may mimic tubercular meningitis in endemic areas, however hypoglycorrhachia is uncommon. Targeted biopsy clinches the diagnosis.

WCN19-1800 doi:10.1016/j.jns.2019.10.1076

Poster shift 02 - Autoimmune disorders - Part I /CNS infections - Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other WCN19-1804

Chronic fungal meningitis secondary to dematiaceous fungi Poster shift 02 - Autoimmune disorders - Part I /CNS infections - masquerading as tubercular meningitis Part I / dementia - Part I /epilepsy - Part I / health economics and outcomes / neuroepidemiology and environmental and other N. Hesarura, S. Donaparthi Venkatagiria, M. Nagappaa, V. Santoshb,N. Chandrashekarc, S. Raob, N. Reddya, P. Sharmaa, J. Sainid, N. Pruthie,D. ﬁ Shuklae, A.B. Talya, S. Sinhaa Nonspeci c low back pain as initial presentation of Morvan's aNational Institute of Mental Health and Neurosciences-NIMHANS, syndrome successfully treated with therapeutic plasma exchange Neurology, Bengaluru, India ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 159

I. Bilic, V. Kosta impairment characterizing the chronic epileptic condition. Thus, the University Hospital Center Split- School of Medicine University of Split, purpose of our study was to further characterize the capacity of Department of Neurology, Split, Croatia neurovespina (a new peptide similar to one found in a social brazilian wasp Polybia occidentalis) to prevent hippocampal neuronal Morvan's syndrome is a rare, complex neurologic disorder loss. After 3 h of SE, mice were treated (i.p. injections) during the characterized by neuromyotonia, dysautonomia and neuropsychiat- epileptogenesis period for 15 days with peptide neurovespina ric features. (doses: 2, 4 or 8 mg/Kg) or vehicle (control groups). Thirty days We present a 22-year old, previously healthy man with 2 month after pilocarpine-induced SE (chronic period), all the animals were duration of nonspecific and nonradiating low back pain which was perfused and their brains were processed (hippocampal slices from evaluated in two neurologic clinics and resulted with no specific each mice group) for histological analysis through Nissl method. We diagnosis. Later, in 2-week time, our patient developed many other also used fluorescent Nissl stain to compare changes in hippocampal „classical“ symptoms of Morvan's syndrome: insomnia, hallucina- structures. The cell counts (Image-J) in the Nissl-stained and tions, agitation, hyperhidrosis, drooling, weight loss, itching, neuro- morphological analysis (Figure) of hippocampal formation shows pathic pain and prominent twitching of muscles mostly in the lower no significant loss of selective populations of neurons in areas CA1 limbs. and CA3 and in the hilus when compared with control group. These Extensive diagnostic evaluation was done. Normal findings were data indicate that neurovespina confers neuroprotective effect in observed for: routine hematology and biochemistry except for CK pilocarpine-induced SE model and has potential for the development which was slightly elevated (380, normal values 50-177); tumor of novel drugs for neurological diseases. markers, hormones (LH, FSH, ACTH, T3, T4, TSH, PTH); urine analysis; anti-ganglioside antibodies; anti-Hu, anti-Ri, anti-Yo antibodies. doi:10.1016/j.jns.2019.10.1078 Liquor analysis showed elevated protein level 497.2 mg/L (normal values 130-370), while cell count, chloride, glucose, lactate, as well as S-100 protein were normal. EEG and EMNG analysis were within normal limits. He was anti-HbsAG, anti-HCV and anti-HIV WCN19-1807 negative. Abdomen ultrasound, X-ray of the coxofemoral joints, MSCT of Poster shift 02 - Autoimmune disorders - Part I/CNS infections - the thorax and brain MRI were without any pathologic findings. Neuropsychologic evaluation showed anxiety, elevated psychomotor Part I/dementia - Part I/epilepsy - Part I/health economics and tension which was projected on physical plan with lowered mental outcomes/neuroepidemiology and environmental and other efficacy. Later, when elevated titer of anti-CASPR2 antibodies were found, Atypical electroencephalographic features in patients with ge- the diagnosis was introduced. netic generalized epilepsy He was treated with carbamazepine without any improvement in clinical symptoms. We proceed with plasmapheresis (5 treatments) I. Abdelkefia, I. Kacemab, A. Nasriab, S. Mrabetab, M. Ben Djebaraab, A. and many symptoms diminished so patient was discharged without Gargouri-Berrechidab, R. Gouiderab any psychopathologic phenomena or vegetative symptoms, and with aRazi Hospital, Neurology- LR18SP03, Mannouba, Tunisia no low back pain. bUniversity of Tunis El Manar, Faculty of Medicine, Tunis, Tunisia doi:10.1016/j.jns.2019.10.1077 Introduction Several studies have reported focal features in genetic generalized epilepsy (GGE). We aimed to point out the significance of such patterns and their impact on the choice, response to anti epileptic WCN19-1805 drugs (AEDs) and the disease's course.

Methodology Poster shift 02 - Autoimmune disorders - Part I/CNS infections - We performed a retrospective study including patients with Part I/dementia - Part I/epilepsy - Part I/health economics and genetic GGE in our department (July 2002–December 2010). We outcomes/neuroepidemiology and environmental and other retrospectively reviewed all EEGs recorded at our EEG unit. We defined 3 clinical groups (juvenile myoclonic epilepsy (JME), absence Neuroaprotective effect of peptide neurovespina after pilocar- epilepsy (AE) and GGE with generalized tonic-clonic seizures pine-induced status epilepticus in mice only) and 3 EEG groups (“typical” generalized features, “atypical” features (focality and asymmetry) and those without epileptiform L. Carneiro, M. Mortari discharges). University of Brasilia, Neuropharmacology Laboratory- Phisiology Department, Brasilia, Brazil Results We included 248 patients and reviewed 517 EEGs. “Atypical” Status epilepticus (SE) is the most common serious neurological features were identified in 16.2% of the tracings (25.4% of the condition triggered by abnormal electrical activity, leading to severe patients). They were more common on the anterior regions (frontal and widespread cell loss in the brain. The muscarinic receptor and fronto-temporal) and in JME group. There was no significant agonist pilocarpine is used to induce SE, which is followed by difference in their frequency before and after therapy (p N 0.05). hippocampal damage. After several hours of SE, pilocarpine-treated Before specialized consultation, the AEDs' choice was inappropriate animals remit spontaneously and go into a seizure-free period, in 12.1% of patients. After adequate treatment, there was no known as pileptogenesis period, before displaying the spontaneous significant difference in seizure outcome according to the presence recurrent seizures (SRSs) that and which could also cause cognitive or absence of focality in EEGs (p N 0.3). ARTICLE IN PRESS

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

Conclusions Atypical and focal EEG features in patients with GGE are common, especially in anterior brain's regions. They usually lead to misdiagnosis of focal epilepsy with secondary generalization then inappropriate AEDs' choice. Hence, their interpretation must be cautious, and the clinical history of the epilepsy remains the main clue for the right diagnosis. The presence of focal EEG features does not alter the course of the disease. doi:10.1016/j.jns.2019.10.1079

WCN19-1832

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

A case of clippers with spinal cord presentation

V. Aglave, P. Ojha, R. Ansari, P. Barvalia Grant Government Medical College and Sir JJ Hospital, Neurology, Mumbai, India

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is an inflammatory condition primarily involving the pons and adjacent structures and presents with cerebellar and brainstem signs such as dysarthria, ataxia, cranial nerve palsies and long tract signs. It has characteristic MRI findings and clinico-radiologic responsiveness to steroids. It is a diagnosis of exclusion.

Case 53 years male patient presented with 1-month history of subacute progressive paraparesis with sensory level at T10, with urinary urgency and constipation. However, there were no signs or symptoms referable to the brainstem.

Results MRI revealed characteristic gadolinium enhancing punctate lesions without any mass effect in brainstem and cerebellum as well as the cervicothoracic cord and cerebral white matter. Autoimmune, malignancy and infection workup were negative. CSF analysis revealed 30 cells/cu.mm,92% lymphocytes along with protein 90 mg %.PET-CT of whole body revealed hypometabolism in the brainstem and cerebellum. Patient was started on intravenous Methylprednis- olone 1 g/day for 5 days followed by Prednisolone 60 mg per day and Methotrexate. He showed signiﬁcant improvement and follow up MRI scan at 2 months revealed clearing of all lesions.

Conclusions Typically, CLIPPERS patients present with symptoms and signs referable to the brainstem and cerebellum, however here we present a case with a predominant spinal cord presentation. Our patient was extensively worked up for alternate causes and showed typical clinical and radiologic responsiveness to steroids.

I have obtained patient and Institutional Review Board approval. An Institutional Review Board and Animal Use Committee have waived the requirement for their formal approval of the study. doi:10.1016/j.jns.2019.10.1080 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 161

WCN19-1834 scientific research has resulted in identification of a huge number of disease targets (e.g. amyloid-β, tau, PP2A, β-secretase), current methods have not sufficiently utilized the identified targets for Poster shift 02 - Autoimmune disorders - Part I/CNS infections - development of drugs using in silico modeling. In this study, we aim Part I/dementia - Part I/epilepsy - Part I/health economics and to produce a computational model (using molecular docking/QSAR outcomes/neuroepidemiology and environmental and other methods) for PP2A, an important disease target responsible for NFTs (neurofibrillary tangles) in AD. After QSAR model validation, a test Clinical and epidemiological characteristics of adult epilepsy set of 20 phytochemicals, identified from medicinal plants tested for population of Bishkek AD in vitro and in vivo research, will be screened and molecular docking on PP2A will be attempted. Finally, such a model will B. Kadyrovaa, A. Jusupovab provide crucial information about the molecular descriptors that are aKyrgyz State Medical Academy, Bishkek, Kyrgyzstan important for generation of newer drugs for AD. bKSMA, Kyrgyz State Medical University, Bishkek, Kyrgyzstan doi:10.1016/j.jns.2019.10.1082 Purpose Increasing the effectiveness of treatment and quality of life of patients with epilepsy of the adult population of Oktober district of Bishkek. WCN19-1838

Method Poster shift 02 - Autoimmune disorders - Part I/CNS infections - The study involved 430 patients with epilepsy during 2014- Part I/dementia - Part I/epilepsy - Part I/health economics and 2016yy from Family Medicine Centers of Oktober district of Bishkek. outcomes/neuroepidemiology and environmental and other Results The non-standardized prevalence of epilepsy in the Oktober Gas geyser syndrome: Preventable emerging toxic encephalopathy in developing countries district of Bishkek was 2.21 persons / 1^000 population, the age onset of the disease prevailed under the age of 20 years (p b 0.05). The study population was dominated by patients with generalized R. Ansari, G. Soni, P. Ojha, V. Aglave, P. Barvalia seizures (78.7%) (p b 0.05). The most frequent etiological factors Grant Government Medical College and J J Hospital, Department of were generalized epilepsy (37.9%), as well as post-traumatic epilepsy Neurology, Mumbai, India (25.3%). It was found that in the majority of cases valproate (20.5%) and carbamazepine (78.3%) were prescribed, at the minimal Background therapeutic level (the average daily dose of valproate was 868.8 ± In developing countries such as India, liqueﬁed petroleum gas 4.3 mg, and the average daily dose of carbamazepine was 378,9 ± geysers are used very frequently for heating water. However 0,9 mg), sometimes even not reaching them, which can lead to these gas geysers emit various toxic gases, one amongst them is pharmacoresistance. In a comparative analysis of the quality of life, colourless, odourless CO (carbon monoxide), which is a preventable depending on the received AED, high values for almost all subscales emerging cause of toxic encephalopathy Here we present case report of the QOLIE-31 questionnaire were obtained in patients receiving of 2 patients with Gas geyser syndrome with varied neurological lamotrigine and polytherapy. events.

Conclusion Case1 The data obtained make it possible to justify the inclusion of 36 year old male presented with history of unconsciousness while “new” AED in the list of state guarantee programs on preferential bathing, which lasted for an hour following which he was apathetic, drugs. Organization of a school of epilepsy in the Family Medicine inattentive and had recent memory loss. No h/o tongue bite. Rest Centers will lead to an improvement in the quality of life of patients examination was normal. with epilepsy. Case2 doi:10.1016/j.jns.2019.10.1081 18 year old male presented with history of unconsciousness in bathroom for 15-20 min, following which he was shouting irrele- vantly for few minutes then become drowsy for 2 h. He was confused, inattentive, immediate recall was impaired, rest of WCN19-1837 examination was normal. Both cases on detailed history regarding usage of gas geyser revealed that they were using gas geyser that was in a small sized ill- Poster shift 02 - Autoimmune disorders - Part I/CNS infections - ventilated bathroom. So based on available history and neuro- Part I/dementia - Part I/epilepsy - Part I/health economics and imaging^, Gas geyser syndrome diagnosis was made. outcomes/neuroepidemiology and environmental and other Conclusion Computational modeling of PP2A as an Alzheimer's disease target Awareness regarding usage of gas geyser is necessary as it is preventable cause of toxic encephalopathy mainly CO intoxication. A.K. Sharma, R. Goyal, G. Kaur Simply by putting burner and heater outside or in a well ventilated Shoolini University, School of Pharmaceutical Sciences, Solan, India room, we can entirely prevent this condition which can cause varied manifestation ranging from confusion to memory loss, even death. Alzheimer's Disease (AD) is a progressive neurodegenerative I have obtained patient and or Institutional review board disorder with minimal curative treatment options. While recent approval^,as necessary. ARTICLE IN PRESS

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

An Institutional review board and Animal Use committee have Purpose waived the requirement for their formal approval of study. Alzheimer's disease is neuro-degenerative and age related disease that causes progressive debility of language and doi:10.1016/j.jns.2019.10.1083 memory.Inapresentday,datamininginthefield of medical is an important tool to explore the sign patterns in term of medical data sets. The aim of current study is to investigate a method for the classification system of Alzheimer's disease in data findings in WCN19-1846 databases utilizing data mining method which are used in present time.

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Methods Part I/dementia - Part I/epilepsy - Part I/health economics and In data mining system and research work, the Tanagra data outcomes/neuroepidemiology and environmental and other mining device, an accurate classifier algorithm and an open source project is used. By using different classification algorithms, we find a Classification system via data mining algorithm: New tool to patterns which is formed in it. Fischer filtering was good feature in diagnose Alzheimer's disease feature selection algorithm. The architecture model flow includes: fi Training Dataset, Feature Relevance Analysis^, Classi cation Process fi M. Singh i.e. Classi cation and regression Tree (C-RT), Random Tree (Rnd HMFA MIET- Handia, Department of Computer Sciences, Allahabad, Tree) Binary Logistic Regression, Support Vector Machine (SVM) India Accuracy Analysis. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 163

Results WCN19-1866 The research work was accomplished on the basis classification algorithms including training and test datsets along with well obtained characterized data. The classification process categorized Poster shift 02 - Autoimmune disorders - Part I/CNS infections - the Alzheimer's disease in dataset and provides a better results. The Part I/dementia - Part I/epilepsy - Part I/health economics and feature relevance analysis displays the different important charac- outcomes/neuroepidemiology and environmental and other teristics which are intended for better classification system. The random tree forms the classification rule to attain a zero error value. Sporadic Creutzfeldt Jakob disease with fast progressive evolution – A challenging diagnosis – A case report Conclusions The experiment is projected to prove the efficiency of the M.G. Catanaa, A. Boiesanb, I. Roman-Filipc, C. Roman-Filipa presentation of numerous classifiers to the Alzheimer's disease and aEmergency Clinical County Hospital of Sibiu, Neurology, Sibiu, Romania dataset used in it. This help the patient to treat at the right time. bEmergency Clinical County Hospital of Sibiu, Anesthesia and Intensive Care, Sibiu, Romania doi:10.1016/j.jns.2019.10.1084 cLucian Blaga University of Sibiu, General Medicine, Sibiu, Romania

Creutzfeldt-Jakob disease (CJD), a spongiform encephalopathy, is a rare and fatal condition that affects the brain. It causes brain WCN19-1849 damage that worsens rapidly over time. We herein report the case of a 67 years old patient without pathological antecedents who was admitted to our department for visual and gait disturbances, coreo- Poster shift 02 - Autoimmune disorders - Part I/CNS infections - athetotic movements and confusional state, symptomatology that had Part I/dementia - Part I/epilepsy - Part I/health economics and started about one month before and worsened progressively. The outcomes/neuroepidemiology and environmental and other neurological exam pointed out: cortical blindness, coreoathetotic movements left more than right, hyperestesia. Laboratory test results Acute stroke care: Experience of a tertiary care center in Delhi, revealed hyperglycemia. All others tests (B12 vitamin, B,C, hepatitis, the way to go HIV, FT4, TSH hormones, paraneoplastic and autoimmune markers – NMDA, GABA, VKGT) were physiologically within limits. The cerebro- V. Kumara, S.K. Saxenaa, R. Guptaa, A. Batraa, G. Rajpalb spinal fluid analysis revealed an intense 14-3-3 and TAU protein aMax Institute of Neuroscience, Neurology, Delhi, India positivation. The CT scan that was performed did not reveal anything bMax Institute of Neuroscience, Interventional Neurology, Delhi, India pathological. A cerebral MRI with contrast injection and an EEG were performed. The MRI, which was performed in general anesthesia, Stroke is one of the leading cause of death and disability in India. revealed: bicerebral cortical cortex hypersignal in diffusion sequence However providing acute stroke care including IV Thrombolysis and (except the left parietal lobe), with no FLAIR correspondence, without Mechanical thrombectomy remains a challenge due to paucity of pathological contrast intakes,while EEG highlighted: slow dysrhyth- stroke centers and poor public awareness about symptoms of stroke mia, delta waves with three phase paroxysmal discharge, Based on and available treatment options. We analyzed our data of patients of these examinations was established the final diagnosis: Probable acute stroke admitted in last two years. Sporadic Creutzfeldt Jakob disease. The patient received treatment 932 patients of acute stroke were admitted between Decem- with valproic acid and clonazepam with no significant improvement. ’ ’ ber 2017 to December 2018. 673(^72.21%) were male and 259 (^27.7) doi:10.1016/j.jns.2019.10.1086 female. Of these 710 were ischemic strokes (^76.18%) and 222 were haemorrhagic (^23.81%). Average age of the patients was 62 years with a range of 15 yrs. to 90 years. 107 patients were b 45 years of age (^11.4%), which corroborates with Asian data. The average NIHSS was 8 with range of 2 to 23. 184 patients WCN19-1879 (^19.7%) were thrombolysed with IV TPA, which is a high number when compared with nationwide data. Average door to needle time was 48 min with a lowest of 4 min. Another major finding was Poster shift 02 - Autoimmune disorders - Part I/CNS infections - outcome at 3 months with Modified Rankin Scale of 0–1 in 60% of Part I/dementia - Part I/epilepsy - Part I/health economics and patients. outcomes/neuroepidemiology and environmental and other 36 patients had large artery occlusion, of which 23 had M1 MCA occlusion, 9 had Tandem occlusion and 4 had T-occlusion. These Refractory and super refractory status epilepticus in the Philip- patients underwent mechanical thrombectomy using Solitaire device pines: A 10-year retrospective study fi in addition to IV thrombolysis. Of these 25 (^69.4%)had signi cant improvement with mRS of 0–1 at 3 months. L.E. Quiles, M.L. Fernandez Although our data has shown encouraging findings, there is still a The Medical City, Neurology, Pasig City, Philippines long way to go. Super refractory status epilepticus (SRSE) is an uncommon, but doi:10.1016/j.jns.2019.10.1085 very important clinical problem that is associated with high ARTICLE IN PRESS

164 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx morbidity and mortality. Studies concerning Super refractory status knowledge this is the first described case of a mutation in epilepticus has been limited. Up to present, there are no existing heterozigoty (mutation 5222_5225+1delTAAAG) in NIPBL gene that Philippine data. The study aims to determine the status of Refractory evolved to an epiletic encephalopathy. (RSE) and Super refractory status epilepticus within a 10-year period in a Tertiary Hospital. This is a retrospective study of adult patients doi:10.1016/j.jns.2019.10.1088 with prolonged seizures admitted at a Tertiary Hospital from January 2009–July 2018. Frequency, Mean and standard deviation was used in the Descriptive analysis. Multinomial Logistic Regression was used to assess probability of good or poor outcome. Significant Correlation WCN19-1895 is defined by P value of b0.05. The Incidence of Refractory Status Epilepticus is as high as 38% (n = 64) and 35% (n = 58) for Super Refractory Status Epilepticus. Mortality rate is 39.1% in Refractory Poster shift 02 - Autoimmune disorders - Part I/CNS infections - and 62.1% in Super refractory status epilepticus. Poor functional Part I/dementia - Part I/epilepsy - Part I/health economics and outcome has been observed in RSE and SRSE whein the majority was outcomes/neuroepidemiology and environmental and other Alive Dependent. Significant factor increasing likelihood of being Alive Dependent is the absence of Arrest. Factors associated with Pneumocephalus as a complication of multidrug-resistant Kleb- likelihood of being alive and independent includes Status Epilepticus siella Pneumoniae bacteremia in patient with acute myeloid which is more benign type of prolonged seizure and younger age leukemia therefore more aggressive control of seizures in preventing progression to SRSE will give higher likelihood of good functional outcome F. Alkindia, N. AlShehhia, A. Khanb, S. Hussainc, M. Szolicsd,M.Ur and elderly patients would need closer and more adept seizure Rahmane control for better functional outcome. aTawam Hospital- SEHA, Internal Medicine, AlAin, Abu Dhabi, United Arab Emirates doi:10.1016/j.jns.2019.10.1087 bTawam Hospital- SEHA, Infectious Disease, AlAin, Abu Dhabi, United Arab Emirates cTawam Hospital- SEHA, Hematology, AlAin, Abu Dhabi, United Arab Emirates WCN19-1887 dTawam Hospital- SEHA, Neurology, AlAin, Abu Dhabi, United Arab Emirates eTawam Hospital- SEHA, Intensive Care, AlAin, Abu Dhabi, United Arab Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Emirates Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Background Pneumocephalus indicates air in the cranial cavity and commonly Epilepsy in cornelia de lounge syndrome results from craniofacial trauma and neurosurgical procedures. Infections can rarely cause pneumocephalus through formation of a b S. França , R. Chorão brain abscess, or as complication of chronic otitis media, or a Unidade Local de Saúde de Matosinhos-Hospital de Pedro Hispano, meningitis. Patients might present with headache, fever, change in Neurologia, Matosinhos, Portugal mental status and diagnosis is confirmed by Brain CT scan. bCentro Materno Infantil do Porto, Neuropediatria, Porto, Portugal Case description Cornelia de Lange Syndrome is a rare, multisystemic disorder, A 51 years old male diagnosed with acute myeloid leukemia linked to genetic alterations. Is characterized by facial dysmorphism, (AML) and admitted for fourth cycle of consolidation chemotherapy. limb malformations, intellectual disability, and developmental delay. He developed prolonged febrile neutropenia and septic shock Hirsutism, growth failure, gastrointestinal problems, and behavioral and multi-organ failure due to Klebsiella Pneumoniae MDR abnormalities are commonly observed. Epilepsy is a clinical feature bacteremia. Patient had persistent bacteremia despite being on found in about 20% of cases: usually the prognosis is favourable and Meropenem, Colisten, Levofloxacin, Caspofungin, Tigecycline. Due therapy can be withdrawn.The authors present a case of a patient to profound thrombocytopenia, he had severe pulmonary hemor- with Cornelia de Lounge owing to a NIPBL gene mutation with rhage and bilateral diffuse subarachnoid hemorrhage. Repeated CT seizures at the age of 3. Electroencephalogram and brain magnetic brain angiography later showed extensive pneumocephalus with ressonance imaging were normal. Latter had seizures an started on finding suggestive of brain death. Ethical approval for case report sodium valproate that was replaced to carbamazepine, but seizures obtained. get worsened. Levetiracetam was started, but with 5 years of age had more than16 daily seizures (dialectic, tonic and myoclonic). EEG Discussion presented a burst suppression pattern. Several antiepileptics were Meningitis associated with pneumocephalus is rare and has been otimized to high dosis VPA, levetiracetam, and zonisamide, but reported in gas-forming organisms such as E. coli, Klebsiella patient maintained daily seizures with clinical regression. Latter on Pneumoniae, Bacteroides, mixed aerobic and anaerobic species and developed a status epileticus very drug resistente. With 7,5 years of streptococcus infection. Treating the underlying meningitis might age patient became seizure free and improved contact and gait and results in resolution of pneumocephalus, although fatal outcomes stayed on monotherapy with rufinamide. Now, with 10 years of age, were reported. Pneumocephalus secondary to Klebsiella Pneumoniae patient remain seizure free but with encephalopathy.The authors meningitis is uncommon and one case reported successful treatment report a patient with a severe and drug resistent epilepsy that using meropenem and amikacin for MDR Klebsiella Pneumoniae evolved to a epileptic encephalopathy wich is very rare in this meningitis associated with pneumocephalous resulting from chronic patients that usually have a mild epilepsy. From the authors otitis media. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 165

Conclusion WCN19-1902 Pneumocephalus associated with MDR K. pneumonia bacteremia is very rare and had high mortality rate especially in setting of febrile neutropenia with multi-organ failure. Intracerebral hemorrhage in Poster shift 02 - Autoimmune disorders - Part I/CNS infections - setting of persistent bacteremia might increase the risk of Part I/dementia - Part I/epilepsy - Part I/health economics and pneumocephalus. outcomes/neuroepidemiology and environmental and other doi:10.1016/j.jns.2019.10.1089 Abscess masquerading as stroke, malignancy and Aspergilloma

J.J.K. Thadam llh Hospital, Neurology, Abu Dhabi, United Arab Emirates WCN19-1898 35 year male with intermittent right hemianaesthesia of 1 month duration presented with abrupt onset right hemiplegia, which Poster shift 02 - Autoimmune disorders - Part I/CNS infections - spontaneously improved over four hours. CT brain showed hypodensity Part I/dementia - Part I/epilepsy - Part I/health economics and in the left frontal subcortical area consistent with TIA. MRI brain outcomes/neuroepidemiology and environmental and other revealed conglomerate ring enhancing lesions in the left postcentral gyrus suggestive of granulomatous lesion possibly Tuberculous (Figure Guillain –Barre syndrome AND central nervous system involve- 1). Chest radiograph revealed a homogenous opacity in the left lung. ment: Two case reports But CT thorax showed a rounded lesion with the Meniscus sign suggestive of Aspergilloma (Figure 3) A week later, he developed M. Nagazi, M. Aissi, N. Daoussi recurrence of right hemiplegia with global aphasia. Repeat CT showed a Fattouma Bourguiba University Hospital Monastir Tunisia, Monastir, large haemorrhagic lesionwith mass effect (Figure 2). At this stage the Tunisia possibilities considered were Fungalgranuloma versus Haemorrhagic metastasis.The doubling time of the lesion favoured infection rather Introduction than malignancy. Patient was started on Voriconazole with initial Although Guillain Barre Syndrome (GBS) is generally considered improvement in speech. MRI brain revealed haemorrhagic lesion as a peripheral neuropathy, there is evidence of central impairment consistent with aspergilloma. Within a week, his sensorium worsened explaining the observed atypies. abruptly with anisocoria. Repeat imaging showed impending brain We report the case of two patients who had a GBS with herniation and he underwent emergency craniectomy with drainage of hyperreflexia and a Babinski sign. foul-smelling black pus (reported intraoperatively as typical of fungal etiology), but microbiological cultures surprisingly grew Staphylococ- Observations cus aureus. He was subsequently treated with culture sensitive Two patients (23 and 70 years old) had simultaneous 4-limb antibiotics following which he improved considerably. weakness in less than 6 hours (aged patient) and 6-day ascending We are presenting this case of a young immunocompetent motor deficit (young). patient with pyogenic brain abscess, masquerading as stroke The examination revealed a tetraplegia with hyperreflexia in both clinically and Aspergilloma radiologically.The interesting fact is that patients with a Babinski sign (young patient ). Facing tetraplegia the diagnosis changed from TIA to cerebral infarct to Tuberculoma to associated with an albumino-cytological dissociation in the cerebro- invasive fungal lesion and finally to Staphylococcal abscess within spinal fluid (young), the normality of the spinal imaging as well as one month. the electrophysiological results in favor of an acute motor axonal Figure1 Conglomerate lesion polyradiculoneuropathy, the diagnosis of pure motor GBS (AMAN) Figure2 Haemorrhagic lesion was retained.Immunoglobulins were introduced with a favorable Figure3 Meniscus Sign evolution (young). The elderly patient developed on the first day a respiratory distress requiring ventilatory support despite the early doi:10.1016/j.jns.2019.10.1091 treatment. Our patients had a GBS with hyperreflexia and a Babinski sign requiring spinal imaging to eliminate a central lesion that could mimic these symptoms. In fact, in the pure motor forms WCN19-1905 (AMAN) and pharyngo-cervico-brachial syndrome (variant of GBS), the osteotendinous reflexes can be present even increased with clonus suggesting an associated central nervous system Poster shift 02 - Autoimmune disorders - Part I/CNS infections - involvement. Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Conclusion The clinical atypia of the GBS are more and more described. Their Favorable effects of plasma exchange in managing neurological lack of knowledge could delay the early management. conditions

F. Alkindia, Y. Boobesb, A. Hassanc aTawam Hospital- SEHA, Internal Medicine, AlAin, United Arab Emirates doi:10.1016/j.jns.2019.10.1090 bTawam Hospital- SEHA, Nephrology, AlAin, United Arab Emirates cTawam Hospital- SEHA, Neurology, AlAin, United Arab Emirates ARTICLE IN PRESS

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

Background There was gradual speech deterioration with loss of speech and Plasma exchange is a well-established therapeutic procedure used the child developed a complex movement disorder, having multiple in various autoimmune neurological disorders. The clinical utility of movement types, difficult to classify into the standard classification plasma exchange is evolving and outcomes vary from partial to of movement disorders. The movements wax and wane with therapy complete improvement in clinical condition with or without support but have not completely responded to maintenance Azathiprine and of immunosuppressant medications. We are reporting beneficial low dose steroids even after 3 years of therapy suggesting long term effects of plasma exchange in two cases as unusual indication. affection and disability due to complex movement disorders in NMDA encephalitis. Case 1 52 years old male, presented with 1 week history of confusion, doi:10.1016/j.jns.2019.10.1093 unsteadiness, dysarthria, progressive to quadriplegia and respiratory failure need ICU admission and intubation. Extensive workup reveal Seronegative Autoimmune Encephalitis associated with predominant cortical, limbic and cerebellar involvement in MRI brain. He was given WCN19-1920 trial of IV immunoglobulin (IVIG) for 5 days followed by prednisolone 60mg daily, with improvement in respiratory status only. Six sessions alternate day of plasma exchange were done, with marked improve- Poster shift 02 - Autoimmune disorders - Part I/CNS infections - ment in clinical condition. He was maintained on azathioprine 100mg. Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Case 2 22 years old lady, had morbid obesity underwent laparoscopic The reliability of the Bonini classification for frontal lobe sleeve gastrectomy. After one week she developed vomiting and seizures: An observational study bilateral limb weakness. She was diagnosed with Guillain-Barré syndrome (GBS) and received IVIG for 5 days. She developed acute N. Bhalerao, N. Shaikh, S. Iyer, J. Mani respiratory failure, intubation and tracheostomy for long term Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, ventilation. She was on regular physical therapy without improve- Neurology, Mumbai, India ment. After one month, she had trial of plasma exchange even though she failed IVIG and she is Category III grade 2C by ASFA Introduction guidelines. She had 6 sessions of alternate days plasma exchanges Frontal lobe seizures have varied clinical presentations. A with clinical improvement in power. semiological classification (4 groups) has been created to localize frontal lobe seizures [FLS] by Bonini et al (Epilepsia 2014) based on Conclusion stereo EEG data. Reliable use of such classification would depend on Plasma exchange is good immunomodulation therapy for Auto- reproducibility of categorisation across observers or by same immune Limbic Encephalitis and severe GBS who failed IVIG. observer at different times. doi:10.1016/j.jns.2019.10.1092 Aim To study the intra-observer and inter-observer reliability of grouping the semiology of FLS at our hospital based on the classification proposed by Bonini. WCN19-1907 Methodology Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Patients of FLS admitted for presurgical evaluation were selected. Two observers (trainee and consultant epileptologist) independently Part I/dementia - Part I/epilepsy - Part I/health economics and classified FLS into four Bonini groups by reviewing seizure videos. outcomes/neuroepidemiology and environmental and other The same observers reclassified the same set of seizures after eight weeks blinded from their first attempt. FLS semiology subgrouping Persistent complex movement disorders in NMDA encehalitis: A data was analysed for intra and inter-observer variability using case report in a child percent of agreement and intra class correlation (ICC coefficient, Cronbach’s alpha). Apart from videos, data from MRI brain and PET a b N. Yardi , R. Yardi CT scan were also available for localization of FLS. aYardi Epilepsy Clinic, KEM Hospital and Jehangir Hospital, Pediatric Neurology & Epileptology, Pune, India Results b Yardi Epilepsy Clinic and Cleveland Clinic USA, Neurology- Epi- MRI brain and PET CT of FLS patients showed focal lesions in 14/ leptology, Cleveland, USA 22 (63%) patients. Inter-rater observations showed substantial agreement ( 77% concordance, ICC average measure =0.724, p A wide variety of movement disorders, often in combination, can b0.002). Intra rater concordance for first rater was 77% and for be observed in children with anti-NMDAR encephalitis. A case of a 8 second rater 90%. Number of FLS patients ascertained Bonini group I year old boy presenting as movement disorder, faciobrachial in this cohort was maximum and had best inter rater concordance. movements and seizures was diagnosed as NMDA encephalitis 3 years ago. CSF and serum NMDA antibodies were positive early in the Conclusion course and early diagnosis could be made. The child deteriorated in Good inter-rater and intra-rater correlation is seen with Bonini spite of IVIGand methylprednisolone, needing ICU care and recov- classification of Frontal lobe seizures suggesting it is suitable for ered gradually in 3-4 weeks after addition of Azathioprine. classifying FLS. Prednisolone tapering doses and Azathioprine were continued as maintenance therapy and is still ongoing after three years doi:10.1016/j.jns.2019.10.1094 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 167

WCN19-1923 R. Kulkarni, S. Pujari Deenanath Mangeshkar Hospital and Research Center, Department of Neurology, Pune, India Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and Introduction outcomes/neuroepidemiology and environmental and other Dengue is an arboviral infection which is common in India. Neurological complications associated with dengue fever are un- Chlorambucil- Induced progressive multifocal leu- common, but are described from various parts of world. Gullain Barre coencephalopathy - A case report syndrome (GBS) is an acute neuropathy following antecedent respiratory or gastrointestinal infection. It has been described M. Danovska, E. Ovcharova, D. Marinova-Trifonova, I. Mladenovski following arboviral infections like dengue, zika, chikungunya fever. University Hospital, Neurology Clinic, Pleven, Bulgaria We describe 3 cases of GBS following dengue fever.

Introduction Cases Progressive multifocal leucoencephalopathy (PML) is a fatal These cases were seen in 2015-2017. All 3 patients had acute demyelinating disease of the CNS caused by reactivation of the neuropathy following dengue fever. The diagnosis of dengue was John-Cunningham (JC) polyomavirus. Only few cases of done on basis of clinical features, laboratory abnormalities and Chlorambucil-induced PML in chronic lymphocytic leukemia (CLL) positive dengue serology (NS1 in 1 and IgM in 2). are described in literature. There were 2 males and 1 female. The ages were 31, 41 and 55 years. The neurological manifestations begin with febrile illness. All 3 Aim patients had limbs weakness, all 3 patients had bilateral facial To present a clinical case of PML in a patient with CLL in weakness and 2 patients had respiratory muscles weakness requiring remission after treatment with Chlorambucil misdiagnosed with mechanical ventilation. ischemic stroke. Nerve conduction study showed severe axonal neuropathy in 2 and demyelinating neuropathy in 1. CSF showed proteins N100 mg% Material and methods in all 3 cases and pleocytosis ranging from 5-30 cells. Somatic and neurological assessment, blood and CSF examination, All patients were treated with intravenous immunoglobulin and immunologic tests and Magnetic Resonance Imaging (MRI) were showed moderate recovery in 2 and excellent recovery in 1. performed. Conclusion Results GBS can occur with dengue fever. More bifacial involvement, A 57-year-old patient with CCL in remission, who had been more axonal involvement and mild CSF pleocytosis are features that treated with Chlorambucil, suffered blurred vision, handwriting are different in dengue fever associated GBS as compared to typical difficulties, posture instability and speech disorders. Following a GBS. Computer tomography of the head, the patient was diagnosed with ischemic stroke. However, due to the progressive neurological doi:10.1016/j.jns.2019.10.1096 deterioration, the patient was admitted to the Neurology Clinic of University Hospital Pleven. On admission, bilateral amblyopia, horizontal and rotatory nystagmus, severe right-sided hemiparesis, facial asymmetry, dysarthria and dysphagia were established. The WCN19-1931 MRI revealed multiple sub- and supra-tentorial white matter lesions, highly likely due to leukoencephalopathy. CSF showed hyper Poster shift 02 - Autoimmune disorders - Part I/CNS infections - proteinorachie and positive PCR for the JC-virus. Despite the 21-day intensive care treatment, the patient ultimately had a fatal outcome. Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Conclusion PML is a rare disease that is difficult to diagnose, especially in Sympathetic dysregulation in the epilepsy patients with hippo- cases with non-typical clinical presentation. In immunocompromised campal sclerosis patients with deteriorating neurological signs MRI and JC-virus identification results allow early diagnosis of PML, improving the D. Zhuravlevab, M.A. Lebedevaac, A.V. Lebedevaab, A.M. Teplyshovaa,F. patient’s chance of survival. K. Ridera, Y.V. Solomatina, N.S. Semenovykha, A.A. Yakovlevad, R.G. Akzhigitova, A.A. Gudkovaab, A.B. Guekhtab doi:10.1016/j.jns.2019.10.1095 aMoscow Research and Clinical Center for Neuropsychiatry, Department of Neurology, Moscow, Russia bPirogov Russian National Research Medical University, Department of Neurology- Neurosurgery and Medical Genetics, Moscow, Russia WCN19-1925 cInstitute of General Pathology and Pathophysiology, Laboratory of Pathophysiology, Moscow, Russia dInstitute of Higher Nervous Activity and Neurophysiology of Russian Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Academy of Sciences, Department of Neurophysiology, Moscow, Russia Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Introduction Interictal autonomic dysfunction in people with epilepsy is Gullain Barre syndrome and dengue fever traditionally considered as the decrease of primarily parasympathetic ARTICLE IN PRESS

168 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx outﬂow. Yet, isolated sympathetic regulation is rarely investigated. We environmental factors. MS affect mainly Caucasian and is almost speculate that hippocampus might be interlinked with sympathetic absent in Black African and Indian.There is a gradient of increasing outﬂow. distribution from low to high latitudes worldwide.The Brazilian population is result of inter-ethnic mixture of Mediterranean Purpose European, African and Amerindian. More than 50% of the Brazilian We aimed to assess parasympathetic and sympathetic regulation population are brown or black by self –declaration according the in patients with focal epilepsy. Brazilian Institute of Geography and Statistics (IBGE).

Methods Objective We enrolled 22 patients with structural epilepsy (age 35,3±8,3 The aim of this study was to identify the European(EUR), African years [M±SD], 16 males) and 22 gender- and age-matched healthy (AFR) and Amerindian(AMR) ancestries on MS Brazilian patients controls. Patients were divided in two subgroups: with hippocam- living in Rio de Janeiro(RJ) city (22.3°S, 42.7°W) located at the pal sclerosis (HS+, n=12) and with other structural changes (HS-, Southeast region of Brazil where the MS prevalence is 20/100.000. n=10). We used time- and frequency-domain analysis of heart rate variability (HRV) and systolic blood pressure variability (sBPV) at Method rest and during orthostatic test including following parameters: A transversal study selected consecutively MS patients attended total power (TP), ranges of low (LF) and high frequency (HF), in the Hospital da Lagoa, the main center for MS treatment in RJ city baroreﬂex sensitivity (BRS), 30:15 ratio. Data are presented in ME in the year 2018.Patients completed a self-declaration race/color [IQR]. questionnaire according to IBGE (black, white, brown, indigenous or yellow) and information about EUR, AFR or AMR family (Grand- Results mother/Grandfather, Father/Mother). Patients with epilepsy revealed the decrease of all autonomic parameters compared to healthy controls. HS+ patients did not Results differ from HS- group in total autonomic and parasympathetic 197 MS patients were selected (157woman,40 men) of whom activity, but demonstrated prominent decrease of sympathetic 57.4% declared to be white, 34% brown, 7.1% black, and 1.5% activity at rest (LF of sBPV, 2,6 [1,72; 3,38] vs 5,75 [2,48; 8,15] indigenous. AFR ancestry familial were referred by 88/197 (44.7%), mmHg2, p=0,025; LF of HRV, 183,4 [76,3; 336] vs 400,9 [213,6; EUR by 65/197 (33%) and AMR by 19/197 (9%). 770,9] msec2, p=0,025) and BRS during orthostatic test (4,2 [3,48; 8,45] vs 6,9 [4,78; 10,03] msec/mmHg, p=0,047) compared to HS- Conclusion patients. Although most of the MS patients living in Rio de Janeiro city declared themselves white, a higher frequency of AFR ancestry was Conclusion found Impaired sympathetic as well as parasympathetic regulation could be observed in patients with focal epilepsy. Hippocampal doi:10.1016/j.jns.2019.10.1098 sclerosis is likely to be associated with decreased sympathetic activity. doi:10.1016/j.jns.2019.10.1097 WCN19-1954

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - WCN19-1942 Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Cognitive impairments study in symptomatic epilepsy and their Part I/dementia - Part I/epilepsy - Part I/health economics and new medical-psychological principles in Uzbekistan outcomes/neuroepidemiology and environmental and other N. Khasanova, G. Ernaeva, S. Kuranbaeva, G. Rakhimbaeva Prevalence of afro-Descendent and of European among patients Tashkent Medical Academy, Department of Neurology, Tashkent, with multiple sclerosis in the city of Rio de Janeiro Uzbekistan

a b c d R. Alvarenga , H. Alvarenga Filho , R. Guimarães , F.P. Carolino Objectives a Universidade Federal do Estado do Rio de Janeiro, Neurology, Rio de To use psychotherapeutic techniques in early diagnostics and Janeiro, Brazil correction of cognitive impairments in symptomatic epilepsy (cog- b Universidade Federal do Estado do Rio de Janeiro- UNESA, Neurology, nitive functions stimulating exercise). Rio de Janeiro, Brazil c Instituto Osvaldo Cruz, Research, Rio de Janeiro, Brazil Materials and methods d Universidade Federal do Estado do Rio de Janeiro, Neurology, RIio de 20 patients with various signs of symptomatic epilepsy were Janeiro, Brazil admitted to the Neurology Department of the TMA Clinic. Of them, 12 (60%) were male and 8 (40%) were women. The mean age was 35 Introduction ±5.7. Patients were classiﬁed into 2 groups: 1 group 11 patients The peculiar distribution of Multiple Sclerosis (MS) in the world paired with pharmacotherapy and psychotherapy (cognitive function is still a puzzle. The prevalence of MS varies worldwide according stimulating exercise); out of 9 patients of the control group, only the ethnic composition of the population and the inﬂuence of pharmacotherapy was used. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 169

Results seizure and neurorehabilitation management are equally important 4 patients (36%) in the Main group on the MMSE scale were light for future outcomes. dementia (20-27 points); 5 (45%) secondary (11-19 points); Two (18%) patients had severe (0-10 points) dementia. 4 (44%) patients Conclusion in the Control group were moderately light; 4 (44%) secondary Dyke Davidoff Masson Syndrome should be considered in patient schools; Severe dementia was detected in 1 (11%) patient. Compar- with classical features and brain imaging showing cerebral ison of the results after 1 month revealed that 5 patients (45%) of the hemiatrophy. Early diagnosis and prompt treatments would affect 1 main group had mild, 3 (27%) dementia and 3 (27%) patients did patient’s prognosis and quality of life. not have cognitive impairments. Five (55%) patients in the control group were mild, and four (44%) had moderate level dementia. The Key Words: Dyke Davidoff Masson Syndrome, Hemicerebral Atro- results of the study showed that cognitive functions improved by 3% phy, Seizure, Magnetic resonance imaging with stimulating exercises in the main group and improvement in 1.5% in control group patients p≤0.05. doi:10.1016/j.jns.2019.10.1100

Conclusion Early diagnosis and correction of cognitive impairments in symptomatic epilepsy, combined with pharmacotherapy, can also WCN19-1969 lead to improved patient survival and improved quality of life in patients with cognitive dysfunction. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and doi:10.1016/j.jns.2019.10.1099 outcomes/neuroepidemiology and environmental and other

"Multiple Sclerosis"- An uninspired name that shocks our patients and their relatives WCN19-1959 C.A. Sirbua, A.M. Preoteasab, I. Caloianuc, O.M. Sirbud Poster shift 02 - Autoimmune disorders - Part I/CNS infections - aDr. Carol Davila Central Military Emergency University Hospital, Part I/dementia - Part I/epilepsy - Part I/health economics and Neurology, Bucharest, Romania b outcomes/neuroepidemiology and environmental and other Romanian Academy, Research Institute for Quality of Life, Bucharest, Romania cRomGerMed, Clinic of Neurology, Bucharest, Romania Dyke Davidoff Masson Syndrome manifested by seizure in dDr. Carol Davila Central Military Emergency University Hospital, adolescent Neurosurgery, Bucharest, Romania

O. Tambun, A. Pradian, S. Handayani, H. Sugiharto Introduction Mohammad Hoesin General Hospital Palembang, Neurology, Palem- This name of the disease, "Multiple Sclerosis”, has a double bang, Indonesia negative impact on the patients. The term "sclerosis" is often mistaken for atherosclerosis by relatives. It is surprising to see young Introduction patients who have a disease called "sclerosis", a term commonly Dyke Davidoff Masson Syndrome is a rare case with the exact associated with elderly people with atheromatosis. On the other number of incidence is still remain unknown. Mostly found in hand, the term "multiple" frightens even more the patients. So these children and characterized by seizure,facial asymmetry,contralateral two combined words have a devastating impact on QoL. This adds to hemiparesis/hemiplegia and mental retardation. Brain imaging that of the disability caused by this demyelinating disease itself. shows cerebral hemiatrophy and calvaria thickening. Establishment Objectives: to demonstrate the negative impact of the name,, fi of diagnosis based on clinical and radiological nding Multiple Sclerosis” on QoL and the need to change it. We will not begin with the eternal "to the best of our knowledge this is the first Case report study", but we have not found such an approach in the literature yet. A postpartum 14 years old woman with seizure and loss of Methods: we conducted an exploratory pilot study, with a self- consciousness. From all oanamnesis, she was experienced seizure administrated survey, with a sample of 34 multiple sclerosis patients. followed by loss of consciousness one day after giving birth. At the The questionnaire included 16 items. Results: the vast majority of beginning of seizure, her head was facing to the right, right respondents consider important to change the name of the disease extremities stiffed then followed by stiffness of the whole body, and propose another name from the variants offered by the both eyes glared up, foaming at the mouth,tongue biting and bed questionnaire.Conclusions: even the health status of the patient is wetting. History of mental retardation, weakness of one side of the very good many years, the name of the disease could be a stressing body and facial asymmetry since childhood. History of seizure at 2 factor. The stigma related with,,Multiple Sclerosis” name is perceived years of age. History of asphyxia right after she was delivered. by our patients and the effects could be very serious. We will not end Neurological examination found left hemiparesis, left seventh nerve up with the famous "further research is needed" because this is paresis and global aphasia. Brain MRI showed right hemicerebral obvious to support the idea of changing the name of the disease. atrophy and thickening of the right parietooccipital calvaria. EEG revealed focal slowing and epileptic activity at left temporal. Based doi:10.1016/j.jns.2019.10.1101 on clinical and radiological finding, patient was diagnosed as Dyke Davidoff Masson Syndrome. Adequate treatment in controlling ARTICLE IN PRESS

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

WCN19-1976 absences induced by eye closure and visual stimulation, seen in an otherwise normal child. The seizure onset started at 2-14 year of age with a female predominance. The eyelid myoclonias occur as Poster shift 02 - Autoimmune disorders - Part I/CNS infections - repetitive, brief and fast myoclonic jerks of the eyelids, induced by Part I/dementia - Part I/epilepsy - Part I/health economics and eye closure and accompanied by high amplitude generalized spike- outcomes/neuroepidemiology and environmental and other and-wave or polyspike-and-wave at a frequency of 3-6 Hz, which classically terminated with complete elimination of light. We report Anthropology and neuroscience- Mixing the morphology with a case of 17 year old Saudi girl who was referred to our center as an culture intractable epilepsy. Her seizure started at age of 14 year old in form of frequent episodes of eyelid flickering, triggered by light exposure. L. Stroicaa, A.T. Ispasa, T. Marinescua, E. Tarta-Arsenea, A. Baciub, L.E. Each of these attacks would last for few minutes but some of them Gamanc can last for full day where she becomes less responsive. No reported aCarol Davila University of Medicine and Pharmacy, Anatomy, Bucha- any other seizure semiology. She has uncomplicated antenatal and rest, Romania perinatal history with an age appropriate developmental milestone. bFrancisc I Rainer Anthropology Institute of the Romanian Academy, No history of febrile convulsion or a family history of seizure. She has Medical Anthropology, Bucharest, Romania a good school performance. By admitting her to our Epilepsy cCarol Davila University of Medicine and Pharmacy, Biochemistry, Monitoring Unit, She was diagnosed as EEM, her medication were Bucharest, Romania adjusted and currently she is free of seizure. Despite being rare, EEM has unique characteristics, unless they are recognized, it can be In the last 30-40 years, the neurosciences became an emerging missed or lead to a misdiagnosis. domain of science, which has known a constant ascending evolution. doi:10.1016/j.jns.2019.10.1103 The pathophysiology of many neurological diseases have been or are being planned to be explained using the cellular and molecular changes in the brain. Recent studies tried to identify biomarkers which would help the diagnosis and treatment of the autistic patients. Unfortunately, despite the hope of scientists, patients and WCN19-1997 their relatives, the results were not as they expected, because the autism isn't a pure morphological disorder, but a mixture of brain Poster shift 02 - Autoimmune disorders - Part I/CNS infections - damage and sociocultural factors. This statement emphasizes the role Part I/dementia - Part I/epilepsy - Part I/health economics and that anthropology must play in neuroscience studies. Five centuries ago, the brain was clearly separated from the culture. But as studies outcomes/neuroepidemiology and environmental and other advanced, we understood that culture has played an active role in brain development. The definition of culture in anthropology is ICTAL kissing of dominant temporal lobe epilepsy wider than the common understanding, meaning everything that man created, reproducted and transmitted. Culture constrained A. Alkhotani, N. Alrishi human actions more than some biological needs, a statement that King Abdullah Medical City, Neurology, Makkah, Saudi Arabia can be proven if we think of Freud's century. Some scientist reported that culture can change even the neuron circuits and the function- Seizure semiology is a crucial element in evaluating epileptic ality of the brain. It's also known that some foods (which are also a patients to aid in the classification and localization to tailor the form of cultural evolution), for example mediterranean dishes, optimal management plan of the patients. There have been few increase the volume of gray matter. Therefore, instead of contradic- reported cases about unusual ictal phenomena of a kissing behavior ting and looking for each other errors, the neuroscientists and which was noticed to be more associated with the temporal lobe anthropologists should form multidisciplinary study teams, because, epilepsy, specially the non-dominant one and the female gender. in the end, the benefit of the patient is all that matters. Such a semiology can harbor a social embarrassment to the patient and impact their quality of life. We report a case of 23 year old, right- doi:10.1016/j.jns.2019.10.1102 handed Saudi gentleman, who presented with a history of frequent stereotypical semiology of generalized tonic clonic seizures. All of these attacks were preceded by impaired awareness accompanying spitting and kissing features. During the EMU admission, he WCN19-1996 developed three similar events, started with oral automatism, partially preserved consciousness and barking. After few seconds, would be followed by blowing kisses, spitting and upper extremities Poster shift 02 - Autoimmune disorders - Part I/CNS infections - automatism, to end up with secondary generalization. The EEG Part I/dementia - Part I/epilepsy - Part I/health economics and revealed a concomitant clear onset of left mesiotemporal focal outcomes/neuroepidemiology and environmental and other seizure. The MRI brain epilepsy protocol showed Left Orbito-frontal cavernoma. The PET scan showed hypometabolism of left temporal The eyes can diagnose an epileptic syndrome lobe. The WADA test was done and indicated the dominance of left temporal lobe. Based on these findings, he has been managed H. Abualela, A. Alkhotani, N. Alrishi, Y. Almatrafi, B. Alweshah medically and upon follow up he is reporting much improvement. By King Abdullah Medical City, Neurology, Makkah, Saudi Arabia reporting this case we contribute to the literature of Ictal kissing by an additional information regarding its association with the domi- Epilepsy with Eyelid myoclonias (EEM) which was previously nant temporal lobe and male gender, in contrary to most of reported known as Jeavons syndrome, has been recognized recently in the cases. latest 2017 Epilepsy classification by ILAE as an Epileptic syndrome. doi:10.1016/j.jns.2019.10.1104 It is characterized by daily eyelid myoclonias with or without ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 171

WCN19-2001 Background Syphilis remains a major public health problem. Neurosyphilis (NS) can affect up to 23% of untreated cases and stroke may be one of Poster shift 02 - Autoimmune disorders - Part I/CNS infections - its presentations. Our aims were to estimate frequency of syphilis Part I/dementia - Part I/epilepsy - Part I/health economics and serology request and NS in acute stroke patients in a large hospital in outcomes/neuroepidemiology and environmental and other southern Brazil.

Incidence of epilepsy and brain toxoplasmosis in hospital Mario Methods Rivas, Honduras All discharges with stroke and syphilis codes from 2015 to 2016 were reviewed. Sex, age, TOAST and Oxford stroke classification, A. Thompson, K. Villalobos mRankin and NIHSS scales, HIV status, syphilis serology (VDRL and National Autonomous University of Honduras, Clinical Medicine, San treponemal – CMIA) and cerebrospinal fluid (CSF) biochemistry were Pedro Sula, Honduras analysed. NS was classified according to CDC criteria.

Introduction Results fi The presence of neurological alterations is a frequent manifesta- We identi ed 1959 discharges (1119 cases), of which 85% tion in patients with HIV, especially focal onset epilepsy. The cerebral ischaemic strokes, 11% TIAs and 4% haemorrhages. CMIA was toxoplasmosis represents the main cause of occupational injury in an requested in 727 (65%) and VDRL in 1091 (97%) cases. CMIA was immunological patient for HIV related to a CD4 T lymphocyte count positive in 143 (13%; 95%CI 10.8% to 14.7%) of tested cases. Of 143 less than 100/uL. cases with serum positive CMIA, 94 (66%) had CSF analysis. Applying CDC criteria, 53/94 cases (56%; 95%CI 48% to 68%) fulfilled diagnostic fi Methods criteria to NS. Eight out of 53 (15%) had de nite NS based on positive A descriptive, cross-sectional study was carried out in which the CSF VDRL; remaining 45 (85%) tested negative for VDRL and had clinical characteristics associated with cerebral toxoplasmosis were presumed NS based in elevated CSF cell or protein. Presumed NS determined, as well as the alterations in the main encephalographic group behaved as non-NS group concerning clinical variables and alterations and the findings in neuroimaging studies (cranial CMIA CSF positivity. tomography or cerebral magnetic resonance). All patients underwent a complete medical history including history of neurological diseases. Conclusion Syphilis serology is frequent in patients with acute stroke in Results southern Brazil. Increased CSF protein or cells due to acute stroke A total of 26 patients with a diagnosis of HIV-AIDS infection and may increase false positivity of current NS criteria leading to NS over cerebral toxoplasmosis were evaluated, of which 15 (57.7%) were diagnosis and treatment. women. The age of the patients ranged between 23 and 65 years doi:10.1016/j.jns.2019.10.1106 (standard deviation 11,081). The manifestation by which the patients were admitted (main symptom) were altered state of consciousness (2, 7.7%), acute psychotic outbreak (4, 15.3%), headache (1, 3.8%), seizures (10, 38.5 %), focal motor deficit (5, 19.2%), vertigo (1, 3.8%), diarrhea (1, 3.8%), fever (1, 3.8%) and WCN19-2013 alteration of cranial nerves (1, 3.8%). Differents findings was found in patients: slow-wave, spike-slow wave complex, polymorphic slow Poster shift 02 - Autoimmune disorders - Part I/CNS infections - wave. Part I/dementia - Part I/epilepsy - Part I/health economics and Conclusion outcomes/neuroepidemiology and environmental and other Toxoplasmosis is a frequent opportunistic infection in patients with HIV, with epilepsy being one of the main manifestations of this. Crisoid Aneurysm of Scalp – A case report Neuroimaging studies (brain CT or brain MRI) allow diagnosis. The encephalogram study is frequently associated with alterations in T. Ramachandran, A. Prem Prakash, N. Balamurugan patients with toxoplasmosis. Timely diagnosis is associated with a Department of Pathology, Vinayaga Mission Kirupananda Variyar good prognosis in our patients. Medical College and Hospital and SIMS Chellum Hospital, Salem 636308, Tamil Nadu, India doi:10.1016/j.jns.2019.10.1105 Introduction Cirsoid aneurysms are rare arterio-venous fistulas of the scalp. A- V malformations may be congenital or traumatic in origin In 90% of WCN19-2009 the patients, the superficial temporal artery is the main supply to the fistula with only one dominant feeding artery in 71% of patients. Objective; Presenting a rare case of Giant Scalp swelling (Crisoid Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Aneurysm of Scalp) Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other Case details A 15-year-old boy presented with large swelling on the left side Prevalence of syphilis and neurosyphilis in a stroke population of the scalp associated with continuous headache for 1 year. Local examination revealed a pulsatile swelling on the left temporal region P.S. Silva, L.S. Costa, R.M. Castilhos, H.L. Moser Filho, R.T. Martins of the scalp. Dilated veins were noted in this region. On auscultation, Hospital Nossa Senhora da Conceição, Neurology, Porto Alegre, Brazil a bruit was detected over the lesion. Other systemic examination ARTICLE IN PRESS

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

was normal. Patient underwent computed tomography of the brain jF. Hoffmann-La Roche Ltd, Basel, Switzerland along with angiography. Aneurysmal dilated mass of vessels were kChugai Pharma USA LLC, Berkeley Heights, NJ, USA l detected in the left temporal region of scalp. A diagnosis of cirsoid Mayo Clinic, Rochester, MN, USA^ aneurysm of scalp was made. Patient underwent surgical excision of the mass. Histopathological examination shows numerous medium Background to large sized, thick and thin walled blood spaces lined by Interleukin-6 (IL-6) is implicated in the immune pathology of endothelium & filled with RBC’s. These blood spaces are separated neuromyelitis optica spectrum disorder (NMOSD). Satralizumab is a by fibrocollagenous tissue. Discussion and Conclusions: Surgical humanized recycling monoclonal antibody that binds to the IL-6 resection of the fistula is usually successful Untreated patients can receptor, inhibiting IL-6-driven proinflammatory signalling processes develop progressive scalp and facial cosmetic deformity. Differential associated with NMOSD.^ diagnosis of a pulsatile scalp swelling includes the following: superficial temporal artery aneurysm and Metastatic deposits from Objective follicular carcinoma of thyroid have to ruled out prior to making the Evaluate the efficacy and safety of satralizumab monotherapy diagnosis of cirsoid aneurysm. This case presented for its rarity of compared with placebo for relapse prevention in patients with Giant size. neuromyelitis optica (NMO) or NMOSD in the SAkuraStar study (NCT02073279).^ doi:10.1016/j.jns.2019.10.1107 Methods In this Phase 3, double-blind, placebo-controlled study, 95 patients were randomised 2:1 to satralizumab (120 mg s.c.) or WCN19-2025 placebo, administered at Weeks 0, 2, 4 and Q4W thereafter. ^ Concomitant immunosuppressant medications were prohibited. Patients either met 2006 NMO diagnostic criteria or were AQP4-IgG Poster shift 02 - Autoimmune disorders - Part I/CNS infections - seropositive and had experienced ≥1 episode of optic neuritis or Part I/dementia - Part I/epilepsy - Part I/health economics and longitudinally extensive myelitis. All had ≥1 documented relapse in outcomes/neuroepidemiology and environmental and other the year prior to screening. The primary endpoint was time to first fi protocol-de ned relapse (PDR).^ Efficacy and safety of satralizumab monotherapy for relapse prevention in neuromyelitis optica spectrum disorder (NMOSD): Results Results from SAkuraStar, a double-blind placebo-controlled Satralizumab monotherapy significantly reduced risk of PDR by phase 3 clinical study 55% compared with placebo (hazard ratio 0.45; 95% confidence interval 0.23–0.89; p = .018). The proportion of relapse-free patients A. Traboulseea, B. Greenbergb, J.L. Bennettc, L. Szczechowskid, E. Foxe, at Weeks 48 and 96 were 76.1% and 72.1% in the satralizumab group, S. Shkrobotf, T. Yamamurag, Y. Teradah, Y. Kawatah, P. Wrighti, H.C. and 61.9% and 51.2% in the placebo group, respectively. Satralizumab von Büdingenj, G. Klingelschmittj, A. Gianella-Borradorik, B.G. was well tolerated, and a similar proportion of patients in the Weinshenkerl satralizumab and placebo groups experienced adverse events. Rates aUniversity of British Columbia, Vancouver, Canada of serious infections were similar between groups. No deaths or b University of Texas Southwestern Medical Center, Dallas, TX, USA anaphylactic reactions were observed.^ cUniversity of Colorado School of Medicine, Aurora, CO, USA dSilesian Centre of Neurology, Katowice, Poland Conclusions eCentral Texas Neurology Consultants, Round Rock, TX, USA In this study, patients treated with satralizumab monotherapy fTernopil State Medical University, Ternopil, Ukraine had a significantly reduced risk of relapse compared with placebo. g National Center of Neurology and Psychiatry,National Institute of Satralizumab was well tolerated as monotherapy.^ Neuroscience, Tokyo, Japan hChugai Pharmaceutical Co., Ltd, Tokyo, Japan doi:10.1016/j.jns.2019.10.1108 iChugai Pharma Europe Ltd. London, United Kingdom ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 173

WCN19-2026 primary endpoint was time to first protocol-defined relapse (PDR). Pre- ^ specified subgroup analyses assessed treatment response by AQP4-IgG serostatus, prior therapy, and relapse history. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and Results outcomes/neuroepidemiology and environmental and other Overall, satralizumab reduced risk of PDR by 55% vs placebo (hazard ratio [HR] 0.45; 95% confidence interval [CI] 0.23–0.89; p = Efficacy of satralizumab as monotherapy in pre-specified sub- .018). There was a 74% reduction in PDR risk with satralizumab vs groups of SAkuraStar, a double-blind placebo-controlled phase 3 placebo in AQP4-IgG-seropositive patients (HR 0.26; 95% CI 0.11– clinical study in patients with neuromyelitis optica spectrum 0.63). In AQP4-IgG-seronegative patients, the HR was 1.19 (95% CI disorder (NMOSD) 0.30–4.78). HRs for additional subgroups are provided in Table 1. The proportion of relapse-free patients at Weeks 48 and 96 in the AQP4- Jeffrey L. Bennetta, B. Greenbergb, A. Traboulseec, L. Szczechowskid,E. IgG-seropositive and seronegative subgroups will be presented. Foxe, S. Shkrobotf, T. Yamamurag, Y. Teradah, Y. Kawatah, H.C. von Conclusions Satralizumab was effective in reducing risk of PDR in Büdingeni, G. Klingelschmitti, A. Gianella-Borradorij, B.G. patients with NMO or NMOSD, particularly in AQP4-IgG-seropositive Weinshenkerk patients. The study was not powered for subgroup analyses; aUniversity of Colorado School of Medicine, Aurora, CO, USA therefore, results should be interpreted with caution. bUniversity of Texas Southwestern Medical Center, Dallas, TX, USA cUniversity of British Columbia, Vancouver, Canada doi:10.1016/j.jns.2019.10.1109 dSilesian Centre of Neurology, Katowice, Poland eCentral Texas Neurology Consultants, Round Rock, TX, USA fTernopil State Medical University, Ternopil, Ukraine gNational Institute of Neuroscience, National Center of Neurology and WCN19-2058 Psychiatry, Tokyo, Japan ^ hChugai Pharmaceutical Co., Ltd, Tokyo, Japan Poster shift 02 - Autoimmune disorders - Part I/CNS infections - iF. Hoffmann-La Roche Ltd, Basel, Switzerland jChugai Pharma USA LLC, Berkeley Heights, NJ, USA Part I/dementia - Part I/epilepsy - Part I/health economics and kMayo Clinic, Rochester, MN, USA outcomes/neuroepidemiology and environmental and other

Background A study of clinical,radiological and thrombophilia profile in Interleukin-6 (IL-6) is implicated in the immune pathology of cerebral venous sinus thrombosis neuromyelitis optica spectrum disorder (NMOSD). Satralizumab, a humanized recycling monoclonal antibody that binds to the IL-6 D. Dave, F. Khan, S. Rohatgi, S. Nirhale, P. Rao, P. Naphade receptor, reduced risk of relapse in patients with NMO or NMOSD as Neurology, Dr. D.Y. Patil Medical College, Pune, India add-on therapy in the SAkuraSky study (NCT02028884) and as monotherapy in the SAkuraStar study (NCT02073279). Introduction A prospective, observational study was done at a tertiary care Objective hospital in Pune to describe the clinical, radiological and Evaluate the efficacy of satralizumab monotherapy vs placebo for thrombophilia profile of cerebral venous sinus thrombosis in an relapse prevention in pre-defined subgroups of the SAkuraStar study. Indian population.

Methods Methods SAkuraStar is a randomized, double-blind, Phase 3 study comparing 30 patients with clinical and MRI features suggestive of cerebral satralizumab monotherapy with placebo. 95 patients with NMO or venous sinus thrombosis (CVST), were studied with detailed clinical NMOSD, with ≥1 documented relapse in the year prior to screening, evaluation and pro-thrombotic work up. All were followed up monthly were randomized 2:1 to satralizumab (120 mg s.c.) or placebo. The for 6 months, to assess the response to therapy and clinical outcomes. ARTICLE IN PRESS

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

Results anti-convulsant medication and had no further seizures. At dis- Headache was the most common presenting symptom and charge, the patient had marked improvement with mild residual papilloedema was the most common neurological sign. The most weakness and was ambulating independently. common sinus involved was transverse sinus in majority of cases. In the unprovoked CVST subset of patients prothrombotic states were Conclusion ﬁ commonly identi ed. Provoked and idiopathic CVST accounted in ^Combined Hormonal Contraceptive NuvaRing devices likely have almost same percentage of patients in this study. prothrombotic potential comparable to oral contraceptives and increased recognition may promote earlier venous imaging and Conclusion result in better clinical care and outcomes of stroke patients. CVST is an important yet under recognized cause of intracranial hypertension and stroke in young. Clinical presentation is extremely doi:10.1016/j.jns.2019.10.1111 varied and a high index of suspicion is needed. Magnetic Resonance Imaging (MRI) brain with Magnetic Resonance Venography (MRV) is the current diagnostic modality of choice. Prothombotic states are common in cases of unprovoked CVST in an Indian population. WCN19-2092 Management with anticoagulants is safe and has excellent clinical ^ outcomes. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - doi:10.1016/j.jns.2019.10.1110 Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

Tumors masquerading as neurological diseases: A caution for clinicians in planning diagnosis and treatment WCN19-^2072 T.A. Sangeetha, S.R. Chandraa, V.A. Chandra Sasitharanb,P. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Mailankodya, H. Padmanabaa, H. Pendharkarc, P.K. Katragadda Sa,S. Part I/dementia - Part I/epilepsy - Part I/health economics and Krishna Pillaid, A. Rangarajan Sa outcomes/neuroepidemiology and environmental and other aNeurology, NIMHANS, Bengaluru, India bPediatrics Surgery, Govt TD Medical College, Alapuzha, India Don't put a ring on it! cerebral venous thrombosis with the cNeuroimaging and Interventional Radiology, NIMHANS, Bengaluru, combined hormonal contraceptive NuvaRing India dPaediatric Surgery,.Sri Avittom Thirunal Hospital-Govt Medical College, L. Jaffea, H. Mohsenb Trivandrum, India aNeurology, Tripler Army Medical Center, Honolulu, USA b Internal Medicine, Tripler Army Medical Center, Honolulu, USA Introduction Neurological diseases can be due to direct diseases of the CNS, Introduction PNS or a bystander syndrome of systemic diseases. Toxic, metabolic ^Combined Hormonal Contraceptive (CHC) vaginal rings are used & nutritional, immune-mediated consequences of clinically occult as an alternative to oral contraceptives with theoretical advantage of neoplasms produce a spectrum of neurological diseases, recognition lower risk of thromboembolism. However, recent studies have of which has therapeutic and prognostic importance. shown that CHC vaginal rings may be as thrombogenic as oral forms. Patients and methods Methods Children and adults who presented in the last ﬁve years with ^We present a case of complicated cerebral venous thrombosis neurological diseases and later could be diagnosed or attributed to (CVT) in a 29 year old using a vaginal contraceptive ring. neoplasms which were occult, were included for the study.

Case presentation Observation^ ^A 29 year old woman presented to the emergency department 28 patients were seen by the authors in the last 5 years with with generalized seizure preceded by worsening headaches and left neurological manifestations and hidden tumour. Maximum incidence upper extremity weakness and numbness. Non-contrast CT revealed was in above 60 years age followed by 21–40 years age group. The subarachnoid hemorrhage in bilateral frontal lobes. CT angiography commonest neurological presentation was muscle and nerve in- revealed an occlusive thrombus within the superior sagittal sinus volvement in adults and seizure in children. extending into the bilateral cerebral veins. MRI revealed infarcts in the adjacent cortices. The patient did not smoke and there was no Discussion^ history of prior vaso-occlusive events, hypercoagulable state, malig- In this study patients presented with a spectrum of neurological nancy, infection or head trauma. Evaluation for inherited or acquired illnesses secondary to underlying malignancy. One paediatric patient thrombophilia was negative. was saved from the fatal diagnosis of rabies who was found to have NMDA encephalitis secondary to ovarian teratoma. Another child Discussion with MERRF faced severe deterioration and fatality due to the use of ﬁ ^The patient was identi ed to have an etonogestrel/ethinyl contraindicated pharmacotherapy due to lack of awareness of estradiol vaginal ring (NuvaRing) for abnormal uterine bleeding. association of midline lipoma and MERRF. In the adult age group, She was treated with intravenous heparin and later transitioned to patients with polymyositis, hypokalemic periodic paralysis, demen- Apixaban. The vaginal ring was removed. She was maintained on tia, MND, SACD were found to have occult neoplasms. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 175

Conclusion^ WCN19-2125 Short duration, rapid progression, severe weight loss and poor response to treatment are the red ﬂags which point to diagnosis of an occult neoplasm. Poster shift 02 - autoimmune disorders - Part I/CNS infections - Part I/dementia - Part I/epilepsy - Part I/health economics and doi:10.1016/j.jns.2019.10.1112 outcomes/neuroepidemiology and environmental and other

Association with elevated level of inflammatory biomarkers and attention deficit in Korean farmers WCN19-2106 ^ G. Kimab, S. Baekab, H.W. Parkac, Y.J. Ryud, E.K. Kangab aCenter for Farmers' Safety and Health,Kangwon National University Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Hospital, Chuncheon, Republic of Korea Part I/dementia - Part I/epilepsy - Part I/health economics and bDepartment of Rehabilitation Medicine,Kangwon National University outcomes/neuroepidemiology and environmental and other Hospital, Chuncheon, Republic of Korea cDepartment of Rehabilitation Medicine, Gangwon Do Rehabilitation Male reprotoxicity effects of psychotropic drugs on men: Evalu- Hospital, Chuncheon, Republic of Korea ation of possible underlying mechanisms dDepartment of Pathology, Kangwon National University Hospital, Chuncheon, Republic of Korea A. Sellami, H. Njeh, S. Daoud, N. Chakroun, T. Rebai Embryology Biology of Reproduction, Medical School Sfax, Sfax, Tunisia Purpose Inflammation plays an important role in cognitive impairments. Various psychotropic medications have been implicated in the This study examined the association of elevated biomarkers, such as transient or permanent disturbance of reproduction in men. The C-reactive protein (CRP) and interleukin-6 (IL-6) concentrations, effects of chronic exposure to some psychotropic drugs on sperm with attentional deficit in Korean farmers. quality and male fertility are widely discussed. Drug safety tests are generally performed on female animals to eliminate effects on Methods reproduction. A total of 504 healthy Korean farmers (255 male, mean age 60 years) were enrolled from the Farmers' cohort for Agricultural work- Objectives Related Musculoskeletal disorders (FARM) study. Attention was evaluated by measuring the accuracy/error rate and speed when ^To analyze the effects of chronic exposure to psychotropic drugs on sperm quality and man fertility. using a computerized Go/No-Go test. Serum level of CRP and IL-6 were measured in specimens. Patients and methods Results ^We conducted a retrospective study of the Cas-Controle type including 70 patients investigated for couple's infertility. 39 patients Higher CRP and IL-6 levels were associated with decreased (Group G1) were treated with psychotropic drugs for a period accuracy rate (p b .001). The increased error rate in the Go test was exceeding 12 months and 31 patients did not take any drug (G2 related to an elevated CRP level (p b .001), while the increased error control group). For all patients, at least 2 spermograms have been rate of the No-Go test was related to an elevated IL-6 level (p b .001). investigated according to WHO guidelines. We excluded patients The correct response time was associated with an increased CRP with azoospermia or any factor that could alter sperm parameters. level in simple and NSAID-adjusted analysis. In the age-adjusted partial correlation analysis, higher CRP and IL-6 levels were Results associated with a decreased accuracy rate (CRP: Partial r [pr] = fi −0.171, p b .001; IL-6: pr = −0.196, p b .001). The increased error ^We noted a signi cant decrease in total and progressive mobility (p = .006;p = .02 respectively) and sperm vitality (p = .03) in G1 rate in the Go-test was related to the elevated CRP level (pr = 0.321, group. Hypospermia and necrospermia were significantly higher in p b .001), while increased error rate in the No-Go test was related to patients treated with psychotropic drugs (p = .04, p = .04 respec- the elevated IL-6 level (pr = 0.256, p b .001). tively). Sexual disorders such as erectile dysfunction and ejaculation were noted in 20.5% of G1 patients. An improvement of sperm Conclusion parameters followed by a spontaneous conception was observed in Elevated serum CRP and IL-6 were associated with attentional two patients after modification of the therapeutic protocol. decline, thus suggesting the involvement of inflammation in cognitive impairments in healthy individuals. Conclusion doi:10.1016/j.jns.2019.10.1114 ^The majority of psychotropic drugs are involved in spermatogen- esis alteration, sperm quality defects and sexual dysfunction. These effects are reversible and may be related to several intricate mechanisms including a direct gonadotoxic effect and endocrine disruption. doi:10.1016/j.jns.2019.10.1113 ARTICLE IN PRESS

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

WCN19-2160 cDepartment of Psychiatry, Gil Medical Center, Gachon University, ^ Incheon, Republic of Korea

Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Background Part I/dementia - Part I/epilepsy - Part I/health economics and Lethality of suicide is associated with medical and social outcomes/neuroepidemiology and environmental and other consequences. It is known that the suicide attempt of elderly is more serious with a three-fold completion rate than that of non- Sexual dysfunctions: The neglected disorders in clinical practice elderly. We investigated the risk factors for serious suicide attempt (SSA) in elderly and non-elderly groups. I. Afridi Jinnah Postgraduate Medical Centre- Karachi- Pakistan & Jinnah Sindh Methods and materials Medical University- Karachi, Department of Psychiatry & Behavioural We enrolled 370 patients who attempted suicide and acquired Sciences, Karachi, Pakistan demographic data, clinical information, and the level of seriousness of suicide with risk rescue rating scale (RRRS) in the emergency Introduction department of a tertiary hospital. RRRS is interviewer-administered The issue of sexual disorders in this part of the world has a scale composed of 10 items about risk and rescue of suicide attempt. peculiar pathetic situation. Seeking help regarding sexual problems Regression analyses were performed to find risk factors for SSA in is generally avoided even talking regarding such issues is considered elderly and non-elderly groups respectively. immoral and is a taboo. Moreover, alternative treatment is sought mainly from non-allopathic traditional practitioners such as herbal- Results ist, Hakims etc. who feels proud to restrict the old and outdated Among 370 patients, elderly suicide attempters were 37 (10%; knowledge with them. It was estimated that, in 1995, over 152 age = 74.41 ± 7.78). They had less education (p b .001), more million men worldwide experienced ED. For 2025, the prevalence of another medical disease (p b .001), and more probability to be men ED is predicted to be approximately 322 million worldwide. (56.1% vs 39.9%, p = .024). In regression analyses in the non-elderly In the past, erectile dysfunction was commonly believed to be group, old age (B = 0.090, p b .001), male (B = -0.038, p = .019), caused by psychological problems. But now it is known that, for most and schizophrenia (B = 0.074, p = .027) associated with higher risk- men, erectile dysfunction is caused by physical problems, usually rescue ratio score and interpersonal stress condition associated with related to the neuro-vascular supply. Many advances have occurred lower risk-rescue ratio score (B = -0.045, p = .006). In the elderly in both diagnosis and treatment of erectile dysfunction. group, however, no demographic or clinical variables were included in the regression model for risk, rescue, and risk-rescue ratio score. Ôbjective To determine the Co-morbid sexual dysfunctions in relation to socio-demographic factors. Conclusion Age, sex, impression of schizophrenia, and interpersonal stress Material & methods were associated with higher or lower lethality in the non-elderly This is a descriptive carried out at The Psychiatric clinic (TMC) in suicide attempters. However, no factors were associated with SSA in Karachi. Structured psychiatric interview was conducted on total the elderly group. These results may imply that different mechanism 5477 patients out of which 4.54% (249 cases) fulfilled the diagnostic underlies for the lethality in old age suicide and old age. criteria of Sexual dysfunction according to WHO ICD-10. Their mean age was 35.32 years with SD 9.8 years. doi:10.1016/j.jns.2019.10.1116

Conclusion Sexual dysfunctions particularly Erectile Dysfunction are common in men. It is imperative that a medical cause must be ruled out prior WCN19-2180 to making any sexual dysfunction diagnosis and the symptoms must ^ be hindering the person's everyday functioning. Poster shift 02 - Autoimmune disorders - Part I/CNS infections - doi:10.1016/j.jns.2019.10.1115 Part I/dementia - Part I/epilepsy - Part I/health economics and outcomes/neuroepidemiology and environmental and other

Upregulation of MIR-132-5P via nicotine enhances cell survival in PC12 cells by targeting the anti-apoptotic protein BCL-2 WCN19-^2177 T. Shresthaa, T. Takahashia,C.Lia, M. Matsumotob, H. Maruyamaa Poster shift 02 - Autoimmune disorders - Part I/CNS infections - a Department of Clinical Neuroscience and Therapeutics, Hiroshima Part I/dementia - Part I/epilepsy - Part I/health economics and University, Hiroshima, Japan^ outcomes/neuroepidemiology and environmental and other bNeurology, Sakai City Medical Center, Osaka, Japan

Risk factors for serious suicide attempts: Differences between in elderly and non-elderly groups Objective Activation of nicotinic acetylcholine receptors (nAChRs) results in J.M. Kanga, D.W. Kimb, S.K. Wooc, B.K. Yeonc, S.J. Choc neuroprotection via a poorly understood molecular mechanism. aDepartment of Psychiatry, Gil Medical Center College of Medicine, Herein, we aimed to investigate the effect of nAChR stimulation with nicotine on the regulation of miRNA expression and identify the Gachon University, Incheon, Republic of Korea^ bCollege of Medicine, Gachon University, Incheon, Republic of Korea molecular pathway involved in neuroprotection. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 177

Materials and methods Results miRNA expression proﬁling using microarray was conducted to 49 patients was enrolled in this study with 18(36.73%) males identify nicotine regulated miRNAs. The expression of miR-132-5p and 31(63.27%) females. Mean age of onset of the disease was was validated by RT-qPCR analysis. Cell viability was assessed after 44.84 years and age of onset of neurological manifestations was treatment of cells with nicotine, miR-132-5p mimic or its inhibitor. 46.65 years (18 yrs–71 yrs). Sjogren syndrome was seen as the The protein expression of CREB, Bcl-2 and Bax was determined by most common diagnosis. CNS manifestations were found more western blotting analysis. commonly than PNS. Mononeuritis multiplex was commonest form of PNS manifestation. Dorsal cord involvement with long segment Results myelitis was commonly seen. Most patients had increase in acute Using miRNA microarray we identiﬁed 37 miRNAs regulated by phase reactants and improved outcome on treatment. 32 patients nicotine. The microarray and the RT-qPCR results showed a 1.67-fold had follow-up of more than 6 months (up to 10 years). and a 1.5-fold increase in miR-132-5p, respectively, upon nicotine treatment. Immunoblotting revealed N2-fold increase in the phosphor- Limitations ylation of CREB with nicotine, peaking at 4 h. Cells treated with nicotine Follow up was limited in some patients. Due to waxing and showed increased viability from 35% to 54%, and the Bcl-2 immunore- waning course of the disease we may not have recorded the best activity increased by 1.4-fold. Overexpression of miR-132-5p increased clinical outcome. cell viability from 38% to 70% and increased the expression level of Bcl-2 by 3.9-fold. Inhibition of miR-132-5p decreased cell viability to 25%, doi:10.1016/j.jns.2019.10.1118 whereas no change was seen in Bcl-2. Bax expression remained unchanged following treatment with miR-132-5p mimic or inhibitor.

Conclusions WCN19-2205 Our results suggest that nAChR activation facilitates cell survival ^ by upregulating miR-132-5p, which upregulates the anti-apoptotic protein, Bcl-2. These results present miR-132-5p as a potential novel Poster shift 02 - Autoimmune disorders - Part I/CNS infections - therapeutic target to achieve neuroprotection via stimulation of Part I/dementia - Part I/epilepsy - Part I/Health economics and nAChR. outcomes/neuroepidemiology and environmental and other

The burden of diagnosis and treatment of neurological disorders doi:10.1016/j.jns.2019.10.1117 in sub-Saharan African countries; As the world accelerates the pace of change

C. Okwuolisa WCN19-^2203 Brain and Spine Foundation- Africa, Non Governmental Organisation, Abuja, Nigeria Poster shift 02 - Autoimmune disorders - Part I/CNS infections - Sub-Saharan Africa is recognizably a region grinding under the Part I/dementia - Part I/epilepsy - Part I/health economics and suffocative weight of various shades of distressing issues. And outcomes/neuroepidemiology and environmental and other bringing it into symphony with the rhythm of a rapidly-changing world becomes a daunting, but very necessary task. There are several Vasculitis and connective tissue disorders-study from Western negative indicators lucidly pointing to crippling deficits, and outright India-A neurology perspective morbidities in the continent's social orientations and societal fabrics. The welfare ladders are tottering, and nearly all institutional tools/ a a a b c N. Ramrakhiani , N. Jain , R. Dubey , A. Sharma , S. Kurapati vehicles for driving towards functional/sustainable social welfare a Department of Neurology,Fortis Escorts Hospital, Jaipur, India systems, are either rickety or outrightly ‘unroadworthy’. Health b ^ Department of Rheumatology, Fortis Escorts Hospital, Jaipur, India sector in sub-Saharan Africa particularly is an emergency situation. c ^ Department of Medicine, Fortis Escorts Hospital, Jaipur, India Neuroscience is one area of medical practice that is still filled with mystique and still evolving, but we are certain those in the developed Introduction countries have not the faintest idea of its ravaging impact in the Vasculitis and connective tissue disorder (CTD) can present to the developing countries of Africa. There is a very poor understanding of neurologist with symptoms involving central nervous system(CNS) pathophysiology of these disorders. 1 in every 5 Nigerian patients, and peripheral nervous system(PNS) sometimes 3 or 4 in an emergency section of any general hospital for example, has neurological/neurosurgical condition and many of them fail to seek early medical attention due to misconception, Mâterials and methods This is a retrospective study in which data of adult patients from myths and belief system. Ours is an environment affected to a larger last 10 years was collected from neurological practice inpatient extent by culture and religion. A lot of affected individuals are and outpatient records with clinical features suggestive of Vasculitis/ oblivious that they have neurological issues. When the fate of a CTD according to American College of rheumatology criteria. All people is hanging precariously on a precipitous threshold, no diagnosis was reviewed with consensus of two neurologists and a passionate Interface can be at ease. The distressing health needs of rheumatologist. Outcome was analyzed by comparing Modified sub-Saharan Africa's indigent communities should constitute emo- Oxford Handicap Scale scoring at the time of first visit and the last tional burdens, as well as moral responsibilities for all genuine and follow up visit. Infectious vasculitis, entrapment neuropathy and committed intermediaries. inflammatory myopathies without vasculitis were excluded. doi:10.1016/j.jns.2019.10.1119 Aims and objectives Âim of the present study was to study the spectrum of neurological manifestations and outcome in CTD/vasculitis. ARTICLE IN PRESS

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

WCN19-2262 N. Ubaidillaha, D. Sargowob ^ a Cardiology and Vascular Medicine, Dustira Army Military Hospital,^ Cimahi, Indonesia Poster shift 02-Autoimmune disorders-Part I/CNS infections-Part bCardiology and Vascular Medicine,Faculty of Medicine, Brawijaya I/dementia-Part I/epilepsy-Part I/Health economics and out- University,^Malang, Indonesia comes/neuroepidemiology and environmental and other Background Encephalocraniocutaneous lipomatosis with meningioma: A Endothelial dysfunction as prime factor contributing in advancing novel presentation of a very rare syndrome atherosclerotic disease. Circulating endothelial cells (CEC) is a reﬂection of endothelial damage or stress, contrary with endothelial M. Alqawasmeh, B. Aldabbour, K. El-Salem progenitor cells (EPC) roles in re-endothelialization and neovascu- Neurology, King Abdullah University Hospital, Irbid, Jordan larization. The link between heart disease and cerebrovascular disease is imminent as vascular is the main site of the problem.

Introduction^ Encephalocraniocutaneous lipomatosis “ECCL” is a rare, sporadic Purpose neurocutaneous disorder hypothesized to result from somatic This study was aimed to evaluate the difference ratio EPC and CEC mosaicism caused by lethal autosomal mutations. It is characterized between two groups of High risk atherosclerotic patient and stable by unilateral involvement of skin, eyes and central nervous system. angina pectoris as endothelial dysfunction predictor. There are nearly 60 cases reported in the literature. Methods This is preliminary data from a quasi clinical trial experimental Methods^ We report a first case of probable ECCL with meningioma. IRB study on 37 High risk atherosclerotic patients was determined based approval was obtained. on Framingham risk score 35 stable angina pectoris with pre-test and post-test design without control. The data was analyzed by paired t- test for parametric data and Wilcoxon test for non-parametric data. Case^ A 30-year-old male of average IQ presented with recurrent generalized seizures after years of medication-controlled epilepsy. He Results also displayed multiple left sided skin lesions on the head, torso and It show ratio EPC and CEC in high risk group 2.57 and in stable limbs, in addition to patches of alopecia and left sided lipomas, angina pectoris group was 4.1 (p = .000), from another ratio of total prompting evaluation for a possible neurocutaneous syndrome. EEG cholesterol and HDL 4.54; ratio LDL and HDL 2.72; ratio TG and LDL was unrevealing. Brain CT showed left parietal hypoattenuation with 2.85 in high risk group. In angina pectoris group ratio of total sulcal effacement, left fronto-temporal gyriform calcification, and left cholesterol and HDL 4.72; ratio LDL and HDL 2.94; ratio TG and HDL relative hemiatrophy. He had history of left mandibular cysts with 3.02. secondary osteomyelitis. MRI with contrast confirmed the presence of a dural-based enhancing left parasagittal mass. Post-excision cytopathol- Conclusion ogy with immunostaining revealed the mass to be fibrous meningioma, There are higher ratio from stable angina pectoris group than WHO grade I. Ophthalmological evaluation was unremarkable. Whole high risk group. spine MRI was unrevealing of lipomas. Upon review of literature, the diagnosis of probable encephalocraniocutaneous lipomatosis was made. doi:10.1016/j.jns.2019.10.1121 Sturge-Weber syndrome is a less-likely diagnosis. Biopsy of skin lesions is planned to confirm nevus psiloliparis.

Conclusion^ WCN19-2111 The findings of this case reflect the heterogeneity of ECCL as a ^ spectrum of disease. Our first reporting of ECCL with an intracranial Poster shift 03 - Autonomic nervous system disorders/history of neoplasm adds to the scarce literature on this syndrome and lays the ground for future expansion of current criteria. neurology/motor neuron disease/movement disorders/MS & Note: poster will include radiology, histopathology and lesion demyelinating diseases/neuromuscular disorders/vestibular dis- images. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1120 Efficacy of cladribine tablets 3.5 mg/kg in patients with relapsing multiple sclerosis aged above and below 45 years; clarity and clarity extension

G. Giovannonia, K. Rammohanb, S. Cookc, P. Soelberg-Sorensend,P. WCN19-^2288 Vermersche, B. Kellerf, E. Verdun di Cantognof aBlizard Institute- Barts and The London School of Medicine and Poster shift 02-Autoimmune disorders-Part I/CNS infections-Part Dentistry, Queen Mary University of London, London, United Kingdom^ I/dementia-Part I/epilepsy-Part I/Health economics and out- bDepartment of Neurology, MS Research Center,School of Medicine, comes/neuroepidemiology and environmental and other University of Miami, Miami, USA^ cNew Jersey Medical School, Rutgers-The State University of New Jersey, Differencies ratio epc and cec as endothelial dysfunction predic- Newyork, USA tor between high risk atherosclerotic group and stable angina dRigshospitalet, Department of Neurology, Danish MS Center, University pectoris patient of Copenhagen, Copenhagen, Denmark^ ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 179 eCHU Lille- LIRIC - INSERM U995- FHU Imminent,Université de Lille, funded central clinic at BMC. The normal range of serum levels of Lille, France vitamin D is 30–60 ng/ml (60–100 ng/ml for patients with multiple fKGaA n/a, Merck Healthcare, Darmstadt, Germany sclerosis).

Introduction Objectives In CLARITY, cladribine tablets 10 mg, (cumulative dose 3.5 mg/kg To correlate the level of serum vitamin D in multiple sclerosis [CT3.5] over 2 years [yrs]) were efficacious versus placebo in patients patients from Benghazi Multiple Sclerosis Clinics at BMC to their with relapsing multiple sclerosis (RMS). This was maintained score of disability using the Extended Disability Status Scale (EDSS). without further treatment in CLARITY Extension. The effect of CT3.5 was evaluated in patients with RMS aged N45 and ≤ 45 yrs. Methods Relying on the BMC Multiple Sclerosis Clinic Questionnaire and Methods patient records we collected the levels of vitamin D and EDSS scores CT3.5 and placebo treatment groups of CLARITY were stratified from 50 prospectively clinically reviewed patients. All the patients according to age (≤45 and N 45 yrs). Efficacy endpoints were were taking disease modifying agents. The questionnaire has medical qualifying relapse, all relapses, and MRI parameters. Equivalent ethics approval. Word Excel was used for the graphs and statistical analyses were conducted for patients who received CT3.5 in CLARITY analysis. and placebo in CLARITY Extension. Results Results 39 patients were female and 11 male. Age ranged 17-63 years. In CLARITY, 649 patients were ≤ 45 yrs. (CT3.5 N = 330; mean The mean age was 37 years. The mean vitamin D level in both age 34 yrs) and 221 were N 45 yrs. (CT3.5 N = 103; mean age 52 genders was 15.7 ng/ml, (females 16.1 ng/ml, and males 14.5 ng/ml). yrs). Annualised qualifying relapse rate (ARR) comparison at Week The mean EDSS score in both genders was 3.76 (females 3.71, males 96 showed that CT3.5 was associated with significant relative risk 3.95). reduction versus placebo in both age groups. ‘All relapse’ data were comparable for the same age groups, respectively. Numbers of Conclusion cumulative new T1 Gd + lesions at Week 96 were reduced with Patients in Benghazi have significantly lower than recommended CT3.5 compared to placebo in both age groups. Mean numbers of levels of vitamin D, and this correlated with a significant degree of active T2 lesions per patient per scan at Week 96 were also disability suggestive of secondary progressive disease. We highlight significantly reduced with CT3.5 versus placebo in both age groups this as a target for future nationwide campaigns and recommend (≤45 yrs.: −0.667 [95%CI −0.67, −0.50], p b .0001; N45 yrs.: -0.167 vitamin D supplementation added to multiple sclerosis treatment. [95%CI -0.33, 0.00], p b .0001), as were mean numbers of CU lesions. CLARITY Extension: mean numbers of qualifying relapses in patients Keywords: Vitamin D level, EDSS score, Multiple sclerosis, Benghazi, ≤45 yrs. (N = 76) and N 45 yrs. (N = 22) were 0.30 and 0.09, Libya respectively. doi:10.1016/j.jns.2019.10.1123 Conclusions CT3.5 reduced relapse and MRI lesion frequency in patients aged ≤45 yrs. and N 45 yrs. The CLARITY study: NCT00213135 The CLARITY Extension study: WCN19-0040 NCT00641537 doi:10.1016/j.jns.2019.10.1122 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 WCN19-0039 Update: Review of multiple sclerosis Poster shift 03 - Autonomic nervous system disorders/history of H.A. El-Zawawi neurology/motor neuron disease/movement disorders/MS & Benghazi Medical Center, Neurological Medicine, Benghazi, Libya demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Background Multiple sclerosis (MS) is an autoimmune demyelinating disease A prospective correlation study of the serum level of vitamin D to of the central nervous system the aetiology of which remains so far the EDSS score in patients with multiple sclerosis in Benghazi, unknown and which probably involves: genetic, immune, inflamma- Libya tory, as well as environmental factors.

H.A. El-Zawawi Objectives Benghazi Medical Center, Neurological Medicine, Benghazi, Libya A review of the current and past literature on multiple sclerosis to provide a concise and comprehensive educational resource. The aim Background being to present a simpliﬁed and clinically relevant summary of Vitamin D has been linked to the aetiology of several autoim- the main manifestations, and management of multiple sclerosis, with mune diseases including multiple sclerosis. In 2016 all patients an update on current theories of pathophysiology, aetiology; and diagnosed with multiple sclerosis in Eastern and other parts of Libya the basis behind the therapeutic agents used in current clinical were directed to an organized, database and treatment public sector practice. ARTICLE IN PRESS

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

Methods were associated with the presence of RBD. The most common dream The standard medical texts and journals relating to MS were phenomenon was fighting with human. The report of sleep-related consulted as well as reliance on own experience of patients injuries showed an injury to the patient was 16.3% an injury to bed attending the teaching hospitals, and clinics in Benghazi Libya with partner was 6.1%. a diagnosis of multiple sclerosis. Local and worldwide epidemiological published data were also reviewed. Conclusion We found that the prevalence of RBD in PD was 35% along with Results male gender, disease duration and disease severity may be the risk The incidence of multiple sclerosis varies worldwide. Eastern factors in our study. The dream content of RBD seemed to be passive Libya is an area of high risk MS incidence. The importance is or defending the attack rather than attack to the others. emerging of a genetic role in the already known immune pathogenesis of multiple sclerosis. Diet and light have regained importance in doi:10.1016/j.jns.2019.10.1125 the aetiology of the disease. Investigations are advancing and help to differentiate the disease from other mimicking conditions. Bio- markers help to define diagnosis and determine prognosis. WCN19-0077 Conclusions Multiple sclerosis is an important neurological disease with protean CNS manifestations which must be considered in the Poster shift 03 - Autonomic nervous system disorders/history of differential diagnosis of neurological symptoms at all ages. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Keywords: Multiple sclerosis, Pathophysiology, Clinical presenta- orders and nutritional disorders or intoxications tion, Management Impact of a high fructose diet in wistar rat – Metabolic and doi:10.1016/j.jns.2019.10.1124 behavioral disorders – Effect of supplementation of flaxseed

H. Elkaoui University Ibn Tofail, Biology, Kenitra, Morocco WCN19-0067 Background Poster shift 03 - Autonomic nervous system disorders/history of The deregulation of the energy balance by the malnutrition leads to the dysfunction of the neuro- The metabolic mechanisms but neurology/motor neuron disease/movement disorders/MS & these homeostatic problems can be corrected by nutritional supple- demyelinating diseases/neuromuscular disorders/vestibular dis- ments. In this study, we investigated the effects of flaxseed on orders and nutritional disorders or intoxications metabolism and anxiety- depressive-like disorders in high fructose- fed (23%) Wistar rat. Rapid eye movement sleep behavior disorder in Parkinson’s disease Methods One-month-old female Wistar rats were divided into four groups H. Tohma-Ngee of six rats: 1. Control: (30 g of standard food + 30 ml of tap water), Taksin Hospital, Brain and Cerebrovascular Center, Bangkok, Thailand 2. Control supplemented with flaxseed (27 g standard food + 7 g flaxseed + 30 ml tap water) 3. Fructose (30 g standard food + 23% Objective fructose) 4. Fructose supplemented with flaxseed (23 g standard To determine the prevalence of REM sleep behavior disorder food + 7gflaxseed + 23% fructose), after two months of diet, the rats (RBD) in patients with Parkinson’s disease and ascertain the clinical underwent a battery of tests to evaluate the affective behavior (Open characteristics and risk factors associated with RBD. Field, Elevated Plus Maze and Forced Swimming Test) and biochemical analyzes of glucose, cholesterol and triglycerides. Method We enrolled subjects with Parkinson’s disease who visited in Results neurology clinic at Prasart neurological institute from 1st January to High fructose diet inducesadverse metabolic effects (increases 31st December, 2017. Subjects and bed partner were interviewed blood sugar, cholesterol, triglycerides) and behavioral ones (depres- with Mayo Sleep Questionnaire to ascertain RBD and obtained all sion). Forflaxseed, it does not correct anxiety, but decreases demographic data and non-motor symptoms. The patients who were depression-like and metabolic disorders. confirmed to have RBD were later interviewed regarding the dream content. Conclusion Our results suggest that a high fructose diet induces metabolic Results and behavioral dysfunctions. Flaxseeds have improved these Forty-nine patients out of 140 PD patients (35%) in our cohort conditions. were defined as RBD. Patients with RBD were male predominance (53 vs 37; p = 0.042), longer disease duration (7.4 vs 5.2; p = Keywords: Flaxseed, Fructose, Wistar rat, Metabolic disorders, 0.003), higher scores of the modified H&Y stage, higher scores of the Affective behavior UPDRS part III (31.0 vs 18.0; P b 0.001), and higher dose of total LEDD (765.0 vs 426.0; P b 0.001). Non-motor symptoms were statistically significance in group of PD with RBD. Logistic regression doi:10.1016/j.jns.2019.10.1126 analysis showed only male gender and non-motor questionnaire ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 181

WCN19-0083 Multiple sclerosis (MS) is the most common immune-mediated inflammatory demyelinating disease of the central nervous system. The cause of MS is unknown. MS is not considered a hereditary Poster shift 03 - Autonomic nervous system disorders/history of disease; however, a number of genetic variations increase the risk. neurology/motor neuron disease/movement disorders/MS & MS can affect people of any age. It’s most commonly diagnosed demyelinating diseases/neuromuscular disorders/vestibular dis- between the ages of 20 and 50 years. The average age when MS orders and nutritional disorders or intoxications symptoms first appear is between the ages of 30–35 years. In this study, it was analyzed medical records of hospitalized Fear of dying related to Parkinson’s disease: Patients’ and family patients for the period 2016–2018 years. Selected patients with members’ view prescription of the disease 10 and more years. Special attention was given to the age when the first symptoms of MS appeared and how it R. Kozákováa,R.Bužgováa, M. Barb,M.Škutováab, P. Ressnerb,P. proceeded. The analysis of data obtained through the register Bártováb demonstrated that during the analyzed period there were 2.1 cases aUniversity of Ostrava, Department of Nursing and Midwifery, Ostrava, of MS per 100 000 per year registered. The average age of patients Czech Republic was 38.3 ± 10.6. It was more men (62,5%, mean age 35 ± 5,3 years). bThe University Hospital Ostrava, Neurologic Clinic, Ostrava, Czech Women formed 37,5% (66,67% of all women with mean age 44 ± 4,8 ’ Republic and 33,33%, mean age 26 ± 3,2). 74,6% of patients don t associate the debut of MS with any condition in anamnesis, but 25,4% of ones associate with frequent colds. From the first symptoms at the disease The aim of the research was to identify fear of dying in onset until final diagnose it passed 9 ± 3.5 years. In 25,7% cases, MS Parkinson’s patients (PD) and their family members and compare has started from a sense of legs numb, in other 23,4% with diplopy reasons of fear according to sociodemographic characteristics and and in 49,9% with a decrease of eyesight on one or both eyes. patients’ health status. The fear of dying was assessed with APND- Blindness appears in only one woman. EoLC questionnaire (Attitudes of Patients with Progressive Neuro- logical Disease to End-of- Life Care, part Fear of Dying – 9 items). The doi:10.1016/j.jns.2019.10.1128 sample consisted of 134 participants, patients with PD (mean age: 71.4 year) and their family members (mean age: 60.3) from the Czech Republic. Patients reported to have a greater fear (p b 0.0001) in the domain “Dependence/Loss of control” (mean = 79.1, 95%CI: 74.6–83.6) than in the domain “One’s own suffering” (mean = 69.3, WCN19-0108 95%CI: 64.6–74.0). Patients worried most about “Being a burden to ” others (mean = 8.2, SD = 2.2), this was most often true for Poster shift 03 - Autonomic nervous system disorders/history of widowers (p = 0.024) and patients without a care allowance (p = neurology/motor neuron disease/movement disorders/MS & 0.006). On the other hand, family members (p = 0.002) were more demyelinating diseases/neuromuscular disorders/vestibular dis- afraid of suffering of their significant other during dying (mean = 79.3, 95%CI: 74.9–83.7) than of “Dependence/Loss of control” (mean orders and nutritional disorders or intoxications = 71.9, 95%CI: 66.3–77.4). Family members feared most “fear of severe pain during dying” (mean = 9.1, SD = 1.6). Family members Association between acute transverse myelitis and acute motor of patients without a care allowance more often feared of axonal neuropathy case report dependence and loss of control (p = 0.031). Fear of suffering during dying was indicated more often by patients’ children rather than A. Ahmeda, M. Elmahalb, A. Musac their life partners (p = 0.021). Other differences were not found as aFaculty of Medicine, University of Khartoum, Physiology, Neurophysi- significant. In the light of the emotive impact of PD in both patients ology- Neurology, Khartoum, Sudan and caregivers, one recommendation is for clinicians to elicit more bSoba University Hospital, Faculty of Medicine, University of Khartoum, information about feelings, such as fear of dying. Neuroscience Centre, Neurology, Khartoum, Sudan cFaculty of Medicine, University of Khartoum, Physiology, Neurophysi- doi:10.1016/j.jns.2019.10.1127 ology, Khartoum, Sudan

We are reporting a case of a 17-year old male who presented with progressive lower limb weakness, sensory disturbance and WCN19-0089 urine retention of 2-weeks duration. There was no history of a preceding febrile illness and his past medical, family, social and drug histories were of no significance. On examination he was oriented in Poster shift 03 - Autonomic nervous system disorders/history of time, place and person, with normal cognition, speech, behavior and neurology/motor neuron disease/movement disorders/MS & memory. There was no cranial nerve involvement. Examination of demyelinating diseases/neuromuscular disorders/vestibular dis- the lower limbs revealed reduction in muscle tone, power was grade orders and nutritional disorders or intoxications zero in all muscle groups, reflexes were absent with an equivocal plantar. Sensory modalities were impaired in an ascending manner Clinical features of the debut and development of multiple up to the fourth thoracic segment level. Other systems examination sclerosis in Uzbekistan was unremarkable. On the basis of clinical judgment and MRI findings (diffuse D. Mirzaeva intramedullary heterogeneous signal of lower cervical and thoracic Family Hospital № 60, Neurology, Tashkent, Uzbekistan segments of the spinal cord), he was diagnosed as a case of transverse myelitis. Three days after receiving intravenous ARTICLE IN PRESS

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

Methylprednisolone, the patient developed difficulty in breathing WCN19-0115 and tachy-brady arrhythmias. Consequently a diagnosis of Guillain- Barré syndrome was made and confirmed using nerve conduction studies which showed severe motor axonapathy of the lower limb Poster shift 03 - Autonomic nervous system disorders/history of nerves. CSF analysis revealed high proteins {343 mg/dl, (normal neurology/motor neuron disease/movement disorders/MS & values 15–45 mg/dl)} without increase in CSF cell count and normal demyelinating diseases/neuromuscular disorders/vestibular dis- sugar concentration. The patient admitted to the ICU and was treated orders and nutritional disorders or intoxications by intravenous immunoglobulin G which resulted in remarkable improvement. Familial mediterranean fever and multiple sclerosis: A case report In conclusion we are reporting a rare association (the second case report in the literature) between transverse myelitis and acute motor E. Gözke, P. Dogan Ak, S.S. Tasdemir, Z. Aktan axonal neuropathy (AMAN). FSM Teaching and Research Hospital, University of Health Sciences, Department of Neurology, Istanbul, Turkey doi:10.1016/j.jns.2019.10.1129 Introduction Familial Mediterranean Fever (FMF) is a hereditary and inflammatory disease characterized by recurrent episodes of febrile WCN19-0114 polyserosities. Herein we present a case of multiple sclerosis associated with FMF.

Poster shift 03 - Autonomic nervous system disorders/history of Case neurology/motor neuron disease/movement disorders/MS & A 40-year-old female patient on colchicine therapy since she had demyelinating diseases/neuromuscular disorders/vestibular dis- been diagnosed as FMF four years ago because of attacks of orders and nutritional disorders or intoxications abdominal pain, was evaluated as for weakness in her right arm and leg. The patient also described, a few attacks of numbness involving her legs which spontaneously resolved for 2–3 days within Nitrosative stress in a group of Parkinson's disease patients from the previous one and a half year. On neurological examination northern India cranial area was intact. Her fundus was normal. Muscle strengths of the left upper and lower extremities were full, but at −5/5 in the G.N. Babu, M. Gupta, V.K. Paliwal right side. Deep tendon reflexes were normoactive. Plantar responses Sanjay Gandhi Post Graduate Institute of Medical Sciences, Neurology, were irrelevant bilaterally. Sensory deficit was not detected. Lucknow, India Cerebellar tests and gait were normal. On cranial MR, demyelinating ovoid plaques in periventricular white matter coursing perpendicu- Parkinsonian syndromes encompass a group of relatively hetero- lar to the corpus callosum were observed. A contrast-enhanced geneous disorders that may share some clinical features with plaque in the left frontal lobe was detected. Cervical MR revealed ’ fi fi Parkinson s disease. The identi cation of sensitive and speci c presence of plaques in the spinal cord. Her right side paresis biomarkers for Parkinson's disease (PD) poses an important clinical completely resolved after pulse steroids treatment with methylpred- challenge. Clinical features of PD are very wide, including both motor nisolone. The patient was followed up with immune modulator and non-motor symptoms. Because of this, PD overlaps other therapy. neurodegenerative diseases. In Progressive supranuclear palsy (PSP), the neurodegeneration is characterized by abnormal tau Conclusion fi pathology in the form of globose neuro brillary tangles, tufted When abnormal findings of central nervous system are encoun- astrocytes, coiled bodies, and threads, with a predominance of 4- tered in cases diagnosed as FMF apart from its typical symptoms, repeat (4R) tau isoforms. Multiple system atrophy (MSA) is a rare concomitant occurrence of multiple sclerosis should be kept in mind, adult-onset rapidly progressive fatal neurodegenerative disorder and the patient should be evaluated accordingly. characterized by the abnormal aggregation of misfolded α-synuclein primarily in oligodendrocytes. On the other hand, 3-nitrotyrosine (3- doi:10.1016/j.jns.2019.10.1131 NT) is a product of tyrosine nitration by ROS. It is considered as an inflammation as well as cellular damage marker. In the present study we wanted to evaluate the serum levels of 3-NT in PD, PSP and MSA patients with respect to controls. UK PDS Brain Bank criteria for the diagnosis of PD were applied to select the patients. For comparative WCN19-0123 studies on serum 3-NT levels, serum samples were also collected from the patients of PSP, MSA and healthy controls. All patients gave Poster shift 03 - Autonomic nervous system disorders/history of written informed consent. Serum levels of 3-NT were determined by neurology/motor neuron disease/movement disorders/MS & fi b ELISA. Results show that 3-NT levels were signi cantly (p 0.01) demyelinating diseases/neuromuscular disorders/vestibular dis- higher in PD patients with respect to control values. Elevated 3-NT orders and nutritional disorders or intoxications could cause dopaminergic neuron damage and thereby could have a potential use as a biomarker in PD patients. Neuroprotective therapy both for als and acute ischemic stroke doi:10.1016/j.jns.2019.10.1130 K. Abe, T. Yamashita, Y. Ohta, R. Morihara Okayama University, Neurology, Okayama, Japan ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 183

Although the underlying mechanism of amyotrophic lateral Results sclerosis (ALS) has not yet been fully clarified, several reports have 19 patients showed abnormal radial and/or sural SNAPs. We implicated the involvement of oxidative stress under selective death analyzed SRARs of 18 patients with neuropathy excluding one of motor neurons in both ALS patients and animal models. Edaravone patient without radial SNAP. Eleven patients (61%) showed ≦0.3 is a free radical scavenger, which is the first clinical drug for SRAR. The time course changes of SRAR had a tendency to decrease. neuroprotection in the world which has been approved from 2001 in Furthermore, 3 of 6 patients had turned into ≦0.3 from N0.3. most ischemic stroke patients in Japan, and some of China and India. A recent multicenter prospective double-blind placebo-control Discussion clinical trial with edaravone for ALS patients conducted in Japan Although the first assessment showed both features of showed a positive effect for delaying the clinical score (ALS FRS-R) neuronopathy and length-dependent axonopathy in 18 hSCA during the 24 weeks of examination. This clinical benefitof patients, the second assessment showed significant decrease of edaravone was shown as an add-on therapy after anti-glutamatergic SRARs in 8 patients, and SRAR N 0.3 had turned into SRAR ≦ 0.3 in 3 riluzole. These data strongly suggest a potential underlying mecha- of 6 patients. Although it has been reported SRAR is useful to nism of oxidative stress in ALS and a clinical delay by a free radical distinguish length-dependent axonopathy from neuronopathy, the scavenger. These translational studies on a free radical scavenger present study clearly demonstrated the limitation of single SRAR Edaravone allowed governmental permissions both for acute ische- assessment, because the SRARs may be significantly changed during mic stroke after 2001 and for ALS after 2015. Edaravone was the disease progression in every single patient. It may be difficult to approved for ALS at 2015 in Japan, 2016 in Korea, and 2017 in USA. distinguish neuronopathy from length-dependent axonopathy by the Use of Edaravone greatly reduced hemorrhagic transformation SRAR when the impairment of axonal transport causes a dying-back accompanied by tissue plasminogen activator (tPA) treatment, and process from the nerve terminals in neuronopathy. may also extend therapeutic time window with tPA therapy for more than 4.5 h in human stroke patients for preserving neurovascular doi:10.1016/j.jns.2019.10.1133 unit (NVU). An intensive Edaravine therapy for 3 days now showed a favorite recovery in 3 European countries. doi:10.1016/j.jns.2019.10.1132 WCN19-1703

Poster shift 03 - Autonomic nervous system disorders/history of WCN19-0124 neurology/motor neuron disease/movement disorders/MS & 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 & Acute hemorrhagic leucoencephalitis (AHLE) - A rare variant of demyelinating diseases/neuromuscular disorders/vestibular dis- ADEM orders and nutritional disorders or intoxications S. Pujari, R. Kulkarni Electrophysiological evaluation of peripheral neuropathies in Deenanath Mangeshkar Hospital and Research Center, Neurology, Pune, hereditary spinocerebellar ataxia India

a a a b a D. Yamamoto , K. Ikeda , B. Hirose , Y. Asada , S. Shimohama ,E. Introduction c c d b Tsuda , T. Hozuki , R. Yamauchi , T. Imai Acute hemorrhagic leucoencephalitis (AHLE), a rare variant of a Sapporo Medical University School of Medicine, Neurology, Sapporo, ADEM, has presentation different from the usual ADEM, and the Japan situation may pose as a diagnostic challenge. bSapporo Medical University Graduate School of Health Sciences, Disability Studies of Peripheral Nerve and Muscle, Sapporo, Japan Aim c Sapporo Shirakabadai Hospital, Neurology, Sapporo, Japan To report AHLE, its clinicoradiological manifestations, process of d Sunagawa City Medical Center, Neurology, Sunagawa, Japan diagnosis and prognosis.

Objective Method and results Peripheral neuropathies often occur in hereditary spinocerebellar Two young patients presented with subacute hemiparesis ataxia (hSCA), but the features of nerve involvement have not been (evolved over 5–7 days); and confusional state. Initial work up of fully elucidated. The aim of this study is to evaluate the prevalence of both patients showed a thalamic/diencephalomesencephalic lesion. peripheral neuropathies in hSCAs and classify the underlying Both patients underwent extensive workup with collagen vascular pathologies into length-dependent axonopathy and neuronopathy disease and vasculitis profile, autoimmune encephalitis panel, based on “Sural/Radial Amplitude Ratio” (SRAR). acquaporin-4 antibody, which were normal. CSF showed raised proteins as the only abnormality in first, and was completely normal Methods in the second patient. CSF viral PCRs, toxoplasma immunoglobulilns, We evaluated 33 patients with hSCA. We measured sensory cryptococcal antigen were also negative. Both patients received nerve action potentials (SNAPs) of radial and sural nerve to intravenous methylprednisolone with only transient improvement calculate SRAR. According to the previous study, we recognized a followed by worsening in both sensorium as well as motor deficits. SRAR ≦0.3 as length-dependent axonopathy, and N0.3 as Repeat MRIs in both showed new large lesions in cortex as well as neuronopathy. white matter and hemorrhagic conversion of the thalamic lesions. ARTICLE IN PRESS

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

Both patients did not improve even after plasmapheresis. Brain WCN19-0126 biopsy of both patients showed demyelination, infiltration by macrophages(some showing ingested myelin) and areas of hemor- Poster shift 03 - Autonomic nervous system disorders/history of rhage. There was preservation of axons. A diagnosis of AHLE was neurology/motor neuron disease/movement disorders/MS & confirmed. Both patients succumbed rapidly and died in a span of 5– 7 days inspite of all standard treatment. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Conclusion AHLE is a hyperacute variant of ADEM, rare, forming 2% of all Prevalence and natural history of X-linked dystonia Parkinson- ADEM cases. It shows lesions with hemorrhages, more edema and ism in Koronadal City, South Cotabato mass effect. Histology shows hemorrhagic foci with fibrinoid necrosis of vessel walls in addition to macrophagic and neutrophilic M.D.F. Tamayoa, R. Suarez-uyb, J. Tamayoc, D.M.L. Buhatd, D. Jamorae infiltrates. Prognosis is worse than ADEM, with 30% mortality. aThe Medical City, Neurology, Pasig, Philippines bDr. Arturo P. Pingoy Medical Center- Koronadal City- South Cotabato, Neurology, Koronadal, Philippines doi:10.1016/j.jns.2019.10.1134 cRoel I Senador Memorial Hospital- South Cotabato, Internal Medicine, Tupi, Philippines ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 185 dSt Anthony Medical Hospital, Neurology, Marikina, Philippines months. Only those with 3 or more relapses were selected for this eMovement Disorders Center and Section of Neurology, International presentation. Institute for Neurosciences, St Luke’s Medical Center –Quezon City and Global City-Philippines, Neurology, Quezon City, Philippines Results A total of eight patient's data were selected. There were seven Introduction Africans and one Asian with two females and six males. The mean X-linked Dystonia Parkinsonism is an adult-onset, progressive, age was 63 years. [Video presentation of three relapses with the debilitating movement disorder, manifesting predominantly with repeat high associated uric acid level each time in ALL IN THE SAME dystonia in combination with Parkinsonism. It was ﬁrst reported in PATIENT WILL BE SHOWN]. 1975 among males in Panay Island, Philippines. The region of South Cotabato in Mindanao with its capital Koronadal City, is populated by Conclusion migrants from Panay Island. The estimated population of the region Benign positional vertigo associated with hyperuricaemia can is 912,957 as of 2015 and the major ethnicity, comprising 51%, are present with many relapses in the SAME patient with high urate from the Panay Island. This paper calls attention to this migration each time. and aims to identify cases and describe the clinical picture of XDP in doi:10.1016/j.jns.2019.10.1136 Koronadal City.

Methodology A descriptive study using the screening questionnaire for X- linked Dystonia Parkinsonism was used per barangay to look for WCN19-0133 possible cases. Cases were conﬁrmed through a one on one interview and assessment by a movement disorder specialist. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Discussion, conclusion and recommendations demyelinating diseases/neuromuscular disorders/vestibular dis- From those screened by barangay health workers, four cases of X -linked dystonia Parkinsonism from Koronadal City were seen. They orders and nutritional disorders or intoxications were all male, presenting with generalized dystonia. The phenom- enology of these cases are similar to the 2011 study involving 312 History of neurosciences from diverse contributions patients. Another 11 patients from other municipalities of South Cotabato came also for evaluation. Hence an expansion of this study A.P. Adam to involve the entire region of South Cotabato is warranted to University of Nairobi, Medicine, Nairobi, Kenya provide a more accurate picture of the prevalence and natural history of the disease in the region. Background Our knowledge of science today is built upon thousands of years doi:10.1016/j.jns.2019.10.1135 of work accumulated throughout ancient civilizations. The historical “collective learning” by people of different languages, faiths and cultures has laid the foundation for modern science. Ibn al-Haytham the father of optics and the experimental method, in the tenth WCN19-0132 century was the ﬁrst historical scientist to argue that vision occurs in the brain rather than the eyes in his Book of Optics (Edition III) [1].

Poster shift 03 - Autonomic nervous system disorders/history of Results neurology/motor neuron disease/movement disorders/MS & The author found: demyelinating diseases/neuromuscular disorders/vestibular dis- 1. An ancient statement that if the skin is completely burnt a new orders and nutritional disorders or intoxications skin is necessary for pain perception. This acknowledged the scientific fact the nerve endings for pain are located in the skin. Third or more relapses of BPV associated with hyperuricaemia: A 2. Another ancient statement that hot fluids do not induce pain in the report of 8 cases with video presentation stomach until they perforate its wall and they enter the peritoneal cavity. This acknowledged the scientific fact of the abundance of A.P. Adam sensory nerve endings for pain in the peritoneum. University of Nairobi {rtd], Medicine, Nairobi, Kenya 3. Another reference of the functions of the forebrain.

Material and methods Conclusion I accessed my neurology clinic database between 1 January 2008 There is still more work required to be done to uncover some to 31 December 2018 situated at doctors plaza clinic at Aga Khan interesting references to neurological and other sciences from old University Hospital Nairobi, all patients with Benign Positional writings. Vertigo [BPV] and high uric acid level in the blood were collected. BPV was diagnosed with a positive Dix-Hallpike manoeuvre with all References is its characteristics.. Each patient underwent Epleys manoeuvre and ﬁ then was put allopurinol 300 orally once a day with con rmation [1] B. Steffens, Ibn al-Haytham: First Scientist, Chapter 5 Morgan Reynolds three weeks later that uric acid levels had normalised. However due Publishing, Greensboro, NC, 2006 (ISBN 1599350246). to poor compliance or fatigue some patients stopped treatment after few months and had relapses. All these patients had a high blood uric doi:10.1016/j.jns.2019.10.1137 acid level, during relapses after stopping allopurinol for 5 to 8 ARTICLE IN PRESS

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

WCN19-0155 dFudan University, Institute of Neurology, Huashan Hospital, Institutes of Brain Science, State Key Laboratory of Medical Neurobiology, Shanghai, China Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Background demyelinating diseases/neuromuscular disorders/vestibular dis- TCM has some advantages over western medicine in delaying the orders and nutritional disorders or intoxications progress of MS/EAE due to its characteristics of multiple targets, overall regulation and less toxic side effects. Qi deficiency and Blood Seropositive myasthenia gravis severity and antibodies profile stasis syndrome is one of typical phenomenon in MS/EAE. AST-IV can significantly relieve clinical symptoms of EAE by supplementing qi A. Alkhotania, N. Alrishib, A. Alkhotanic and promoting blood flow. aUMM Alqura University, Medicine, Makakh, Saudi Arabia bKING Abdulla Edical City, Neurology, Makkah, Saudi Arabia Objective cUm Alqura University, Pathology, Makkah, Saudi Arabia To investigate the efficacy and mechanism of AST-IV in the treatment of EAE. Myasthenia Gravis (MG) is an acquired autoimmune disease of the neuromuscular junction. Patient with MG present with fatigable Material and methods muscle weakness. It is resulted from impairment of neuromuscular Female C57BL/6 mice were induced to establish EAE model by transmission. The majority of MG patients have a positive antibodies. myelin oligodendrocyte glycoprotein peptides (MOG35–55), which The most common antibody is antibodies against acetyl choline were randomly divided into EAE group, high-dose and low-dose receptors (AChR) and less commonly antibodies to muscle specific AST-IV group. After 3 days of modeling, the mice were injected with kinase antibodies (MUSK) and antibodies against low density drug till day 28. High-dose and low-dose AST-IV group were lipoprotein receptor related protein 4 (LRP4). intraperitoneally injected with AST-IV 30 mg/kg/day and 15 mg/kg/ This study aimed to correlate the severity of MG to their anti day respectively. The injection of PBS was set up as control in a AChR and or anti-MUSK antibody status. We reviewed the MG similar manner. patients in King Abdullah Medical City from period 2011 to 2019. Out of the total 27 MG patients, 16 were seropositivity. The severity was Results fi correlated with antibodies status. We found 87.5% of those patients High-dose and low-dose AST-IV could signi cantly reduce the to be positive to AChR antibodies only while 12.5% were positive to average highest clinical score and alleviate the clinical symptoms of fl both Anti-AChR and Anti-MuSK. Thymoma was found in 21% of EAE. AST-IV inhibited the in ammatory cells in spinal cord. Flow fi single seropositivity and in 100% with dual seropositivity. Crises cytometry analysis showed that AST-IV signi cantly increased the + + + β+ were was seen in 21% of single seropositivity patients while 100% in proportion of CD4 CD25 and CD4 TGF- , decrease the + + patients with dual seropositivity. Patients with dual seropositivity proportion of CD4 IL17 , inhibiting the expression of TLR4, Myd88 have more aggressive disease and required prolonged ICU admis- and NF-kappa B/p65 in spinal cord. sions. Our Observational study concluded that having dual seropositivity may carry a more aggressive course of the MG and an Conclusion fl association to Thymoma. More clinical trials are needed to confirm AST-IV is effective on EAE by regulating the in ammatory the severity of MG presentation and dual seropositivity. pathway and maintains the balance between Th17 cells and Treg cells. (NNSF of China 81473577, Collaborative Innovation Center doi:10.1016/j.jns.2019.10.1138 Project of Astragalus Resource Industrialization and Industrial Internationalization-hqxtcxzx2016-020. Ma and Xiao are corresponding authors).

doi:10.1016/j.jns.2019.10.1139 WCN19-0180

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-0188 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 & The preventive and therapeutic effects of astragaloside IV (AST- demyelinating diseases/neuromuscular disorders/vestibular dis- IV) on experimental autoimmune encephalomyelitis in mice orders and nutritional disorders or intoxications

J.C. Liua, H.Z. Zhanga, W.J. Guoa, Z. Chaia, J.Z. Yuab, C.Y. Liub, J.J. Anti-glutamic acid decarboxylase positive stiff person syndrome Huangac, L. Fengb, B.G. Xiaod, C.G. Maab treated with immunoglobulin in a 38-year old filipina: A case aShanxi University of Chinese Medicine, The Key Research Laboratory of report Benefiting Qi for Acting Blood Circulation Method to Treat Multiple Sclerosis of State Administration of Traditional Chinese Medicine, Scientific and Technological Innovation Team of Integrated Chinese J.A.L. Perez, L. Damian and Western M, Jingzhong, China St. Luke's Medical Center, Institute for Neurosciences, Quezon City, bShanxi Datong University, Institute of Brain Science, Shanxi Key Philippines Laboratory of Inflammatory Neurodegenerative Diseases, Datong, China cGeneral Hospital of Datong Coal Mining Group, Dept. of Neurosurgery, Stiff Person Syndrome (SPS) is a rare autoimmune neuromuscular Datong, China disorder that impairs GABAergic inhibition of skeletal muscle. It is a ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 187

debilitating disease that leads to muscle rigidity, spasms and poor S. Gulera, N. Turanb quality of life. The syndrome is managed symptomatically with GABA aTrakya University, Department of Neurology, Edirne,̇ Turkey agonists while clinical trials of immunoglobulin and rituximab have bTrakya University, Faculty of Medicine, Biostatistics, Edirne,̇ Turkey shown promise of longer lasting relief. We present a case of Stiff Person Syndrome in a 38-year old Background and purpose Filipina presenting with two years’ history of progressive axial and Our aim was to modify the Restless Legs Syndrome Quality of Life peripheral muscle rigidity. There were associated painful spasms (RLS-QoL) questionnaire advanced by Abetz (Health Qual Life triggered by startling visual, auditory and tactile stimuli. Physical Outcomes 3:79, 2005) and to analyse the validity and reliability of examination showed rigidity of the neck, back, abdomen, arms and the questionnaire. legs. The rest of the neurologic examination was unremarkable. Blood examinations showed diabetes mellitus and elevated anti- Methods glutamic acid decarboxylase titers (1,708 IU/mL, cut-off N5 IU/mL). Two hundred and one consecutive patients with RLS and forty- Surface electromyography demonstrated continuous involuntary three control subjects were included in the study. Permission activation of the lumbar paraspinals, rectus abdominis and ster- regarding the translation and validation of the RLS-QoL question- nocleidomastoids (Figure 1). There was no myotonia, neuromyotonia naire was obtained. The translation was conducted according to the or denervation changes. guidelines provided by the publisher. Imaging of the brain and spine was normal. Electroencephalo- gram did not show any epileptiform discharges. Screening for Results malignancies was unremarkable. There was short-term reduction in For the RLS subjects, the mean Insomnia Severity Index (ISI) stiffness with clonazepam (2 mg/tab 1 tab thrice daily), but titration score, the International Restless Legs Syndrome Severity Rating Scale was limited by dose-dependent somnolence. Intravenous immuno- (IRLSSG) score and the computed score of the RLS-QoL questionnaire globulin (2 g/kg over 5 days) provided further reduction in stiffness were 22.60 ± 3.39, 24.83 ± 5.28 and 45.93 ± 17.62, respectively. and improved quality of life for as long as three months after Among the RLS subjects without insomnia, the mean (±standard infusion. No adverse events were encountered. deviation) ISI score, IRLSSG score and computed score of the RLS-QoL questionnaire were 6.67 ± 2.34, 15.11 ± 4.03 and 41.93 ± 16.12, doi:10.1016/j.jns.2019.10.1140 respectively. A significant difference was identified between both groups on all scores (ISI: p = 0.001, RLS: p = 0.001). According to a correlation analysis, a significant correlation was identified between the ISI and IRLSSG or RLS-QoL scores (r = 0.513, p = 0.001 and r = WCN19-0204 −0.383, p = 0.001, respectively). Most items also exhibited a strong correlation with each other. The internal consistency determined by Cronbach's alpha indicated an extremely good correlation (0.975). Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Discussion demyelinating diseases/neuromuscular disorders/vestibular dis- These findings suggest the Turkish version of the RLS-QoL orders and nutritional disorders or intoxications questionnaire is a valid and reliable tool for the assessment of the quality of life in patients with RLS. Turkish version of the Johns Hopkins restless legs syndrome quality of life questionnaire (RLS-QOL): Validity and reliability doi:10.1016/j.jns.2019.10.1141 study ARTICLE IN PRESS

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

WCN19-0205 A. Kulakowskaa, K. Kapica-Topczewskaa, J. Tarasiuka, F. Collinb,M. Kwasniewskib, W. Brolac, H. Bartosik-Psujekd, J. Kochanowicza aMedical University of Bialystok, Department of Neurology, Bialystok, Poster shift 03 - Autonomic nervous system disorders/history of Poland neurology/motor neuron disease/movement disorders/MS & bMedical University of Bialystok, Centre for Bioinformatics and Data demyelinating diseases/neuromuscular disorders/vestibular dis- Analysis, Bialystok, Poland orders and nutritional disorders or intoxications cSpecialist Hospital in Konskie, Department of Neurology, Konskie, Poland d Validation Turkish version of the simple screening vehicle for Clinical Hospital No. 2 in Rzeszow, Medical Faculty, University of early diagnosis of advanced Parkinson’s disease in daily practice: Rzeszow, Neurology Clinic with Brain Stroke Sub-Unit, Rzeszow, Poland The CDEPA questionnaire Background S. Gulera, N. Turanb The aim of study was to collect and analyze data on relapsing- aTrakya University, Department of Neurology, Edirne,̇ Turkey remitting multiple sclerosis (RRMS) patients receiving disease bTrakya University, Faculty of Medicine, Biostatistics, Edirne,̇ Turkey modifying therapies (DMTs) in Poland.

Material and methods Background and purpose The observational, multi-center with prospective data collection We aimed was to modify to validate a simple screening tool, the study included all RRMS patients receiving DMTs reimbursed by the CDEPA questionnaire (“Cuestionario De Enfermedad de Parkinson National Health Fund (NFZ) in Poland, monitored by the Therapeutic Avanzada” [Questionnaire for Advanced Parkinson’s Disease (APD)]), Program Monitoring System (SMPT). The demographic profile, for the identification of APD in daily practice and to analyse the disability status and treatment modalities were analyzed. validity and reliability of the questionnaire. Results Methods Data from 12,341 patients were collected, including 70.1% women One hundred and twenty consecutive patients with Advanced and 29.9% men, therein 11,653 patients in the first-line and 1026 in Parkinson’s Disease (45% were women, mean age was 69.3 ± 11.6 second-line therapeutic DMTs programme. Proportion female to years) included in the study and stratified according to the Hoehn and male was 2.36 in the first-line and 1.9 in the second-line. The mean Yahr (HY) scale. Permission regarding the translation and validation of age was 36.4 years (±SD 10.6) in the first-line and 35.1 years (±SD the CDEPA questionnaire was obtained. The CDEPA questionnaire 9.7) in the second line. The mean time of observation in the first line defined APD as the presence of severe disability requiring help for was 3.7 years (±SD 2.4) and in the second line was 2.8 years (±SD activities of daily living, motor fluctuations with limitation or inability 1.3). The mean time from the first symptoms to MS diagnosis was to perform ADL, severe dysphagia, recurrent falls, or dementia. 24.54 months (median 7.23), and from MS diagnosis to treatment: 39.35 months (median 10.12). There was a positive correlation Results between time to start treatment and higher the initial EDSS score PD was categorized as advanced in 45 (37.5%) patients when using (R 0.29, p b 0.001). The choice of the first-line medication depends the gold standard and in 75 (62.5%) patients when the CDEPA on the region of the country. questionnaire was used. The CDEPA questionnaire and the gold standard agreed moderately (P b 0.001). The CDEPA classified APD Conclusions with a sensitivity of 98%; specificity of 58%; total accuracy of 74.6%; and In Poland, only about one third of all MS patients is treated with area under the curve of 78.5%. The internal consistency determined by DMTs. There is a need to improve the quality of MS patients Cronbach's alpha indicated an extremely good correlation (0.978). healthcare and their access to DMTs. Significant differences were found between the groups created by the CDEPA in several usual PD evaluations (HY Scale, SCOPA Motor Scale, Keywords: Multiple sclerosis, Disease modifying treatment, Non-motor Symptoms Scale for PD, Clinical Global Impression–Severity Epidemiology Scale, and Patient Global Impression–Severity Scale).

Discussion doi:10.1016/j.jns.2019.10.1143 These ﬁndings suggest, Turkish version of the CDEPA questionnaire is a valid, reliable, and useful instrument for easily screening APD. doi:10.1016/j.jns.2019.10.1142 WCN19-0214

Poster shift 03 - Autonomic nervous system disorders/history of WCN19-0206 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- Gastrointestinal disturbances in various forms of parkinsonism orders and nutritional disorders or intoxications O. Naimova, R. Matmurodovb, E. Abdukodirova a Clinical and epidemiological characteristics of multiple sclerosis Tashkent, Neurology, Tashkent, Uzbekistan b patients receiving disease modifying treatment in Poland Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 189

One of the non-motor autonomic disorders of parkinsonism is Material and methods gastrointestinal disorders, which depend on the form and stage of 46 patients with PD at the age of 33–65 years (mean age 46.7 ± the disease. 8.1 years) were examined, of whom 23 were men and 23 women. All patients underwent clinical, neurological and neuropsychological Aim studies before and after physical with music therapy. The quality of To examine the types of gastrointestinal disorders in parkinson- life of patients is assessed with the EuroQol-5D scale. ism, depending on the form of the disease. Results Material and methods Аll patients underwent special physical exercises with music 103 patients with PD were examined, of them primary parkin- therapy. The analysis revealed a statistically signiﬁcant difference sonism was 51, secondary parkinsonism was 44, tertiary parkinson- before and after physical therapy activities. The scale of assessment ism was 8. The average age of patients was from 33 to 65 years of the quality of life shows that, prior to the events, the moveability (mean age 46.7 ± 8.1 years). The diagnosis was made according to is 87.5%, pain/discomfort - 84.3%, anxiety/depression - 90.6%, which special criteria that can be differentiated. All patients had various all had pronounced and very important problems. And after gastrointestinal disorders, according to the type of discomfort after conducting special exercises without music therapy, the scale eating, hypersolivation and defecation. Evaluated on the scale showed 60.1% movable, 65.7% pain/discomfort and 64.3% anxiety/ UPDRS. depression, (p b 0.001). In these patients, unexpressed and unim- portant problems. We evaluated the quality of life of patients with Results physical therapy exercises with music therapy and the scale showed After our careful analysis, the results show that with Parkinson's 56.2% of the movement, pain/discomfort 59.3% and anxiety/depres- disease, discomfort after eating is 2.1 ± 1.4 points, hypersolivation sion 62.5%, (p b 0.001). These patients have not encountered any 0.85 ± 2.2 points and defecation 1.5 ± 1.4 points. In secondary problems. An interesting fact is that the patients after music therapy parkinsonism, discomfort after eating is 1.5 ± 1.7, hypersolivation - did not feel anxiety and depression, as well as discomfort. 0.9 ± 2.1, and defecation 1.8 ± 1.6 points. When at tertiary parkinsonism, this data is 1.8 ± 1.8–0.76 ± 1.7 - 1.2 ± 2.5 points. The total Conclusion points for PD are 5.4, for secondary parkinsonism 2.51 and for Conducting special physical therapy exercises with music therapy tertiary parkinsonism 4.5 points. After eating and parkinsonism more allows patients not only to improve the quality of life, but also to bowel movements. inﬂuence the working ability and disability of patients.

Conclusion doi:10.1016/j.jns.2019.10.1145 Gastrointestinal disturbances of the type of discomfort after eating, hypersolivation and defecation depend on the form of parkinsonism. With the help of these disorders it is possible to differentiate the forms and course of the disease.Gastrointestinal WCN19-0265 disturbances may be a predictor of Parkinson's disease, which can be observed in preclinical manifestations. Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1144 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-0217 Protective and therapeutic activity of cinnamaldehyde in the mouse model of Parkinson’s disease Poster shift 03 - Autonomic nervous system disorders/history of S. Rajput, S. Sinha neurology/motor neuron disease/movement disorders/MS & Guru Gobind Singh Indraprastha University, Biotechnology, New Delhi, demyelinating diseases/neuromuscular disorders/vestibular dis- India orders and nutritional disorders or intoxications Objectives New approaches to the rehabilitation of patients with Parkinson's Objective of our study was to evaluate protective and therapeutic disease activity of Cinnamaldehyde in the mouse model of Parkinson’s disease (PD). R. Juraeva, R. Matmurodova, A. Yokubovb a Tashkent, Neurology, Tashkent, Uzbekistan Methods b Tashkent Medical Academy, Rehabilitology, Tashkent, Uzbekistan In present study 6-hydroxydopamine (6-OHDA) model of PD mice were used. The symptoms of PD such as tremors, akinesia, Parkinson's disease (PD) is very relevant in all countries of the rigidity, catalepsy, and vacuous chewing movements (VCMs) were world. evaluated. The methanolic extract of CA was administered orally at doses of 100 mg, 200 mg and 500 mg/kg body weight followed by Objective stress. The combination of L-dopa and carbidopa was used as a To study the quality of life of patients with the help of physical standard drug. Behavioral studies such as locomotor activity and grip therapy and music therapy. strength were determined, and memory test (Morris water maze) ARTICLE IN PRESS

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

was evaluated in mice. Antioxidants enzymes, neurolipofusicin, lipid correlated with standing systolic and diastolic BP at month 24 (rp = peroxides and inflammatory cytokines (TNF-a, IL-1β, and IL-6) were 0.448, p b 0.001 and rp = 0.342, p = 0.005, respectively). Univariable measured in hippocampus using corresponding commercial kits linear regression analysis revealed that α-BRSa at baseline predicts were analyzed in rat model of PD. the value of standing systolic and diastolic BP at month 24 (B = 2.724, 95% CI 1.357–4.091, p b 0.001 and B = 1.489, 95% CI 0.459– Results 2.519, p = 0.005). Furthermore, baseline α-BRSa predicted those Animal exposed to stress showed significant decrease in antiox- patients with supine systolic blood pressure N120 mmHg at month idant enzyme, glutathione and total protein. This was accompanied 24 (Exp(B) = 1.40, 95% CI 1.046–1.875, p = 0.024), indicating by simultaneous increase in lipid peroxides and neurolipofusicin elevated blood pressure according to American Heart Association. level. Treatment with CA had no significant but moderate effect on antioxidant enzyme. Pretreatment with CA (200 and 500 mg/kg) Conclusion significantly reduced the intensity of muscular rigidity, duration of This study provides further evidence for possible role of α-BRSa catalepsy, akinesia, the number of tremors, and significantly as a marker of adrenergic hyperactivity in pwMS which could increased locomotor activity and grip strength. Treatment with CA explain increased risk for cardiovascular diseases. significantly reduced inflammatory cytokines, ROS level, lipid peroxides level and restored the defensive antioxidant enzymes in doi:10.1016/j.jns.2019.10.1147 mice brain.

Conclusions CA may extract appreciably improve the neuroinﬂammatory WCN19-0291 processes and also restore biochemical and behavioral parameters with a potential therapeutic candidate for anti-aging studies. Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1146 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-0289 Facial expression processing is not affected by Parkinson’s disease itself, but by age related factors Poster shift 03 - Autonomic nervous system disorders/history of D.Y. Kwona, M.H. Parka, M.H. Songb neurology/motor neuron disease/movement disorders/MS & aKorea University, Ansan Hospital, Neurology, Ansan, Republic of Korea demyelinating diseases/neuromuscular disorders/vestibular dis- bBHS Hanseo Hospital, Neurology, Busan, Republic of Korea orders and nutritional disorders or intoxications Background Adrenergic hyperactivity predicts people with multiple sclerosis Although the degree of facial movement decrease in patients with who will develop elevated blood pressure Parkinson’s disease (PD), the question whether facial expression processing may be impaired in PD so far has yielded equivocal a b b b c M. Habek , D. Pucic , T. Mutak , L. Crnosija , M. Lovric , M. Krbot results. Recent studies have focused on testing expression processing b Skoric in recognition tasks with static images, not with dynamic conditions. aUniversity of Zagreb, School of Medicine, Neurology, Zagreb, Croatia b University Hospital Center Zagreb, Neurology, Zagreb, Croatia Methods c University Hospital Center Zagreb, Laboratory Diagnostics, Zagreb, Both conversational, emotional and dynamic facial expressions in Croatia three groups were tested. PD patients (n = 20), age- and education- matched healthy controls (n = 20), and young adult healthy controls Objectives (n = 20) were recruited. This setup allows us to address both effects To evaluate the evolution of adrenergic baroreﬂex sensitivity of PD and age-related differences. We employed a multi-dimensional (BRSa) over two-year follow-up and to investigate the effect of rating task for all groups in which 12 rating dimensions were used to baseline BRSa indices on blood pressure (BP) values after two years assess evaluative processing of 27 expression videos from 6 different in pwRRMS. actors.

Methods Results After two-year follow-up, data on BRSa indices were available for We found that ratings overall were consistent across groups with 67 pwRRMS both on baseline and at follow-up. Following data were several rating dimensions having a strong correlation with the analysed at baseline and after two years: BRSa measured with BRSa1, expressions’ motion energy content as measured by optic flow α-BRSa and β-BRSa, supine and standing levels of epinephrine and analysis. Most importantly, we found that the PD group did not differ norepinephrine, supine and standing systolic and diastolic BP levels. in any rating dimension from the older healthy control group, indicating highly similar evaluation processing. Both older groups, Results however, showed significant differences for several rating scales in Compared to baseline values, BRSa1 was significantly lower comparison with the younger control group. (22.57 vs. 28.73, p = 0.020), β-BRSa significantly higher (1.52 vs. 1.10, p = 0.001), and no change was observed in α-BRSa (6.96 ± Conclusions 2.56 vs. 6.64 ± 2.24, p = 0.379) at moth 24. α-BRSa at month 24 Older participants rated negative expressions compared to the positively correlated with standing levels of norepinephrine at younger participants as more positive, but also as less natural, month 24 (rp = 0.357, p = 0.005). α-BRSa at baseline positively persuasive, empathic, and sincere. We interpret these in the context ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 191 of positivity effect and in-group processing advantages. Overall, our Subjects findings do not support processing deficits due to PD, but rather Thirty PD patients (Table) were rated with the Movement point to age-related differences in facial expression processing. Disorder Society- Unified Parkinson's Disease Rating Scale part I hallucination/psychosis item scores, and were scanned with Techne- tium-99m- labeled ethyl cysteinate dimer single photon emission doi:10.1016/j.jns.2019.10.1148 computed tomography both before and after a single administration of antiparkinsonian drugs. Imaging data were processed using Statistical Parametric Mapping 2, easy Z-score Imaging System, and voxel-based Stereotactic Extraction Estimation. The study was WCN19-0295 approved by the ethical review board of the university and conformed with the Declaration of Helsinki.

Poster shift 03 - Autonomic nervous system disorders/history of Results neurology/motor neuron disease/movement disorders/MS & rCBF increases in the parietal association cortex (r = 0.46), demyelinating diseases/neuromuscular disorders/vestibular dis- primary visual cortex (r = 0.40), and caudate nucleus (r = 0.45) orders and nutritional disorders or intoxications correlated with hallucination/psychosis item scores (Spearman's rank correlation coefficient; p b 0.05, respectively), but there was no significant correlation in the LG. Spect findings in Parkinson’s disease – 2nd report: Relevance to hallucinations induced by antiparkinsonian drugs Conclusion Although some regions showed CBF responses correlating with S. Taguchi, N. Tanabe, J.I. Niwa, M. Doyu hallucination scores, the influence of hallucination on the rCBF Aichi Medical University, Neurology, Nagakute, Japan response in the LG can be assumed to be minor.

Background doi:10.1016/j.jns.2019.10.1149 We previously showed motor improvement-related regional cerebral blood ﬂow (CBF) changes in Parkinson's disease (PD) patients in response to the administration of antiparkinsonian drugs. These results suggested that hallucination might have affected regional CBF (rCBF) changes in the lateral geniculate body (LG), WCN19-0300 and indicated the need for further studies focusing on the relationship between antiparkinsonian drug-induced hallucination Poster shift 03 - Autonomic nervous system disorders/history of and drug-driven rCBF changes. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Objective orders and nutritional disorders or intoxications We examined whether rCBF responses in the LG are affected by hallucination. Furthermore, to help elucidate the mechanism Effect of opicapone in Parkinson’s disease patients with ‘early’ of antiparkinsonian drug-induced hallucination, the regions motor ﬂuctuations: Parkinson’s disease questionnaire (PDQ-39) where a CBF response was affected by hallucination were also analysis from the BIPARK-I double-blind experience determined. ARTICLE IN PRESS

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

F. Rochaa, E. Tolosab, J. Ferreirac, O. Rascold, W. Poewee, A. Santosf,D. References Magalhaesa, P. Soares-da-Silvaf a BIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. Portugal [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. bHospital Clinic Universitari de Barcelona, Neurology Department, Barcelona, Spain cFaculty of Medicine, University of Lisbon, Neurology Department, Lisbon, Portugal doi:10.1016/j.jns.2019.10.1150 dUniversity Hospital of Toulouse, Neurology Department, Toulouse, France eMedical University of Innsbruck, Neurology Department, Innsbruck, Austria WCN19-0301 fBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Objective To evaluate the impact of opicapone (OPC) on PDQ-39 in demyelinating diseases/neuromuscular disorders/vestibular dis- levodopa-treated Parkinson’s Disease (PD) patients with ‘early’ orders and nutritional disorders or intoxications motor fluctuations. OPC, a once-daily COMT inhibitor, proved effective in the treatment of motor fluctuations in PD patients in Efficacy of opicapone in Parkinson’s disease patients with ‘early’ two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. motor fluctuations: Patient and clinical global impression of change from the BIPARK-I double-blind experience Methods Double-blind, 14 to 15-week, placebo- and active-controlled F. Rochaa, F. Stocchib, A. Leesc, J. Ferreirad, W. Poewee, O. Rascolf,A. study [1]. Dopamine-agonists and MAO-B inhibitors used for PD Santosg, D. Magalhaesa, P. Soares-da-Silvag treatment were allowed provided their stable regimen for 4-weeks aBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, before and throughout the study. ‘Early-fluctuators’ (EF) were Portugal defined as subjects with onset of motor fluctuations within b2 years bIRCCS San Raffaele, Neurology Department, Rome, Portugal of study baseline. This post-hoc analysis investigated changes in cNational Hospital for Neurology and Neurosurgery, Neurology Depart- PDQ-39 at the end of double-blind period in OPC-50 mg, entacapone ment, London, United Kingdom (ENT) and placebo (PLC) groups in EF levodopa-treated patients. dFaculty of Medicine, University of Lisbon, Neurology Department, Data was analyzed via an ANCOVA model with treatment group and Lisbon, Portugal region as fixed effects. eMedical University of Innsbruck, Neurology Department, Innsbruck, Austria Results fUniversity Hospital of Toulouse, Neurology Department, Toulouse, In total, 359 patients were randomized; 206 patients were EF France (PLC, n = 66; ENT, n = 70; OPC-50 mg, n = 70). Overall, LS mean gBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, changes from baseline were −2.6 for PLC, −4.0 for ENT and −2.8 for Portugal OPC-50 mg. Regarding EF, LS mean changes [95%CI] of PDQ-39 from − − − − − double-blind baseline were 2.0 [ 4.4,0.5], 3.3 [ 5.7, 0.8] and Objective − − − 3.8 [ 6.3, 1.3] for PLC, ENT (p = 0.0085) and OPC-50 mg (p = To evaluate, in levodopa-treated Parkinson’s Disease (PD) 0.0026), indicating improvements in health status and quality of life. patients with ‘early’ motor fluctuations, the patients’ and clinicians’ perception of PD symptoms improvement [as assessed by Patient Conclusion and Clinical Global Impression of Change (PGI-C and CGI-C)]. ‘ Similar to total study population, adding OPC-50 mg to early- Opicapone (OPC), a once-daily COMT inhibitor, proved effective in fl ’ uctuators improved health status and quality of life. In contrast to the treatment of motor fluctuations in PD patients in two large, ‘ PLC and ENT, PQD-39-decreasing was higher in OPC-50 mg early pivotal, multinational trials (BIPARK-I and II) [1,2]. fluctuators’ than in the overall population. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 193

Methods effective in the treatment of motor fluctuations in PD patients in two Double-blind, 14 to 15-week, placebo- and active-controlled large, pivotal, multinational trials (BIPARK-I and II) [1,2]. study [1]. Dopamine-agonists and MAO-B inhibitors used for PD treatment were allowed provided their stable regimen for at least 4- Methods weeks before and throughout the study. ‘Early-fluctuators’ (EF) were Patient-level data from matching treatment arms in BIPARK-I and defined as subjects with an onset of motor fluctuation within b2 II were combined in placebo (PLC) and OPC-50 mg groups. Studies years of study baseline. This post-hoc analysis investigated both PGI- had similar designs (primary efficacy endpoint: change from baseline C and CGI-C in levodopa-treated EF. in patient diaries-based absolute OFF-time) and eligibility criteria [1,2]. Key secondary endpoints included analysis of responders (N1h Results OFF-time reduction or N1 h ON-time increase). A post-hoc analysis In total, 359 patients were randomized (placebo (PLC), n = 121; was performed to evaluate OPC-50 mg and PLC efficacies in entacapone (ENT), n = 122; OPC-50 mg, n = 116); 206 patients levodopa-treated PD patients according to BPDys (assessed by were EF (PLC, n = 66; ENT, n = 70; OPC-50 mg, n = 70). Table 1 UPDRS question 32). describes CGI-C and PGI-C improvement (comprising minimally, much, and very much improved) at endpoint in overall population Results and EF. In contrast to OPC, no differences were noted for ENT In total, 535 patients were randomized; Full Analysis Set compared with PLC in both total and EF population. comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. 47% (PLC) to 50% (OPC-50 mg) of patients reported BPDys. Mean placebo- Conclusion adjusted OFF-time reduction was −50.5 min (p = 0.0080) and Similar to total study population, the ‘early-fluctuators’ patients’ −67.3 min (p = 0.0005) for OPC-50 mg patients with and without and clinicians’ perception of improvement was significant for BPDys. There was ~65% more OFF-time reduction in PLC patients opicapone in comparison to placebo or entacapone in this post-hoc with BPDys than in those without. A higher-than-PLC proportion of analysis. OPC-50 mg-receiving patients with BPDys achieved OFF- (67.9% vs. 50.8%; p b 0.0060) and ON-time (65.6% vs. 45.1%; p = 0.0011) References responders’ endpoint. Consistently, a higher-than-PLC proportion of OPC-50 mg-receiving patients with absence of dyskinesia achieved [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. OFF- (67.2% vs. 47.4%; p = 0.0010) and ON-time (61.1% vs. 45.9%; p ’ [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. = 0.0115) responders endpoint.

Conclusion Opicapone was effective regardless of baseline presence of doi:10.1016/j.jns.2019.10.1151 dyskinesia.

References

WCN19-0302 [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- doi:10.1016/j.jns.2019.10.1152 orders and nutritional disorders or intoxications

Efficacy of opicapone in Parkinson’s disease patients according to baseline presence of dyskinesia: A post-hoc analysis from WCN19-0303 combined BIPARK-I and II Poster shift 03 - Autonomic nervous system disorders/history of A. Leesa, J. Ferreirab, A. Antoninic, H. Reichmannd, R. Costae, D. neurology/motor neuron disease/movement disorders/MS & Magalhaesf, F. Rochaf, P. Soares-da-Silvae demyelinating diseases/neuromuscular disorders/vestibular dis- aNational Hospital for Neurology and Neurosurgery, Neurology Depart- ment, London, United Kingdom orders and nutritional disorders or intoxications bFaculty of Medicine, University of Lisbon, Neurology Department, Lisbon, Portugal Efficacy of opicapone in Parkinson’s disease patients according to cNeurology Department, University of Padova, Padova, Italy baseline pramipexole use: A post-hoc analysis from combined dUniversity of Dresden, Neurology Department, Dresden, Germany BIPARK-I and II eBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal A. Leesa, J. Ferreirab, O. Rascolc, W. Poewed, F. Stocchie, A. Santosf, D. fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Magalhaesg, F. Rochag, P. Soares-da-Silvaf Portugal aNational Hospital for Neurology and Neurosurgery, Neurology Depart- ment, London, United Kingdom b Objective Faculty of Medicine, University of Lisbon, Neurology Department, To evaluate the efficacy of opicapone (OPC) in levodopa-treated Lisbon, Portugal c Parkinson’s Disease (PD) patients with or without baseline presence University Hospital of Toulouse, Neurology Department, Toulouse, of dyskinesia (BPDys). OPC, a once-daily COMT inhibitor, proved France ARTICLE IN PRESS

194 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx dMedical University of Innsbruck, Neurology Department, Innsbruck, A. Leesa, J. Ferreirab, O. Rascolc, W. Poewed, E. Tolosae, H. Gamaf, D. Austria Magalhaesg, F. Rochag, P. Soares-da-Silvaf eIRCCS San Raffaele, Neurology Department, Rome, Italy aNational Hospital for Neurology and Neurosurgery, Neurology Depart- fBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, ment, London, United Kingdom Portugal bFaculty of Medicine, University of Lisbon, Neurology Department, gBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Lisbon, Portugal Portugal cUniversity Hospital of Toulouse, Neurology Department, Toulouse, France d Objective Medical University of Innsbruck, Neurology Department, Innsbruck, To evaluate opicapone’s (OPC) efficacy in levodopa-treated Austria e Parkinson’s Disease (PD) patients with or without concurrent Hospital Clinic Universitari de Barcelona, Neurology Department, pramipexole use (CPU). OPC, a once-daily COMT inhibitor, proved Barcelona, Spain f effective in the treatment of motor fluctuations in PD patients in two BIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, large, pivotal, multinational trials (BIPARK-I and II) [1,2]. Portugal gBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Methods Portugal Patient-level data from matching treatment arms in BIPARK-I and II were combined in placebo (PLC) and OPC-50 mg groups. Studies had Objective similar designs (primary efficacy endpoint: change from baseline in To evaluate opicapone’s(OPC)efficacy in levodopa- patient diaries-based absolute OFF-time) and eligibility criteria [1,2]. treated Parkinson’s Disease (PD) patients with or without This exploratory post-hoc analysis was performed to evaluate OPC-50 concurrent rasagiline use (CRU). OPC, a once-daily COMT inhib- mg and PLC efficacies in levodopa-treated PD patients with or without itor, proved effective in the treatment of motor fluctuations in PD baseline CPU (the most commonly used dopamine agonist). patients in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. Results In total, 535 patients were randomized; Full Analysis Set Methods comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. About 37% Patient-level data from matching treatment arms in BIPARK-I and of patients in each arm were using pramipexole at baseline. Mean II were combined in placebo (PLC) and OPC-50 mg groups. Studies placebo-adjusted OFF-time reductions were −89.0 min (p = 0.0001) had similar designs (primary efficacy endpoint: change from baseline and −39.8 min (p = 0.0165) for OPC-50 mg patients with and in patient diaries-based absolute OFF-time) and eligibility criteria without CPU. There was ~45% more OFF-time reduction in PLC [1,2]. This exploratory post-hoc analysis was performed to evaluate patients without CPU than in those with, but ~20% less OFF-time OPC-50 mg and PLC efficacies in levodopa-treated PD patients with reduction in OPC-50 mg patients without CPU than in those with. A or without baseline CRU (the most commonly used MAO-B higher-than-PLC proportion of OPC-50 mg-receiving patients with inhibitor). CPU achieved OFF- (72.9% vs. 40.4%; p b 0.0001) and ON-time (68.8% vs. 42.6%; p = 0.0003) responders’ endpoint. Consistently, a higher- Results than-PLC proportion of OPC-50 mg-receiving patients without CPU In total, 535 patients were randomized; Full Analysis Set achieved OFF- (64.5% vs. 54.0%; p = 0.0551) and ON-time (60.2% vs. comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. 12 (PLC) 47.2%; p = 0.0169) responders’ endpoint. to 15% (OPC-50 mg) of patients were using rasagiline at baseline. Mean placebo-adjusted OFF-time reductions were Conclusion −81.1 min (p = 0.0469) and −54.2 min (p = 0.0002) for OPC- Opicapone was effective regardless of concurrent pramipexole 50 mg patients with and without CRU. There was ~35% more OFF- use. time reduction in PLC patients without CRU than in those with, but ~9% less OFF-time reduction in OPC-50 mg patients without References CRU than in those with. A higher-than-PLC proportion of OPC-50 mg-receiving patients with CRU achieved OFF- (40.0% vs. 66.7%; p ’ [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. = 0.0283) and ON-time (40.0% vs. 64.1%; p = 0.0491) responders [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. endpoint. Consistently, a higher-than-PLC proportion of OPC-50 mg-receiving patients without CRU achieved OFF- (50.2% vs. doi:10.1016/j.jns.2019.10.1153 67.7%; p = 0.0002) and ON-time (46.2% vs. 63.2%; p = 0.0003) responders’ endpoint.

Conclusion Opicapone was effective regardless of concurrent rasagiline use. WCN19-0304 References Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. demyelinating diseases/neuromuscular disorders/vestibular dis- [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. orders and nutritional disorders or intoxications

ﬁ ’ Ef cacy of opicapone in Parkinson s disease patients according to doi:10.1016/j.jns.2019.10.1154 baseline rasagiline use: A post-hoc analysis from combined BIPARK-I and II ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 195

WCN19-0305 WCN19-0306

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

Influence of demographic characteristics in the efficacy response Levodopa-carbidopa intestinal gel treatment of motor fluctua- of Parkinson’s disease patients with motor fluctuations: Post-hoc tions and dyskinesia in advanced Parkinson’s disease patients in a analysis from combined BIPARK-I and II ‘real world’ setting: Interim results from the DUOGLOBE study

A. Santosa, J. Ferreirab, A. Leesc, H. Reichmannd, W. Poewee, F. Ikedoa, D.G. Standaerta, J. Aldredb, M. Anca-Herschkovitschc, T.L. Davisd,R. D. Magalhaesf, F. Rochaf, P. Soares-da-Silvaa, N. Savica Ianseke, N. Kovacsf, M. Siddiquig, F.E. Pontierih, M. Simui, L. aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Bergmannj, P. Kukrejaj,M.Lij, K.R. Chaudhurik Portugal aUniversity of Alabama at Birmingham, Neurology, Birmingham, USA bFaculty of Medicine, University of Lisbon, Neurology Department, bSelkirk Neurology, Neurology, Spokane, USA Lisbon, Portugal cEdith Wolfson Medical Center, Movement Disorder Clinic, Holon, Israel; cNational Hospital for Neurology and Neurosurgery, Neurology Depart- dVanderbilt University Medical Center, Nashville, USA; ment, London, United Kingdom eMonash Health, Clinical Research Centre for Movement, Melbourne, dUniversity of Dresden, Neurology Department, Dresden, Germany Australia; eMedical University of Innsbruck, Neurology Department, Innsbruck, Austria fUniversity of Pécs, Neurology, Pécs, Hungary; fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Portugal gWake Forest School of Medicine, Neurology, Winston Salem, USA; hSapienza University of Rome, Neurology, Rome, Italy; i Objective Victor Babes University of Medicine and Pharmacy, Neurology, To evaluate the impact of demographic characteristics in the Timisoara, Romania; j efficacy response of levodopa-treated Parkinson’s Disease (PD) AbbVie- Inc, Neuroscience, North Chicago, USA k patients with motor fluctuations. Opicapone (OPC), a once-daily King’s College and King’s College Hospital, Neurology/Movement COMT inhibitor, proved effective in the treatment of motor Disorders, London, United Kingdom fluctuations in PD patients in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. Objective Assess the long-term effectiveness of levodopa-carbidopa intes- Methods tinal gel (LCIG) on motor fluctuations and dyskinesias in advanced Patient-level data from matching treatment arms in BIPARK-I and Parkinson’s disease (PD) patients treated in routine clinical practice. II were combined in placebo (PLC) and OPC-50 mg groups. The studies had similar designs (primary efficacy endpoint: change from Methods baseline in patient diaries-based absolute OFF-time) and eligibility DUOGLOBE is the first multinational observational routine care criteria [1,2]. An exploratory post-hoc analysis was performed to study of LCIG with 3 years follow-up. Primary outcome is mean evaluate the influence of demographic characteristics [i.e., age, change in patient-reported “Off” time; secondary outcomes include gender, race (Caucasian vs. Asian), weight and Body Mass Index motor complications (dyskinesia duration/severity), as measured by (BMI)] in efficacy outcomes (i.e., change from baseline in OFF-/ON- the recently developed Unified Dyskinesia Rating Scale (UDysRS). time). Linear regression was applied and the slope was statistically Outcomes were collected at baseline, day (D) 1 and scheduled visits tested for deviation from zero. closest to months (M) 3, 6, and 12 (±14 days) in this interim analysis. Results In total, 1027 patients were randomized to BIPARK-I and II and 522 patients took a dose of study medication; Full Analysis Set comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. Neither OFF- nor ON- time magnitude of responses were influenced by age, gender, race, weight and BMI at baseline. Linear regression applied was found to be non-significant for both treatment arms and all variables analyzed.

Conclusion Demographic characteristics (i.e., age, gender, race, weight and BMI) do not appear to impact either OFF- or ON-time magnitude of responses in a clinical study setting.

References

[1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. doi:10.1016/j.jns.2019.10.1155 ARTICLE IN PRESS

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

Results reduced patient-reported “Off” time (mean decrease from baseline to 139 patients were included in the analysis (78% ≥ 65 years M12: −4.1 h) irrespective of sex, age, and disease duration. old; 51% ≥ 10 years’ disease duration. LCIG treatment significantly Dyskinesia assessed by UDysRS significantly improved at D1 and was maintained through M12 (mean decrease from baseline: −12.7). Improvements according to UPDRS part IV scores for duration of “Off” time (through M12), dyskinesia duration (through M6), dyskinesia-related disability (through M12), and dyskinesia- related pain (through M12) were also observed. Safety was consistent with LCIG’s known profile.

Conclusion This interim analysis confirms the marked improvement in “Off” time with LCIG in routine clinical practice. This first analysis using UDysRS, and supporting data from UPDRS part IV, showed that LCIG treatment was also associated with a marked reduction in dyskinesia duration and severity. These findings provide further evidence for the real-world effectiveness of LCIG on motor fluctuations and duration and severity of dyskinesia.

doi:10.1016/j.jns.2019.10.1156 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 197

WCN19-0309 D.Y. Kwona, M.H. Parka, M.H. Songb aKorea University Ansan Hospital, Neurology, Ansan, Republic of Korea bBHS Hanseo Hospital, Neurology, Busan, Republic of Korea Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Background demyelinating diseases/neuromuscular disorders/vestibular dis- Although epidemiologic studies have consistently reported an orders and nutritional disorders or intoxications increase in mortality associated with Parkinson's disease (PD), the relationship between cardiovascular disease (CVD)—the leading Harris-Benedict equation well predicts resting energy expendi- cause of death—and PD is unclear. In this nationwide population- ture in Parkinson's disease patients under dopaminergic treat- based, matched cohort study, we assessed the risk of myocardial ment: A control-case study infarction (MI), stroke, and all-cause mortality in patients with PD in Korea. M. Barichellaa, G. Piuria, S.A. Faiermana, E. Cassania, E. Ceredab,E. Vaccarellaa, O.V. Donnarummaa, V. Ferria, G. Pinellia, C. Bolliria, S. Methods Caronnia, C. Pusania, G. Pezzolia A population-based, age- and sex-matched cohort study was aParkinson Institute, ASST G.Pini-CTO, Milan, Italy conducted using the database from the Rare Intractable Disease bClinical Nutrition and Dietetics Unit, Fondazione IRCCS Policlinico San registry and the Health Insurance Review and Assessment Service, Matteo, Pavia, Italy which covers the entire Korean population, from 2010 to 2015. We analyzed 25,624 PD and 128,120 non-PD cases (≥40 years; mean age, Introduction 69.0 ± 10.2 years; 42.5% men) without previous diagnosis of MI or The body weight in Parkinson's disease (PD) patients changes stroke. Survival analysis, log-rank test, and Cox proportional hazards according to the progression of disease and the scientific evidence regression models were used to calculate the incidence, survival rate, regarding the effects of PD on energy expenditure (REE) is still and hazard ratio of CVD and mortality. incomplete and inconsistent: some studies showed an increase in REE in PD patients compared to healthy subjects, unlike other studies Results fi did not find this difference. The aim of this study is to investigate REE PD was signi cantly associated with the prospective development fi – by indirect calorimetry in a larger group of patients suffering from of MI (hazard ratio [HR]: 1.43, 95% con dence interval [CI]: 1.28 – PD (matched with healthy controls) and to assess if the Harris- 1.59), stroke (HR: 1.42, 95% CI: 1.31 1.54), and all-cause death (HR: – Benedict equation can predict REE in PD patients. 2.7, 95% CI: 2.60 2.81) after adjustment for multiple covariates during the 498,811.6 person-years of follow-up. The PD group had fi Methods signi cantly lower 6-year MI- and stroke-free rates and survival b All subjects underwent REE measurement through indirect calorime- rates than the non-PD group (log-rank p .0001). try (FitMate, Cosmed, Rome, Italy), in the morning at about 8 a.m. after 12 h fasting. We used Harris-Benedict's equation for the estimation of REE. Conclusions There is an increased risk of MI, stroke, and all-cause death in fi Results patients with PD. This nding has important implications for We enrolled 244 subjects (112 males and 132 females) of whom clinicians and supports the need for preventative measures for CVD 122 subjects suffering from PD and 122 healthy control subjects. REE in patients with PD. measured by indirect calorimetry was similar both for PD and for doi:10.1016/j.jns.2019.10.1158 healthy controls, 1364 kcal (95% IC 1295–1414) and 1313 kcal (95% IC 1257–1361), respectively. There were no statistical differences between measured and estimated REE.

Conclusions WCN19-0314 Body weight loss in advanced stages of PD seems to be due to increased muscular energy expenditure (induced by tremor and/or Poster shift 03 - Autonomic nervous system disorders / history of rigidity and/or dyskinesias), but not to increased REE. Harris-Benedict neurology /motor neuron disease / movement disorders / MS & equation seems to be adequate to predict REE in PD patients; indirect demyelinating diseases / neuromuscular disorders / vestibular calorimetry might be necessary only in selected cases. disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1157 Visual pathway demyelination in neurofascin-155 IGG4- positive combined central and peripheral demyelination

WCN19-0310 A. Verghesea, F.L. Hiewa, Y.K. Chiab, L. Querolc aHospital Kuala Lumpur, Department of Neurology, Kuala Lumpur, Malaysia Poster shift 03 - Autonomic nervous system disorders / history of bHospital Queen Elizabeth, Department of Neurology, Kota Kinabalu, neurology /motor neuron disease / movement disorders / MS & Malaysia demyelinating diseases / neuromuscular disorders / vestibular cAutoimmune Neurology, Neuromuscular Diseases Unit, Hospital de la disorders and nutritional disorders or intoxications Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Neuromus- cular Diseases Unit, Barcelona, Spain Association between PARKINSON's disease and the risk of cardiovascular disease and all-cause mortality: A nationwide, Combined central and peripheral demyelination (CCPD) is an register-based cohort study entity that has clinical and laboratory characteristics showing ARTICLE IN PRESS

198 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx overlap between multiple sclerosis (MS) and chronic inflammatory bulbar syndrome, the patient underwent electrophysiological and demyelinating polyneuropathy (CIDP) [1]. Before the description of laboratory test and was diagnosed with anti-MuSC MG. Later in the CCPD, optic nerve abnormalities were reported among CIDP patients. course of the disease he developed bilateral facial paralysis and However, the occurrence of optic nerve and visual pathway respiratory distress. He was twice under mechanical ventilation and demyelination in CCPD patients with anti Neurofascin-155 IgG4 under plasmapheresis treatment before being stabilized with a antibodies has not been properly characterized. Anti-NF155 anti- corticosteroid regimen. body-positivity has been reported in 18% of CIDP cases [2]. The second patient, a 84 year old man with a one year history of In this case series, we report two young men who had initially intermittent dysphagia and dysphonia. He was hospitalized because presented with a chronic progressive form of CIDP and were then of severe difficulty swallowing. The lab studies showed anti AChR Ab found to have subclinical visual pathway demyelination. The first level of 4.98 nmol/L. After the treatment with Pyridostigmine the patient, then 22 years old, presented with a progressive symmetrical symptoms resolved quickly and the patient has been in optimal upper and lower limb distal weakness one month following yellow conditions with a 180 mg/day dose of Pyridostigmine. fever vaccination while the second was a 19-year-old who presented with progressive numbness and weakness in all four limbs following Discussion an episode of febrile illness. The first patient had a right relative Approximately 15% of patients with MG present with bulbar afferent pupillary defect (RAPD) with normal visual acuity while the symptoms, while isolated bulbar presentation is less common, second had no visual abnormalities. Both had normal MRI scans of especially in the patients positive for anti AChR Ab. Anti MuSC MG the brain but prolonged P100 latencies on visual evoked potentials. patients don't respond to Pyridostigmine and are more prone to The detection of a high titer of IgG4 antibodies against neurofascin myasthenic crisis. MG should be included in the differential diagnosis 155 (NF 155) revealed an underlying combined central and of patients presenting with bulbar syndrome regardless having or peripheral paranodopathy syndrome in them. not other MG features.

References doi:10.1016/j.jns.2019.10.1160

[1] J.I. Kira, R. Yamasaki, H. Ogata, Anti-neurofascin autoantibody and demyelination, Neurochem. Int. (2018)(Dec 22. pii: S0197- 0186(18)30433-9). WCN19-0327 [2] H. Ogata, R. Yamasaki, A. Hiwatashi, et al., Characterization of IgG4 Anti-Neurofascin 155 Antibody-Positive Polyneuropathy, Poster shift 03 - Autonomic nervous system disorders / history of 2019. neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1159 Coagulation activation and cerebral hypoperfusion in relapsing- remitting multiple sclerosis

WCN19-0319 T. Koudriavtsevaa, S. Zanninoa, M.M. Filippib, A. Cortesec,C. Piantadosid, C. Lapuccie, M. Fiorellic, D. Giannarellif, C. Mandoja,A. a a g ehi Poster shift 03 - Autonomic nervous system disorders / history of Stefanile , L. Conti , M. Salvetti , M. Inglese aIRCCS Regina Elena National Cancer Institute, Department of Clinical neurology /motor neuron disease / movement disorders / MS & Experimental Oncology, Rome, Italy demyelinating diseases / neuromuscular disorders / vestibular bFatebenefratelli Hospital- Isola Tiberina, Fatebenefratelli Foundation- disorders and nutritional disorders or intoxications Afar Division, Rome, Italy cSapienza University of Rome, Department of Human Neurosciences, Myasthenia gravis with isolated bulbar syndrome on presenta- Rome, Italy tion, two case reports dSan Giovanni-Addolorata Hospital, Neurology Unit, Rome, Italy eUniversity of Genoa, Department of Neuroscience- Rehabilitation- G. Çakçiria, A. Kuqoa, J. Krujab Ophthalmology- Genetics- Maternal And Child Health DINOGMI, Genoa, aUHC Mother Teresa, Neurology, Tirana, Albania Italy bFaculty of Medicine, University of Medicine- Tirana, Neurology, Tirana, fIRCCS Regina Elena National Cancer Institute, Biostatistics- Scientiﬁc Albania Direction, Rome, Italy gSapienza University of Rome, Department Of Neuroscience Mental Background Health And Sensory Organs NEMOS, Rome, Italy h Myasthenia Gravis is an autoimmune disorder mediated from Mount Sinai Hospital, Department of Neurology, New York, USA i antibodies clinically presented with easy fatigability. The most Ospedale Policlinico San Martino IRCCS, Department of Neurology, common antibodies causing the clinical features are anti-AChR and Genoa, Italy anti-MuSC antibodies. Bulbar syndrome is common during the course of the disease but is rare at onset. Background Several recent studies showed coagulation involvement in Case report Multiple sclerosis (MS). The aim of our study is to evaluate the From our MG database we selected two cases presenting with bulbar serum/plasma levels of coagulation factors in relapsing MS patients symptoms. The ﬁrst patient is a 55 year old man with a 2 months history compared to remitting ones and to healthy controls, and to correlate of isolated dysphagia and dysarthria. After excluding other causes of their status with MRI perfusion data. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 199

Methods scale (GCS) score of 3/15 and bilaterally dilated unreactive pupils. A Thirty relapsing and 30 remitting MS patients, and 30 age/sex- brain CT-scan showed right frontal fracture, bifrontal hematoma matched healthy controls will be tested for complement/coagulation contusion, a fronto-temporo-parietal acute subdural hematoma assays. Patients will undergo dynamic-susceptibility contrast-en- (SDH) with a thickness of 14 mm on the right side, traumatic hanced 3.0-T MRI. subarachnoid hemorrhage, with 20 mm of midline shift to the left side, diffuse brain edema. The second one presented with severe TBI Preliminary results following an automobile accident with a GCS score of 4/15 and 48 MS patients [36 remitting (27F/9M, age 41,8 ± 8,9 years), 12 iso^reactive pupils. A brain CT-scan showed bilateral fronto-temporal relapsing (9F/3M, 41,3 ± 7,3)] and 35 controls (26F/9M, 41,5 ± 8,9) fracture, diffuse brain hematoma contusion, traumatic subarachnoid have entered the study. Relapsing and remitting MS patients hemorrhage, right extradural hematoma (EDH) and bilateral fronto- compared to controls had a higher protein-C (116,5 ± 22,4, 112,6 temporo-parietal acute subdural hematoma (SDH) more important ± 17,4, 98,7 ± 14.9% respectively, p = .001) and ﬁbrinogen (334,2 in the right side. ± 111,7, 318,8 ± 76,6, 283,6 ± 44,5 mg/dl, p = .04) with a trend for a lower hematocrit (41,4 ± 2,9, 40,8 ± 3,3, 42,7 ± 2,9%, p = .06) and Discussion and conclusion hemoglobin (13,8 ± 1.2, 13,5 ± 1,1, 14,1 ± 1.1 g/dl, p = .09). Re- Our case series suggest that the wide adequate decompressive lapsing patients had a lower lymphocyte count (1,4 ± 0,48, 1,9 ± craniectomy in patients with severe TBI and GCS score of 3 or 4 can 0,6, 1,9 ± 0,6 × 10^3/μL, p = .01). At MRI perfusion analysis, cerebral be performed and useful to obtain good long-term neurological blood volume (CBV), ﬂow (CBF) and Mean Transit Time (MTT) were outcomes with a good functional recovery. The rapidity of the in the Normal Appearing White Matter: 14,39 ± 5,13, 147,33 ± surgical indication decision can be option to obtain the better 25,61, 6,11 ± 2,11 and 12,29 ± 2,24, 139,58 ± 22,31, 5,28 ± 0,35 neurological outcomes. respectively in remitting and relapsing patients, and in the deep gray matter 20,11 ± 6,33, 223,31 ± 41,17, 5,71 ± 2,14 and 18,24 ± 3,02, Keywords: Traumatic brain injury, Decompressive craniectomy, 208,19 ± 40,73, 5,26 ± 0,42 (thalamus) and 18,0 ± 4,5, 189,18 ± Glasgow coma scale, Functional recovery 42,1, 5,75 ± 0,74 (caudate).

Conclusion Our preliminary data indicate coagulation activation and slight doi:10.1016/j.jns.2019.10.1162 anemia in relapsing-remitting MS patients compared to healthy controls with reduction of circulating lymphocytes during the relapse. These data suggest an inflammatory-thrombotic status with activation of immune cells requiring more energy to moderate their WCN19-0334 metabolic functions. Poster shift 03 - Autonomic nervous system disorders / history of doi:10.1016/j.jns.2019.10.1161 neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications WCN19-0333 The importance of somatotopy to achieve clinical benefitin motor cortex stimulation for pain relief Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & A. Afifa, L. Garcia-Larreab, P. Mertensa demyelinating diseases / neuromuscular disorders / vestibular aLyon 1 University, Department of Neurosurgery, Department of disorders and nutritional disorders or intoxications Anatomy, INSERM U 1028, Pain Center, Pierre WERTHEIMER Hospital, Lyon, France Decompressive craniectomy following severe traumatic brain bLyon 1 University, INSERM U 1028, Pain Center, Pierre WERTHEIMER injury with an initial Glasgow coma scale score of 3 and 4 Hospital, Lyon, France

A. Afif Introduction Lyon 1 University, Department of Neurosurgery, Department of The aim of this study was to search the relationship between the Anatomy, INSERM U 1028, Pain Center, Pierre WERTHEIMER Hospital, anatomical location and the eventual analgesic effect of each contact. Lyon, France Materials and methods 22 patients suffering from neuropathic pain were implanted with Abstract^ Background stimulation of the precentral cortex. Decompressive craniectomy formed as surgical management The implantation of the electrodes was performed using intraop- option for severe traumatic brain injury (TBI). Few studies that erative: 1) Anatomical identification by Neuronavigation with 3D follow the TBI patients with a Glasgow coma scale (GCS) score of 3 or MRI, 2) Somesthetic evoqued potentials monitoring to check the 4. Decompressive craniectomy was avoided in these patients due to potential reverse over the central sulcus, 3) Electrical stimulations the poor outcomes and the worse functional recovery. through the dura to identify the motor responses and its somatotopy. In order to locate postoperatively the electrodes, a 3D-CT was Clinical presentation performed and fused with the preoperative MRI. Data were analyzed Two patients were presented in our case series. The first one to search a correlation between the anatomical position of contacts suffered of severe TBI following an aggression with a Glasgow coma and analgesic effects. ARTICLE IN PRESS

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

Results Conclusion Post implantation analgesic effects were obtained in 81.81^%of Cardiovascular disorders are more characteristic of PD, according patients. The post-operative 3D CT analysis shows a correspondence to the type of orthostatic hypotension and arterial hypotension after between the effective contacts localization and the motor cerebral eating, than secondary and tertiary Parkinsonism. With the help of cortex somatotopy in the patients with post-operative good these disorders it is possible to differentiate the forms and course of analgesic effects. No correspondence was found between the the disease. contacts localization and the motor cerebral cortex somatotopy in the 4 patients with no analgesic effects. In three out of these four doi:10.1016/j.jns.2019.10.1164 patients, analgesic effects were obtained after a new surgery allowing a replacement of the electrode position over the motor cortex somatotopy corresponding to the painful area. WCN19-0341 Conclusion This study shows the correlation between position of the contact over the precentral cortex and the analgesia obtained when the Poster shift 03 - Autonomic nervous system disorders / history of somatotopy of the stimulated cortex correspond to the painful area. neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular doi:10.1016/j.jns.2019.10.1163 disorders and nutritional disorders or intoxications

Prevalence of comorbidities in relapsing-remitting and secondary progressive multiple sclerosis WCN19-0336 A.H.M. Kurnaza, M.D. Ünlüb, S. Demircib a Poster shift 03 - Autonomic nervous system disorders / history of Süleyman Demirel University School of Medicine, Isparta, Turkey bSüleyman Demirel University School of Medicine, Neurology, Isparta, neurology /motor neuron disease / movement disorders / MS & Turkey demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications Background Comorbidity may have an impact on multiple sclerosis course.The Cardiovascular disorders in parkinsonism depending on the form prevalence of comorbidities may vary along with disease duration as of the disease the patients get older.

a b b R. Matmurodov , K. Khalimova , E. Abdukodirov Objective a Tashkent, Neurology, Tashkent, Uzbekistan To compare the prevalence of comorbidity between relapsing- b Tashkent Medical Academy, Neurology, Tashkent, Uzbekistan remitting(RRMS) and secondary progressive MS (SPMS) patients.

One of the non-motor autonomic disorders of Parkinsonism is Methods cardiovascular disorders, which depend not only on the form and Medical records of a total of 126 MS-patients(with no comorbid- stage of the disease. ities on MS onset) evaluated retrospectively. The group is composed of 94 with RRMS and 32 with SPMS.Mean age was 43.1 ± 12.6 years Aim (18–72 years) and 70.6% was female.Disease duration was 11.4 ± To study cardiovascular disorders in Parkinsonism depending on 6.5 years. the form of the disease. Results Material and methods ^SPMS patients were older and had higher EDSS scores than 103 patients with PD were examined, 51 of them were primary RRMS patients.Presence of any comorbidity was similar in both parkinsonism, 44 were secondary parkinsonism, and 8 were tertiary groups(70.2% in RRMS,78.1%in SPMS, p = .495). The most common parkinsonism. The mean age of patients was from 33 to 65 years comorbidity was the psychiatric ones(57 had depression, 4 had (mean age 46.7 ± 8.1 years). It can be differentiated. All patients bipolar disorder and 10 had anxiety disorder).Other common were examined cardiovascular disorders, the type of orthostatic comorbidites were fatigue(45.2%), headache of any type(31.2%), hypotension and arterial hypothesis after eating and arterial pain(29.8%),hypothyroidism(11.1%), hypertension(9.5%), hyper- hypertension in the bed. cholesterolemia(8.7%) and hypertriglyceridemia(7.1%). Other comorbidities(hepatitis, asthma, epilepsy, thalassemia, FMF and Results malignancy-1breast and 1thyroid-papillary-carcinoma, psoriasis, After our careful analysis, the results show that with PD, allergy) were present in less than 5% of patients. Fatigue, orthostatic hypotension is 5.4 ± 2.1 points, hypotension after eating depression and pain were more common in SPMS patients, but is 4.3 ± 1.8 points, arterial hypertension is lower than 4.1 points, and only fatigue showed a statistical signiﬁcance(p = .000).Headache in secondary parkinsonism, these indicators were 3.8 ± 2.4–2.9 ± was more prevalent in RRMS group.In RRMS group EDSS showed 2.0–3.2 ± 2.6 points. When, as with tertiary Parkinsonism, 4.2 ± moderate correlation with the presence of depression, pain and 2.7–3.3 ± 1.8–3.6 ± 2.6 points. The total points for PD are 13.8, for fatigue. In both groups the presence of depression showed positive secondary Parkinsonism, − 9.9 and for tertiary parkinsonism −11.1 correlations with headache, fatigue and pain and these were more points. When PD was expressed orthostatic hypotension and arterial frequent in middle age group.Epilepsy and vascular comorbidities hypotension after eating. Arterial hypotension in bed is more like hypertension, hypercholesterolemia and hypertriglyceridemia common in tertiary Parkinsonism. were more frequent in the elderly. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 201

Conclusion WCN19-0351 Associated comorbidities may have an impact on physical and psychological well-being of patients and may change with age. Poster shift 03 - Autonomic nervous system disorders / history of doi:10.1016/j.jns.2019.10.1165 neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications

WCN19-0348 Incidence of treatment-emergent adverse events in Parkinson's Disease patients according to baseline body mass index: Post-hoc analysis from double-blind combined BIPARK-I and II data Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & R. Costaa, J. Ferreirab, A. Leesc, E. Tolosad, W. Poewee, F. Ikedoa,M. demyelinating diseases / neuromuscular disorders / vestibular Diogof, F. Rochaf, P. Soares-da-Silvaa disorders and nutritional disorders or intoxications aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal Comparison of quantitative analysis of hand movements with bFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal gyroscope between early stage Parkinson's disease and scans cNational Hospital for Neurology and Neurosurgery, Neurology, London, without evidence of dopaminergic deficit (SWEDD) United Kingdom dHospital Clinic Universitari de Barcelona, Neurology, Barcelona, Spain H.I. Maa, D.Y. Kwonb, M.H. Parkb, J.H. Kangc eMedical University of Innsbruck, Neurology, Innsbruck, Austria aHallym University, Neurology, Gyeonggi-do, Republic of Korea fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, bKorea University, Neurology, Ansan-city, Republic of Korea Portugal cJeju National University Hospital, Neurology, Jeju-si, Republic of Korea Objective Backgrounds To evaluate the incidence of treatment-emergent adverse events Scans without evidence of dopaminergic deficit (SWEDD) is a (TEAEs) in levodopa-treated Parkinson's Disease (PD) patients with motor radiological nomenclature, which refers to patients with a normal fluctuations and taking opicapone (OPC), a once-daily COMT inhibitor that dopamine transporter scan presumed to have parkinsonism. It is proved effective in the treatment of motor fluctuations in PD patients in important to differentiate SWEDD from Parkinson's disease (PD) in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. the early stage of disease because they have similar clinical features. As bradykinesia is a cardinal clinical feature of parkinsonism, we Methods quantitatively investigated detailed presentations of hand movement Patient-level data from matching treatment arms in BIPARK-I and by using an angular measurement system in SWEDD and PD patients, II were combined in placebo (PLC) and OPC-50 mg groups. The and normal controls. studies had similar designs (primary efficacy endpoint: change from baseline in patient diaries-based absolute OFF-time) and eligibility Methods criteria [1,2]. Safety was assessed by incidence of TEAEs, laboratory Twenty-three patients with SWEDD, 23 patients with drug- parameters, ECGs and vital signs. An exploratory post-hoc analysis naïve early stage PD, and 23 age-matched healthy controls was performed to evaluate the incidence of TEAEs according to participated in this study. Angular velocity was measured during baseline body mass index (BMI). finger-tapping and forearm-rotation movement for assessment of bradykinesia. RMS (root mean square) velocity and amplitude, Results and movement irregularity were derived from the gyro sensor Of the 1027 patients randomized to BIPARK-I and II, 522 patients signal. took a dose of study medication (OPC-50 mg n = 265, PLC n = 257) and were included in this analysis. 18–30 kg/m2 BMI patients Results represented ~78% of both OPC-50 mg and PLC groups. Overall, SWEDD patients presented a greater average speed, a larger 64.2% of OPC-50 mg patients reported any TEAE compared to 57.2% average amplitude, and more irregular speed and amplitude only in PLC. There was a comparable-to-overall incidence of TEAEs for 18– during forearm rotation compared to PD patients (p b .05). SWEDD 30 kg/m2 BMI-matched patients (65.9% vs. 55.5% for OPC-50 mg and patients also exhibited a smaller average speed and average PLC groups). In contrast, for N30 kg/m2 BMI-matched patients, TEAEs' amplitude, and a more irregular velocity usually during finger incidence was lower in OPC-treated patients (54.5%) than PLC- tapping compared to controls (p b .05). treated ones (63.0%). The most frequently occurring TEAE was dyskinesia in both subgroups. Conclusions Quantitative variables of forearm rotation movement Conclusion showed significant differences between SWEDD and PD patients, Opicapone appears to be well-tolerated in both 18–30 kg/m2 and and quantitative variables of finger tapping movement showed a N 30 kg/m2 BMI patients. significant difference between SWEDD patients and the control group. These results suggest that quantitatively determined/ References assessed variables using biomedical engineering devices can distinguish SWEDD patients from PD patients as well as from [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. normal subjects. [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. doi:10.1016/j.jns.2019.10.1166 doi:10.1016/j.jns.2019.10.1167 ARTICLE IN PRESS

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

WCN19-0352 WCN19-0353

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

Incidence of treatment-emergent adverse events in Parkinson's Incidence of treatment-emergent adverse events in Parkinson's disease patients according to baseline dopamine agonist use: disease patients according to gender: Post-hoc analysis from Post-hoc analysis from double-blind combined BIPARK-I and II double-blind combined BIPARK-I and II data data R. Costaa, J. Ferreirab, A. Leesc, E. Tolosad, W. Poewee, F. Ikedoa,D. R. Costaa, A. Leesb, J. Ferreirac, O. Rascold, F. Stocchie, F. Ikedoa,D. Magalhaesf, F. Rochaf, P. Soares-da-Silvaa Magalhaesf, F. Rochaf, P. Soares-da-Silvaa aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal Portugal bFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal bNational Hospital for Neurology and Neurosurgery, Neurology, London, cNational Hospital for Neurology and Neurosurgery, Neurology, London, United Kingdom United Kingdom cFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal dHospital Clinic Universitari de Barcelona, Neurology, Barcelona, Spain dUniversity Hospital of Toulouse, Neurology, Toulouse, France eMedical University of Innsbruck, Neurology, Innsbruck, Austria eIRCCS San Raffaele, Neurology, Rome, Italy fBIAL - Portela & Co SA, Global Parkinson's disease, S. Mamede fBIAL - Portela & Co SA, Global Parkinson's disease, S. Mamede Coronado, Portugal Coronado, Portugal Objective Objective To evaluate the incidence of treatment-emergent adverse events To evaluate the incidence of treatment-emergent adverse events (TEAEs) in levodopa-treated Parkinson's Disease (PD) patients with (TEAEs) in levodopa-treated Parkinson's Disease (PD) patients with motor fluctuations and taking opicapone (OPC), a once-daily COMT motor fluctuations and taking opicapone (OPC), a once-daily COMT inhibitor that proved effective in the treatment of motor fluctuations inhibitor that proved effective in the treatment of motor fluctuations in PD patients in two large, pivotal, multinational trials (BIPARK-I in PD patients in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. and II) [1,2]. Methods Methods Patient-level data from matching treatment arms in BIPARK-I and Patient-level data from matching treatment arms in BIPARK-I and II were combined in placebo (PLC) and OPC-50 mg groups. The II were combined in placebo (PLC) and OPC-50 mg groups. The studies had similar designs (primary efficacy endpoint: change from studies had similar designs (primary efficacy endpoint: change from baseline in patient diaries-based absolute OFF-time) and eligibility baseline in patient diaries-based absolute OFF-time) and eligibility criteria [1,2]. Safety was assessed by incidence of TEAEs, laboratory criteria [1,2]. Safety was assessed by incidence of TEAEs, laboratory parameters, ECGs and vital signs. An exploratory post-hoc analysis parameters, ECGs and vital signs. An exploratory post-hoc analysis was performed to evaluate the incidence of TEAEs according to was performed to evaluate the incidence of TEAEs according to gender. baseline dopamine agonist (DA) use. Results Results Of the 1027 patients randomized to BIPARK-I and II, 522 patients Of the 1027 patients randomized to BIPARK-I and II, 522 patients took a dose of study medication (OPC-50 mg n = 265, PLC n = 257) took a dose of study medication (OPC-50 mg n = 265, PLC n = 257) and were included in this analysis. Male patients represented ~58% of and were included in this analysis. Non-DA user patients represented both OPC-50 mg and PLC groups. Overall, 64.2% of OPC-50 mg ~30% of both OPC-50 mg and PLC groups and used higher amount of patients reported any TEAE compared to 57.2% in PLC. There was a levodopa at baseline. Overall, 64.2% of OPC-50 mg patients reported comparable incidence of TEAEs for Male-matched user patients any TEAE compared to 57.2% in PLC. Incidence of TEAEs was 59.4% (65.0% vs. 56.3% for OPC-50 mg and PLC groups) and Female- (OPC-50 mg) and 56.1% (PLC) for DA-matched user patients and matched patients (62.9% vs. 58.3% for OPC-50 mg and PLC groups). 74.1% (OPC-50 mg) and 60.0% (PLC) for non-DA-user patients. The The most frequently occurring TEAE was dyskinesia in both most frequently occurring TEAE was dyskinesia in both subgroups. subgroups.

Conclusion Conclusion Opicapone appears to be well-tolerated in both DA and non-DA Opicapone appears to be equally well-tolerated in both Male and user patients. Female patients.

References References

[1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. doi:10.1016/j.jns.2019.10.1168 doi:10.1016/j.jns.2019.10.1169 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 203

WCN19-0354 WCN19-0355

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

Incidence of treatment-emergent adverse events in Parkinson's Incidence of treatment-emergent adverse events in Parkinson's disease patients according to baseline disease severity: Post-hoc disease patients according to baseline mao-b inhibitors use: Post- analysis from double-blind combined BIPARK-I and II data hoc analysis from double-blind combined BIPARK-I and II data

R. Costaa, A. Leesb, J. Ferreirac, O. Rascold, F. Stocchie, H. Gamaa,D. R. Costaa, J. Ferreirab, A. Leesc, E. Tolosad, W. Poewee, H. Gamaa,D. Magalhaesf, F. Rochaf, P. Soares-da-Silvaa Magalhaesf, F. Rochaf, P. Soares-da-Silvaa aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal Portugal bNational Hospital for Neurology and Neurosurgery, Neurology, London, bFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal United Kingdom cNational Hospital for Neurology and Neurosurgery, Neurology, London, cFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal United Kingdom dUniversity Hospital of Toulouse, Neurology, Toulouse, France dHospital Clinic Universitari de Barcelona, Neurology, Barcelona, Spain eIRCCS San Raffaele, Neurology, Rome, Italy eMedical University of Innsbruck, Neurology, Innsbruck, Austria fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Portugal Portugal

Objective Objective To evaluate the incidence of treatment-emergent adverse events To evaluate the incidence of treatment-emergent adverse events (TEAEs) in levodopa-treated Parkinson's Disease (PD) patients with (TEAEs) in levodopa-treated Parkinson's Disease (PD) patients with motor fluctuations and taking opicapone (OPC), a once-daily COMT motor fluctuations and taking opicapone (OPC), a once-daily COMT inhibitor that proved effective in the treatment of motor fluctuations inhibitor that proved effective in the treatment of motor fluctuations in PD patients in two large, pivotal, multinational trials (BIPARK-I in PD patients in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. and II) [1,2].

Methods Methods Patient-level data from matching treatment arms in BIPARK-I and II Patient-level data from matching treatment arms in BIPARK-I and were combined in placebo (PLC) and OPC-50 mg groups. The studies II were combined in placebo (PLC) and OPC-50 mg groups. The had similar designs (primary efficacy endpoint: change from baseline studies had similar designs (primary efficacy endpoint: change from in patient diaries-based absolute OFF-time) and eligibility criteria baseline in patient diaries-based absolute OFF-time) and eligibility [1,2]. Safety was assessed by incidence of TEAEs, laboratory parame- criteria [1,2]. Safety was assessed by incidence of TEAEs, laboratory ters, ECGs and vital signs. An exploratory post-hoc analysis was parameters, ECGs and vital signs. An exploratory post-hoc analysis performed to evaluate the incidence of TEAEs according to baseline was performed to evaluate the incidence of TEAEs according to disease severity (assessed as modified Hoehn and Yahr, H&Y). baseline MAO-B inhibitors (MAOBi) use.

Results Results Of the 1027 patients randomized to BIPARK-I and II, 522 patients Of the 1027 patients randomized to BIPARK-I and II, 522 patients took took a dose of relevant study medication (OPC-50 mg n = 265, PLC n a dose of relevant study medication (OPC-50 mg n = 265, PLC n = 257) = 257) and were included in this analysis. H&Y ≥2.5 patients and were included in this analysis. Non-MAOBi user patients represented represented ~57% of both OPC-50 mg and PLC groups. Overall, ~80% of both OPC-50 mg and PLC groups. In total, 64.2% of OPC-50 mg 64.2% of OPC-50 mg patients reported any TEAE compared to 57.2% patients reported any TEAE compared to 57.2% in PLC. There was a in PLC. There was a comparable incidence of TEAEs for both H&Y b comparable-to-overall incidence of TEAEs for non-MAOBi matched user 2.5-matched patients (66.4% vs. 63.2% for OPC-50 mg and PLC patients (62.5% vs. 53.8% for OPC-50 mg and PLC groups). In contrast, for groups) and H&Y ≥2.5-matched patients (62.5% vs. 52.4% for OPC- MAOBi-matched patients, incidence of TEAEs was lower in OPC-treated 50 mg and PLC groups). The most frequently occurring TEAE was patients (70.2%) than PLC-treated ones (71.4%), but overall higher. Most dyskinesia in both subgroups. frequently occurring TEAE was dyskinesia in both subgroups.

Conclusion Conclusion Opicapone appears to be well-tolerated in both 2.5 ≤H&Y and Opicapone appears to be well-tolerated in both MAOBi and non- H&Y b 2.5 patients. MAOBi user patients.

References References

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

WCN19-0357 WCN19-0358

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

Incidence of treatment-emergent adverse events in Parkinson's Influence of motor fluctuations duration, levodopa dose and disease patients according to race: Post-hoc analysis from double- duration of use in efficacy responses of Parkinson's disease blind combined BIPARK-I and II data patients: Post-hoc analysis from combined BIPARK-I and II

R. Costaa, A. Leesb, J. Ferreirac, O. Rascold, F. Stocchie, H. Gamaa,D. A. Santosa, A. Leesb, J. Ferreirac, O. Rascold, A. Antoninie, F. Ikedoa,D. Magalhaesf, F. Rochaf, P. Soares-da-Silvaa, N. Savica Magalhaesf, F. Rochaf, P. Soares-da-Silvaa, N. Savica aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal Portugal bNational Hospital for Neurology and Neurosurgery, Neurology, London, bNational Hospital for Neurology and Neurosurgery, Neurology, London, United Kingdom United Kingdom cFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal cFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal dUniversity Hospital of Toulouse, Neurology, Toulouse, France dUniversity Hospital of Toulouse, Neurology, Toulouse, France eIRCCS San Raffaele, Neurology, Rome, Italy eUniversity of Padova, Neurology, Padova, Italy fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, fBIAL - Portela & Co SA, Global Parkinson's disease, S. Mamede Portugal Coronado, Portugal

Objective Objective To evaluate the incidence of treatment-emergent adverse events To evaluate the impact of motor fluctuations (MF) duration, (TEAEs) in levodopa-treated Parkinson's Disease (PD) patients with levodopa dose and levodopa duration of use (LDU) in efficacy motor fluctuations and taking opicapone (OPC), a once-daily COMT responses of levodopa-treated Parkinson's Disease (PD) patients inhibitor that proved effective in the treatment of motor fluctuations with MF. Opicapone (OPC), a once-daily COMT inhibitor, proved in PD patients in two large, pivotal, multinational trials (BIPARK-I effective in the treatment of MF in PD in two large, pivotal, and II) [1,2]. multinational trials (BIPARK-I and II) [1,2].

Methods Methods Patient-level data from matching treatment arms in BIPARK-I and Patient-level data from matching treatment arms in BIPARK-I and II were combined in placebo (PLC) and OPC-50 mg groups. The II were combined in placebo (PLC) and OPC-50 mg groups. Studies studies had similar designs (primary efficacy endpoint: change from had similar designs (primary efficacy endpoint: change from baseline baseline in patient diaries-based absolute OFF-time) and eligibility in patient diaries-based absolute OFF-time) and eligibility criteria criteria [1,2]. Safety was assessed by incidence of TEAEs, laboratory [1,2]. This post-hoc analysis assessed influence of MF duration parameters, ECGs and vital signs. An exploratory post-hoc analysis (years), levodopa dose (mg) and LDU (years) in efficacy outcomes was performed to evaluate the incidence of TEAEs according to race (OFF-/ON-time change from baseline). Applied linear regression's (Caucasian vs Asian). slope was statistically tested for deviation from zero.

Results Results Of the 1027 patients randomized to BIPARK-I and II, 522 patients 1027 patients were randomized to BIPARK-I and II; Full Analysis took a dose of relevant study medication (OPC-50 mg n =265,PLCn Set comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. Due to = 257) and were included in this analysis. Caucasian patients lack of matched-patients, four OPC-50 mg patients (MF N 12 years) represented ~85% of both OPC-50 mg and PLC groups. Overall, 64.2% were excluded from MF analysis and two PLC (b1 year) and five OPC- of OPC-50 mg patients reported any TEAE compared to 57.2% in PLC. 50 mg (N18 years) patients were excluded from LDU analysis. Mean There was a comparable-to-overall incidence of TEAEs for Caucasian- baseline values were 2.4 (PLC) to 2.5 (OPC-50 mg) years of MF, 696 matched patients (65.8% vs. 58.3% for OPC-50 mg and PLC groups). In mg (PLC) to 698 mg (OPC-50 mg) of levodopa and 6.4 (PLC) to 6.0 contrast, for Asian-matched patients, the incidence of TEAEs was (OPC-50 mg) years of use. OFF-/ON-time magnitude of responses lower in OPC treated patients (51.5%) than PLC-treated ones (52.4%). was not influenced by any parameter. Linear regression was non- The most frequently occurring TEAE was dyskinesia in both subgroups. significant for each arm and variables analyzed.

Conclusion Conclusion Opicapone appears to be well-tolerated in both Caucasian and MF duration, levodopa dose and LDU do not appear to impact Asian patients. OFF-/ON-time magnitude of responses in a clinical study setting.

References References

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 205

WCN19-0359 WCN19-0363

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

Influence of baseline off-time in the efficacy response of Psychiatric disorders in PARKINSON's disease Parkinson's disease patients with motor fluctuations: Post-hoc analysis from combined BIPARK-I and II H. Benjebara, I. Najmi, N. Chtaou, Z. Souirti, A. El Midaoui, M.F. Belahsen A. Santosa, J. Ferreirab, A. Leesc, H. Reichmannd, W. Poewee, F. Ikedoa, Neurology, Fez, Morocco D. Magalhaesf, F. Rochaf, P. Soares-da-Silvaa, N. Savica aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Background^ Portugal Parkinson's disease (PD) is a chronic neurological disease that can b Faculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal lead to physical and psychological impairment. Although the motor c National Hospital for Neurology and Neurosurgery, Neurology, London, symptoms are preponderant, psychiatric disorders becoming very United Kingdom common today. The objective of our study was to evaluate d University of Dresden, Nurology, Dresden, Germany psychiatric disorders in a population with PD. eMedical University of Innsbruck, Neurology, Innsbruck, Austria fBIAL - Portela & Co SA, Global Parkinson's disease, S. Mamede Materials and methods^ Coronado, Portugal We report a series of 50 patients followed for Parkinson's disease in the Department of Neurology of CHU Hassan II Fes after the exclusion Objective of other causes of parkinsonism. For each patient, we established an To evaluate the impact of baseline OFF-time on the efficacy of exploitation file to collect the epidemiological, clinical and psychiatric opicapone (OPC) in levodopa-treated Parkinson's Disease (PD) disorders evaluated in patients with Parkinson's disease. patients with motor fluctuations. OPC, a once-daily COMT inhibitor, fl proved effective in the treatment of motor uctuations in PD patients Results^ in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. 50 patients were included: 22 women and 28 men, the mean age was 58 years (38–83). The behavioral changes evaluated by the Methods PDQ39 self-questionnaire were found in the majority of cases, Patient-level data from matching treatment arms in BIPARK-I and marked by isolation (33 patients), a depression in 88% of cases. II were combined in placebo (PLC) and OPC-50 mg groups. The Concentration disorders were found in 31 patients, 88% presented studies had similar designs (primary efficacy endpoint: change from with anxiety disorders and 58% with hallucinations. 22 cases needed baseline in patient diaries-based absolute OFF-time) and eligibility daily support from their husbands or wives, family and friends. criteria [1,2]. An exploratory post-hoc analysis was performed to fl fi evaluate the in uence of baseline OFF-time in ef cacy outcomes (i.e., Conclusion^ change from baseline in OFF-/ON-time). Linear regression was Our study evaluated psychiatric disorders in our patients; These applied and the slope was statistically tested for deviation from zero. aspects should be considered in the management of these topics. The intervention system should include in the first instance the Results psychiatric disorders in order to propose specific interventions 1027 patients were randomized to BIPARK-I and II; 522 patients adapted to the needs of each patient. took a dose of study medication; Full Analysis Set comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. As expected, both treat- doi:10.1016/j.jns.2019.10.1175 ments arms presented the same increased dependent tendency for OFF-/ON-time at endpoint, i.e., the longer OFF-time at baseline the greater magnitude of response at endpoint (ending in a linear regression slope statistically significant). When OFF-time at endpoint WCN19-0395 was ‘normalized’ as % of change in relation to baseline, the linear regression slope was found non-significant for both treatment arms, though the increased dependent tendency was still found. Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & Conclusion demyelinating diseases / neuromuscular disorders / vestibular Baseline OFF-time appears to impact the magnitude of both OFF- disorders and nutritional disorders or intoxications and ON-time responses in a clinical study setting. Phantom pseudoathetosis: Successful treatment with pregabalin References N. Biary, N. Alnawshan, W. Khoja [1] Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. Prince Sultan Military Medical City, Neurology, Riyadh, Saudi Arabia [2] Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206.

Background^ doi:10.1016/j.jns.2019.10.1174 Pseudoathetosis is caused by loss of proprioception and has been reported to improve with pregabalin. ARTICLE IN PRESS

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

fi Objective^ 7 (43.75%)-of them 5 (31.25%)were task-speci c related to speaking We report a patient who was diagnosed with phantom pseudo- and reciting Quran verse,mixed type 4(25%)-of them 2(12.5%)were athetosis that subsequently improved after administration of task-specific related to eating and causing weight reduction),jaw pregabalin. opening(JOD) 3(18.75%)and 2(12.5%)jaw deviation.Selection of muscles for injection was individualized according to the type of OMD Case report and the pattern of muscle involvement, EMG-guidance used for fi Â 56-year-old man with diabetic polyneuropathy had bilateral injecting dif cult cases especially those with complex muscle above the knee amputations (AKA) secondary to ulcerations and involvement to improve localization especially when injecting the gangrenous changes in his legs. He also had chronic renal failure and pterygoids, digastric or intrinsic tongue muscles. JCD commonly was on regular dialysis. He felt phantom pain in his feet shortly after injected muscles were masseter and temporalis, while medial the amputations and then began feeling abnormal movements in his pterygoids in refractory cases.JOD lateral pterygoids, platysma and amputated legs two years following the AKA. The patient had mild anterior belly of digastric. Contralateral Lateral pterygoid and pseudoathetosis in his hands, which significantly decreased when he ipsilateral temporalis in jaw deviation, Genioglossus and Hyoglossus looked at his hands. He mimicked the phantom movements of his in tongue protrusion, other muscles injected were orbicularis oris, feet with his hands, and these movements were severe undulating mentalis,platysma,depressor anguli,levator labii and other intrinsic and writhing movements of his toes. The patient was started on muscles of the tongue. Mild and transient dysphagia in 4(25%) pregabalin 75 mg orally every other day, with an addition 75 mg patients and no serious side effects observed. given after each dialysis session. This regimen resulted in a significant improvement of both his pain, his pseudoathetosis and Conclusion his phantom pseudoathetosis. BTX is useful treatment of OMD with minimal complications. A video of these movements was recorded with the patient's consent. doi:10.1016/j.jns.2019.10.1177

Conclusion^ We report a case of a patient who had improvement of his phantom pseudoathetosis following pregabalin administration. To WCN19-0417 our knowledge, this is the ﬁrst report of phantom pseudoathetosis. Further study is warranted to explore both this phenomenon and the use of pregabalin as a therapy. Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & doi:10.1016/j.jns.2019.10.1176 demyelinating diseases / neuromuscular disorders / vestibular disorders and nutritional disorders or intoxications

Amelioration of cognitive, motor and endogenous defense WCN19-0397 functions by Gingko Biloba in D-galactose induced mice model of neurodegeneration Poster shift 03 - Autonomic nervous system disorders / history of P. Rane neurology /motor neuron disease / movement disorders / MS & D.Y. Patil Institute of Pharmaceutical Sciences and Research, Pharma- demyelinating diseases / neuromuscular disorders / vestibular cology, Pune, India disorders and nutritional disorders or intoxications

Background^ Botulinum toxin a for oromandibular dystonia: Individualized The role of advanced glycation end products (AGEs) in AD due to approach and clinical experience increased cytotoxicity via oxidative stress. D-galactose (D-gal) induced amnesia stimulates Aβ overproduction via increased ab bc D. Youssry , J. Alhashel oxidative stress and AGEs. Gingko Biloba (GB), a naturally occurring a Cairo University, Neurology Departement, Cairo, Egypt plant has been reported to have neuroprotective potential in many b Ibn Sina Hospital, Neurology, Kuwait, Kuwait brain disorders. cKuwait Univeresity, Medical Departement, Kuwait, Kuwait Materials and methods^ Oromandibular dystonia(OMD)is a focal dystonia involves mouth, In the present study we have investigated the possible neuro- jaw and tongue muscles, responds poorly to oral medications and protective effect of GB against in-vitro AGEs formation and in-vivo. Botulinum toxin(BTX)is treatment of choice. Because of complex Chronic administration of D-gal (150 mg/kg) for 6 weeks in mice muscle activity and involvement of several small muscles OMD is increases AGEs, we consequently investigated the neuroprotective difficult to treat. role of GB (50 and 100 mg/kg as per body weight) against D-gal induced amnesia. Behavioral assessments in Morris water maze Objectives (MWM) and Y-maze were performed, followed by biochemical We present our experience of OMD patients treated with BTX. estimations to find out the plausible mechanism of action. Sixteen OMD patients treated with BTX at our injection clinic, 9 (65.25%)females and 7(43.75%)males, their age ranged from 21 to Results^ 52 years. Duration of OMD ranged from 1 to 6 years, duration of The results of in vitro experiments showed that GB alleviates early BTX treatment ranged from 6 months-9 years.13(81.25%) patients and late AGEs formation. Chronic (6 weeks) D-gal administration were idiopathic, 2(12.5%)tardive dyskinesia and one (6.25%)with considerably impaired cognitive performance in MWM and Y maze, neurodegenerative disorder. Other types of dystonia were noted in caused marked oxidative damage, elevated the AGEs levels and 2 (12.5%)patients. Regarding the type of OMD; jaw closure(JCD) significantly increased the acetylcholinesterase activity as compared ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 207 to sham group. GB treatment at different doses (50 and 100 mg/kg) WCN19-0421 able to restore the cognitive performance, reversed the oxidative fi damage^, decreased AGE levels and caused signi cant reduction in acetylcholine esterase levels as compared to D-gal group. Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Conclusion^ The outcome of present study provides a strong justification for disorders and nutritional disorders or intoxications the neuroprotective role of GB against D-gal induced amnesia due to the antioxidant, antiglycative and anticholinesterase properties. Association of infection with flaviviruses with occurrence of Guillain-Barre syndrome (GBS) doi:10.1016/j.jns.2019.10.1178 A. Goonatilakaa, L. Gomesb, S. Dayarathnaa, N. Malavigeb, T. Changa aUniversity of Colombo, Department of Clinical Medicine, Colombo, Sri Lanka WCN19-0420 bUniversity of Sri Jayewardenepura, Centre for Dengue Research, Nugegoda, Sri Lanka Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & Objectives Antecedent infections are common in GBS and are thought to demyelinating diseases / neuromuscular disorders / vestibular trigger its immunopathogenesis. Although several associated viruses disorders and nutritional disorders or intoxications have been identified, apart from Zika, the other flaviviruses have not been systematically studied. We aimed to characterize the associa- Neuroprotective role of silyamarin in stereotaxically rotenone- tion of flaviviruses with GBS. induced rat model of Parkinson's disease Methods D. Khatri We recruited 37 patients with GBS admitted to the National Institute of Chemical Technology, Department of Pharmacology, Mum- Hospital of Sri Lanka during 2017 and 2018. Nerve conduction bai, India studies and CSF analysis were performed in all and clinical and laboratory data were recorded. IgM and IgG antibodies for dengue and IgM antibodies for Japanese Encephalitis (JE), West Nile virus Background^ Silybum marianum L. (Milk thistle), a member of Carduus (WNV) and Zika were performed on serum samples. marianum family, is an ancient medicinal plant which has been used for centuries for treatment of different diseases. Silymarin is a Results polyphenolic flavanoid of Silybum marianum, a potent antioxidant, The mean age of the patients was 48 (SD ±20years)with62.2% which crosses the blood brain barrier and elicited neuroprotection males. Mean duration since onset was 11 days (SD ± 6). Six patients and antidepressant like activity in stressed model. had AMAN, 30 had AIDP and one had Miller Fisher syndrome. 18.9% (7/ 37) had evidence of a recent flavivirus infection. 6/37 patients had dengue virus-specific antibodies and one patient had positive dengue Materials and methods^ In the present study we used Rotenone a neurotoxic to induce NS1 antigen without any detectable antibodies to any of the flaviviruses. Parkinson symptoms. A pre-treatment with Silymarin (50, 100, 200 Only one of the patients who had dengue specific-antibodies reported mg/kg body weight p.o.) once daily for 7 days was given to male an acute dengue infection, 2 weeks prior to GBS. The patient with a wistar rat. On the 8th day animals were subjected to unilateral positive dengue NS1 antigen had thrombocytopenia but did not develop μ dengue haemorrhagic fever. WNV-specific IgM was detected in one intrastriatal injection of rotenone (8 g in 0.1^% ascorbic acid in normal saline). The Silyamarin treatment was continued for patient in the absence of IgM antibodies to other flaviviruses. Two subsequent two weeks after rotenone infusion. Rats were tested for patients who had dengue specific-antibodies also had antibodies to JE neurobehavioral activity and were sacrificed for estimation of lipid possibly due to the presence of cross-reactive antibodies. peroxidation (TBARS), total glutathione (GSH) content, and activity of antioxidant enzymes glutathione peroxidase (GPx), catalase Conclusion (CAT), and superoxide dismutase (SOD) in brain homogenates. Flavivirus infection appears to be frequently associated with GBS in Sri Lanka. Results^ Three weeks of oral administration of Silyamarin significantly doi:10.1016/j.jns.2019.10.1180 attenuated motor disabilities (actophotometer, rotarod and Morris water maze test). Rat treated with rotenone showed reduced levels of thiobarbituric acid reactive substance (TBARS) and increased level of GSH content and antioxidants enzymes activities (GPX, SOD and WCN19-0423 CAT) in the Silyamarin treated PD rat.

Poster shift 03 - Autonomic nervous system disorders / history of Conclusion^ The outcome of present suggest that Silyamarin is a potential neurology /motor neuron disease / movement disorders / MS & drug for treating oxidative damage, physiological abnormalities and demyelinating diseases / neuromuscular disorders / vestibular is effective in neuroprotection in experimental models of PD. disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1179 Acute encephalopathy followed by delayed myelopathy a rare presentation of chlorpyriphos (organophosphorus) poisoning ARTICLE IN PRESS

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

A. Agarwal, V. Goyal, V. Vy, M. Singh Bhushan, A. Garg, M.P. evaluated by FDG PET CT on a separate day and patterns of Srivastava hypometabolism recorded. All India Institute of Medical Sciences, Neurology, New Delhi, India Results Organophosphorus compounds (OPC) are commonly used pesti- Reduced striatal uptake of 99mTc-TRODAT-1 with increasing cides and suicidal ingestion is common mode of poisoning. The disease severity was found in Parkinson's disease patients with a manifestation of OPC poisoning and its severity depend upon the significant rostro-caudal gradient. The mean putamen uptake type, dose and potency of the OPC consumed. Neurological contralateral to the more affected limbs was significantly decreased presentations are well defined clinical syndromes consisting of early, compared to the ipsilateral sides. Significant reduction of 99mTc- intermediate and delayed manifestations (rare), categorized on the TRODAT-1 was also found in atypical PD and other parkinson plus basis of time elapsed since OPC exposure. A 21-years-old male syndromes. FDG PET CT images showed normal to increased FDG presented to us with 100 ml of pesticide consumption (containing uptake in the basal ganglia of patients suspected or diagnosed as PD. chlorpyriphos 50% and cypermethrin 5%) 18 months prior to However different patterns of basal ganglionic and cortical hypo presentation. Following intake, he developed one episode of seizure metabolism noted in patients with Parkinson plus syndromes. followed by altered sensorium for 3 weeks during which he was treated with atropine infusion and supportive management. He was ^Conclusion well for the next two months. Subsequently he developed insidious 99mTc-TRODAT-1, in conjunction with FDG PET CT, may serve as onset and progressive proximal followed by distal weakness leading a useful imaging agent for the early detection of Parkinson's disease to pure motor spastic paraparesis without bowel or bladder and differentiation of different Parkinson plus syndromes. involvement. He had brisk lower limb reflexes with extensor plantar response. Routine investigations were normal. Electrophysiological doi:10.1016/j.jns.2019.10.1182 studies (nerve conduction studies, electromyography, visual evoked potentials, brainstem auditory evoked potentials and somatosensory evoked potentials[SSEP] in upper limbs) were normal. SSEP of lower limb showed prolonged potentials beyond the popliteal fossa with WCN19-0430 normal upper limbs. MRI of the spine showed dorsal cord atrophy and MRI brain was normal. This case reports a rare delayed Poster shift 03 - Autonomic nervous system disorders / history of manifestation of organophosphorus poisoning in the form of pure motor spastic paraparesis due to dorsal myelopathy. A possibility of neurology /motor neuron disease / movement disorders / MS & delayed manifestations of toxicity should be considered in individ- demyelinating diseases / neuromuscular disorders / vestibular uals presenting with features suggestive of myelopathy and a disorders and nutritional disorders or intoxications previous history of organophosphate exposure. Bumetanide to treat Parkinson's disease patients suffering from doi:10.1016/j.jns.2019.10.1181 freezing of gait resistant to both dopamine replacement treatment and STN DBS: An open-label pilot study (BUMFOG)

W. Elsayedab, P. Damierc, J. Al Hashelad WCN19-0424 aIbn Sina Hospital, Neurology, Kuwait, Kuwait bBeni-Suef University, Neurology, Beni-Suef, Egypt cUniversity of Nantes, Neurology, Nantes, France Poster shift 03 - Autonomic nervous system disorders / history of dFaculty of Medicine, Medicine, Kuwait, Kuwait neurology /motor neuron disease / movement disorders / MS & demyelinating diseases / neuromuscular disorders / vestibular Objectives disorders and nutritional disorders or intoxications To investigate the effects of bumetanide in Parkinson's disease (PD) patients suffering from freezing of gait (FOG) resistant to Complementary role of 99m-Tc-TRODAT-1 and FDG PET-CT in dopamine replacement therapy and subthalamic (STN) DBS. evaluating patients of Parkinson's disease and other movement disorders: 7 years experience from a tertiary care center Outcomes Primary outcome measure: change in the number of steps and a b b b R. Verma , R. Ranjan , C. Agrawal , A. Batra the completion time during the stand-walk-sit (SWS) test^,performed aConsultant Physician, Nuclear Medicine, New Delhi, India in OFF and ON-drug stage. Secondary outcome measures: MDS- b Sir Ganga Ram Hospital, Neurology, New Delhi, India UPDRS III,Giladi's questionnaire^, patients' 2 week falls diary, Blood potassium. Aims and objectives To gain ﬁrsthand experience in documenting the complementary Results role of 99mTc-TRODAT-1 and FDG PET CT imaging in the Six PD patients treated by STN DBS and suffering from disabling diagnosis and differentiation of various parkinsonian movement FOG were included (age 61 ± 12 yrs., 4 males, disease duration 23 ± disorders. 7 yrs., duration of STN DBS 9.6 ± 2.9 yrs., with good to excellent improvement (CGI-Patient) 1 yr after lead implantation, dopamine Methods replacement treatment 640 ± 310 mg LED. Bumetanide was titrated 850 patients with movement disorders were evaluated over a until 1 mg bid. All patients complained from a polyuria leading two period of 7 years, using 99mTc-TRODAT-1. Speciﬁc uptake in the patients to withdraw from the trial. Potassium decreased after 1- striatum and its sub regions, including the putamen and caudate month bumetanide treatment (from 3.9 ± 0.3 to 3.6 ± 0.5) without b nucleus, was calculated and compared with that of the other sides reaching values ^2.5 meq/l.After 3 months of bumetanide treatment: and of healthy volunteers. Approximately 700 patients were further SWS completion time performed in the OFF-drug stage decreased ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 209

fl (49.2 ± 23.2 s vs. 61.2 ± 20.6 s at baseline^, p = .03)and gait assessed decreases uctuations in plasma concentrations of levodopa, reduces on the Giladi's questionnaire improved (7.6 ± 3.5 vs. 14.6 ± 1.5). No NMS burden and motor fluctuations in APD, however; adequate dose significant changes on the MDS-UPDRS III in the OFF or ON-drug modification and individualization of therapy are essential for condition were observed. optimal effect.

Conclusion doi:10.1016/j.jns.2019.10.1184 This pilot study found that bumetanide improves FOG resistant to dopamine replacement tretment and DBS in PD. These results call for a double-blind placebo controlled study. doi:10.1016/j.jns.2019.10.1183 WCN19-0434

Poster shift 03 - Autonomic nervous system disorders / history of WCN19-0431 neurology /motor neuron disease / movement disorders / MS & 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 & Safety and tolerability data of a new brand-generic product of demyelinating diseases / neuromuscular disorders / vestibular teriﬂunomide in iranian relapsing-remitting multiple sclerosis disorders and nutritional disorders or intoxications patients: An observational cohort study

Levodopa/carbidopa intestinal gel in treatment of non-motor R. Abolfazlia, A. Hekmatb, M. Ghazaeianc, S. Samadzadehd symptoms in advanced Parkinson's disease aTehran University of Medical Sciences. Amiralam Hospital, Department of Neurology, Tehran, Iran W. Elsayeda, J. Al Hashelab bZistdaru Danesh Pharmaceutical Company, Medical Department, aIbn Sina Hospital, Neurology, Kuwait, Kuwait Tehran, Iran bFaculty of Medicine, Medicine, Kuwait, Kuwait cMazandaran University of Medical Sciences, Faculty of Pharmacy, Department of Clinical Pharmacy, Sari, Iran d Objectives Tehran University of Medical Sciences, Department of Neurology, To identify the advantages of LCIG in the treatment of NMS Tehran, Iran among APD patients and appraise the currently available literature to identify the gaps in the available evidence. Background Teriflunomide, a once-daily oral immunomodulator for the Objectives treatment of relapsing-remitting multiple sclerosis, has shown background:Levodopa is the most commonly used drug for the consistent efficacy and safety in MS patients. treatment of motor symptoms of Parkinson's Disease (PD). For managing non-motor symptoms (NMS) in advanced Parkinson's disease (APD), Objectives levodopa-carbidopa intestinal gel (LCIG) infusion is preferred over oral This study aimed to evaluate the safety and tolerability of a levodopa-carbidopa because it bypasses the gastrointestinal tract and brand-generic Teriflunomide product (Tebazio®, 14 mg tablet) overcomes fluctuation and variation in the plasma drug levels. produced by Zistdaru Danesh biopharmaceutical company in Iranian patients with relapsing-remitting MS over 12 months. Methods Levodopa is the most commonly used drug for the treatment of Methods motor symptoms of Parkinson's Disease (PD). For managing non- A non interventional cohort study was conducted on 746 patients. motor symptoms (NMS) in advanced Parkinson's disease (APD), The patients had a confirmed and documented diagnosis of RRMS as levodopa-carbidopa intestinal gel (LCIG) infusion is preferred over defined by the Revised McDonald Criteria (2010), were ambulatory oral levodopa-carbidopa because it bypasses the gastrointestinal tract with a Kurtzke Expanded Disability Status Scale score of 0 to 5.5, and and overcomes fluctuation and variation in the plasma drug levels. their treatment by Teriflunomide 14 mg was just started. Adverse drug reactions (ADRs), and its severity, laboratory tests and physical Results examinations were evaluated over 1 year. LCIG has been found to be beneficial in improving or relieving various NMS. (central nervous system symptoms, cardiovascular system symp- Results toms, gastrointestinal tract symptoms, systemic symptoms, urinary The most common adverse drug reactions were hair thinning symptoms, reproductive system symptoms) in patients with APD. (39.6%), skin dryness (10%), diarrhea (7%), alanine aminotransferase (ALT) increase (6%), and headache (5.4%) which all were mild to Conclusion moderate and rarely caused treatment discontinuation. Elevated ALT (N 3 times upper limit of normal; according to the monitoring ^LCIG provides an uninterrupted intestinal levodopa infusion by percutaneous endoscopic gastrojejunostomy (PEG-J). It effectively protocol) was the most common reason for drug discontinuation. ARTICLE IN PRESS

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

Conclusion (p = .011) has been demonstrated. Cognition, fatigue and depression The 14 mg brand-generic Teriﬂunomide product was well have been found to predict employment status in persons with MS, tolerated in Iranian RRMS patients and no new alarming signal was independent of physical MS symptoms. detected during the study period. Conclusion doi:10.1016/j.jns.2019.10.1185 It has been clearly demonstrated that work capacity in MS signiﬁcantly declines with increased physical disability and cognitive impairment.

WCN19-0435 doi:10.1016/j.jns.2019.10.1186

Poster shift 03 - Autonomic nervous system disorders / history of neurology /motor neuron disease / movement disorders / MS & WCN19-0440 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 & Working status in persons with multiple sclerosis demyelinating diseases / neuromuscular disorders / vestibular J. Drulovica, S. Mesarosa, G. Maricb, V. Martinovicc, J. Ivanovicd,M. disorders and nutritional disorders or intoxications Andabakae, T. Pekmezovicb aClinic of Neurology- CCS, Faculty of Medicine, University of Belgrade, Rituximab in neurogical diseases Department of immune-Mediated CNS Disorders, Belgrade, Serbia bFaculty of Medicine, University of Belgrade, Institute of Epidemiology, G. Ibnmajdoub Belgrade, Serbia Chu Hassan II, Neurology, Fez, Morocco cClinic of Neurology- CCS, Department of Immune-Mediated CNS Disorders, Belgrade, Serbia Introduction^ dFaculty of Medicine, University of Belgrade, Clinic of Neurology, ^RITUXIMAB: is a chimeric monoclonal antibody against the CD20 Belgrade, Serbia surface molecule Its use during certain inflammatory neurological e Faculty of Medicine, University of Belgrade, Institute of Genetics, pathologies. It has shown good Efficacy according to randomized Belgrade, Serbia trials. It is for this reason that studies based on daily practice are needed to confirm these results. Background and aim The ability of people with multiple sclerosis (MS) to maintain Objectives^ their employment is of increasing concern. To assess work status of The objective of this study is to concretely assess the tolerance patients with relapsing-remitting (RR) MS in Serbia and Montenegro and effectiveness of RITUXIMAB in the treatment of inflammatory and the influence of physical disability, depression, cognitive status pathologies, while trying to identify predictive factors response to it. and treatment with interferon (IFN)-beta 1a on the work status of MS patients and their quality of life. ^Methods^ This is a retrospective observational study, conducted in the Material and methods neurology department at the CHU Hassan II Fez, including 38 Multicentre cross-sectional study comprised RRMS patients in patients who were given Rituximab retained from May 2013 until Serbia and Montenegro treated with IFN-beta-1a sc (n = 261), and March2019. control group consisting of 239 naive RRMS patients, from five university MS centres in our countries. In all patients following were ^Result^ performed: EDSS, MSFC, the Brief International Cognitive Assessment In the 38 observations: 48% are followed for MS; 11% followed for for Multiple Sclerosis, Beck Depression Inventory, Fatigue Impact a Neurogical gougero-Sjogren syndrome, 7% for a Deuvic, 14% for Scale, MSQoL-54, and online vocational status monitoring tool. vasculitis, 7% for myasthenia, 3% for a neurowegner, 3% for autoimmune encephalitis and 3% for inflammatory meningo-myeli- Results tis. The evolution of all the patients put on treatment with Rituximab In the IFN-beta-treated group, proportion of employed persons was marked by the stabilization and limitation of relapses; exept the was significantly higher (59.4%) in comparison to 49.3% in the autoimmune encephalitis and neuro inflammatory myelitis that have untreated group (p = .024). It has been demonstrated that the worsened under treatment. crucial reason for retirement (disability pension) in RRMS patients fi was walking problem which was signi cantly more frequent (p = Discussion^ .002) in the untreated group (20.5%) in comparison to the IFN-beta- The results of our study are quite similar compared to other treated (10.3%). Via the online vocational status monitoring tool series; encouraging the use of RITUXIMAB in patients with autoim- (Serbian version), significant correlation between baseline EDSS mune neurological diseases and provide us with some assurance and score of the domain related to difficulties at working place during his administration. ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 211

^Conclusion^ WCN19-0453 Despite the fact that it is still used off-label in Neurology, RITUXIMAB is a therapeutic revolution, and becomes a key in the treatment of autoimmune neurological disease resistant to conven- Poster shift 03 - Autonomic nervous system disorders/history of tional treatments: neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- doi:10.1016/j.jns.2019.10.1187 orders and nutritional disorders or intoxications

Poor sleep quality is related to depression but not to fatigue in Indonesian multiple sclerosis patients WCN19-0447 R. Estiasari, C. Baziad, M. Hakim, D. Pangeran, K. Maharani, D. Imran Faculty of Medicine Universitas Indonesia, Neurology, Jakarta, Indonesia Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Background demyelinating diseases/neuromuscular disorders/vestibular dis- Multiple Sclerosis (MS) is a chronic progressive disease in which orders and nutritional disorders or intoxications sleep disorder, besides various neurologic manifestations, highly impacts the patients but is often neglected in clinical settings. Several Autonomic symptoms do not correlate with motor severity in studies had discovered that sleep disorder was more prevalent in MS multiple system atrophy subtypes than general population. However, study in sleep quality in MS patients in South East Asia is scarce. This study aimed to investigate D. Garga, A. Srivastava Kumarb, A. Jaryal Kumarc, R. Rajanb, A. Pandit the prevalence of sleep quality and associated factors in MS patients Kishorb, D. Vibhab, G. Shuklab, R. Pandey Mohand, K. Prasadb in Indonesia. aLady Hardinge Medical College, Neurology, New Delhi, India bAll India Institute of Medical Sciences, Neurology, New Delhi, India Methods cAll India Institute of Medical Sciences, Physiology, New Delhi, India A descriptive cross-sectional study involving MS patients was dAll India Institute of Medical Sciences, Biostatistics, New Delhi, India conducted at Dr. Cipto Mangunkusumo National General Hospital Jakarta. In addition to clinicodemographic data collection, data Autonomic dysfunction is a hallmark of Multiple System Atrophy regarding sleep quality, depression, fatigue and sleepiness were (MSA) and forms the core diagnostic feature. There is extremely assessed using Indonesian previously-validated Pittsburgh Sleep limited data on the correlation between autonomic dysfunction and Quality Index (PSQI), The Mini International Neuropsychiatric severity of motor manifestations in patients with MSA. We Interview ICD-10, Fatigue severity scale and Epworth sleepiness prospectively evaluated 100 patients with probable MSA (48 patients scale respectively. with MSA-C and 52 patients with MSA-P) diagnosed as per the 2008 Gilman criteria at our centre and assessed autonomic symptoms Results using the quantitative SCOPA-Aut scale in these patients. Motor Of forty MS participants included in this study, 32 (76.2%) aged severity was assessed using Unified MSA Rating Scale (UMSARS) part less than 40 years, 32 (80.0%) were women, 20 (50.0%) were II as well as Movement Disorders Society-Unified Parkinson's Disease unemployed, 14 (35%) had depression, and 27 (67.5%) had fatigue. Rating Scale (MDS-UPDRS) part III. The mean age of MSA-P patients Poor sleep quality was found in 33 (82.5%) participants and fi at presentation was 63.6 ± 9.7 years and for MSA-C was 58.0 ± 8.1 signi cantly correlate with depression and sleepiness. No correlation years SCOPA-Aut total score (maximum score possible being 69) was between PSQI score and fatigue unless in daytime dysfunction 19.1 ± 10.2 and 18.3 ± 7.9 for MSA-P and MSA-C patients respec- domain. tively. The mean MDS-UPDRS III score for MSA-P patients was 46.9 ± 16.6 and for MSA-C patients was 38.3 ± 16.8. UMSARS part II Conclusion score was 45.1 ± 16.9 for MSA-P and 43.5 ± 12.3 for MSA-C patients. Poor sleep quality in Indonesia MS patients is prevalent and Statistical analysis using Pearson's correlation coefficient did not associated with depression. Detection of sleep quality is important in demonstrate any correlation between autonomic symptom severity MS comprehensive care. assessed using SCOPA-Aut total score and motor severity scores for either MSA subtype. In conclusion, our study suggests that auto- Keywords: Poor sleep quality, Fatigue, MS, PSQI, MINI ICD-10, nomic dysfunction exists independent of motor severity in patients Depression, FSS with MSA. This suggests that the presence of autonomic dysfunction supports the early diagnosis of MSA even before motor symptoms have become full-fledged. Autonomic dysfunction in MSA is there- doi:10.1016/j.jns.2019.10.1189 fore an important diagnostic and therapeutic target. doi:10.1016/j.jns.2019.10.1188 ARTICLE IN PRESS

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

WCN19-0457 2017-19, Shanxi International Science and Technology cooperation project 201703D421016, Scientiﬁc and technological innovation team of integrated Chinese and western medicine for the prevention Poster shift 03 - Autonomic nervous system disorders/history of and treatment on nervous system diseases of Shanxi university of neurology/motor neuron disease/movement disorders/MS & Chinese medicine 2018TD-012, Shanxi Key Laboratory project SXIDL- demyelinating diseases/neuromuscular disorders/vestibular dis- 2018-04, Shanxi Key project 201803D31209. Ma and Xiao are orders and nutritional disorders or intoxications corresponding authors).

Efﬁcacy and mechanism of Wuzi Yanzong pill in the treatment of EAE doi:10.1016/j.jns.2019.10.1190

Z. Chaia, R.N. Zhanga, H.J. Fana, Y.R. Lia, M.Y. Suna, Q. Miaoa, J.Z. Yub,Y. H. Lib, X.M. Jinc, B. Zhangd, J.J. Huange, B.G. Xiaof, C.G. Maab aShanxi University of Chinese Medicine, The Key Research Laboratory of WCN19-0462 Benefiting Qi for Acting Blood Circulation Method to Treat Multiple Sclerosis of State Administration of Traditional Chinese Medicine- and Poster shift 03 - Autonomic nervous system disorders/history of Scientific and Technological Innovation Team of Integrated Chinese and Western M, Jinzhong, China neurology/motor neuron disease/movement disorders/MS & bShanxi Datong University, Institute of Brain Science, Shanxi Key demyelinating diseases/neuromuscular disorders/vestibular dis- Laboratory of Inflammatory Neurodegenerative Diseases, Datong, China orders and nutritional disorders or intoxications cIndiana University, Stark Neuroscience Research Institute, Indiana University School of Medicine, Indianapolis, USA The growing suicidal behavioural changes in patients with dShanxi Health Vocational College, Dept. of Traditional Chinese multiple sclerosis in Uzbekistan Medicine, Jingzhong, China e General Hospital of Datong Coalmine Group, Dept. of Neurosurgery, M. Salokhiddinova, E. Stamoub Datong, China aTashkent Medical Academy, Neurology, Olmazor, Uzbekistan f Fudan University, Institute of Neurology-Huashan Hospital-Institutes of bUniversity of Aberdeen, Medical Imaging, Aberdeen, United Kingdom Brain Science, State Key Laboratory of Medical Neurobiology, Shanghai, China Background Epidemiological data suggest that the standardized mortality Background ratio (SMR) for suicide in MS is approximately twice that of the Wuzi Yanzong Pill (WYP, a classical Chinese herbal formula) has a general population with younger males in the first few years potential therapeutic effect on experimental autoimmune encepha- following diagnosis most at risk. lomyelitis (EAE), but the mechanism is not clear. Objective Objective To analyse mental disorders in patients with suicidal behavior To determine the therapeutic effect of WYP in EAE, and explore and identify the leading somatic and socio-psychological risk factors its mechanism. for suicidal behavior of patients.

Material and methods Methods C57BL/6 mice were randomly divided into control, EAE, and high/ The study included 120 patients aged from 18 to 64 years who medium/low dose WYP groups. Mice received WYP twice daily from were treated at the 2nd Clinic of Neurology Department of 7 days before immunization to 14 and 28 days post immunization (p. Tashkent Medical Academy. The main group consisted of 85 i.) (high/medium/low dose =16/8/4 g/kg). At days 14 and 28 p.i., people while remaining 35 patients (without suicidal behaviour) spinal cord and spleen were obtained. were subsequently included in the comparison group. When studying the mental state of patients, individual interviews, direct Results observation, Spilberger-Hanin, the Hamilton scale were used. WYP treatment delayed EAE onset and relieved the severity of ﬂ ﬁ EAE accompanied by the inhibition of in ammatory cell in ltration Results and the improvement of demyelination in spinal cord. WYP The study showed that high proportion of suicidal behavior treatment may more effectively induce M2 macrophage polarization (68.0%), in the structure of which anti-vital experiences (37.6%) and γ α that was related with decreased IFN- , IL-17 and TNF- .WYP suicidal thoughts (32.1%). Less common are suicidal plans (9.6%) and treatment also inhibited the expression/activation of ROCK and TLR- intentions (8.2%). 5.8% of people committed suicidal attempts. κ 4/NF- B signaling pathway and reduced the expression of MCP-1 and Blackmail forms of suicidal behavior were detected in 6.4% of CCR-2 in brain, spinal cord and spleen compared to EAE control. patients. All cases of suicidal actions (6.4%) of patients with multiple sclerosis were true. The structure of the leading mental disorders in Conclusion patients is anxiety-depressive (37.4%), astheno-depressive (41.8%), Provides an experimental basis for further clinical application encephalopathic (2.6%), depressive-hypochondriac (2.6%) and dys- of WYP for the treatment of MS. (NNSF of China 81102552 phoric (1.2%) syndromes. and 81703978, Shanxi Scholarship Council project 2017-129 and ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 213

Conclusion OFF-/ON-time magnitude of responses was not influenced (non- Suicidal risk remains high at all stages of treatment and significant linear regression). For PLC, the higher H&Y staging at rehabilitation of patients. It requires early prevention and correction baseline the greater magnitude of OFF-/ON-time response at of suicidal behavior that are based on early detection of disorders in endpoint (ending in a linear regression slope statistically significant). the emotional sphere, complex application at all stages of treatment and rehabilitation of patients. Conclusion Disease severity impacted OFF-/ON-time magnitude of responses doi:10.1016/j.jns.2019.10.1191 for PLC but not OPC-50 mg patients in a clinical study setting.

References

WCN19-0490 [1] J.J. Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [2] A.J. Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1192 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Influence of disease severity in the efficacy response of WCN19-0494 Parkinson's disease patients with motor fluctuations: Post-hoc analysis from combined BIPARK-I and II Poster shift 03 - autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & A. Santosa, A. Leesb, J. Ferreirac, O. Rascold, A. Antoninie, H. Gamaa,D. demyelinating diseases/neuromuscular disorders/vestibular dis- Magalhaesf, F. Rochaf, P. Soares-da-Silvaa aBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, orders and nutritional disorders or intoxications Portugal bNational Hospital for Neurology and Neurosurgery, Neurology, London, Comorbidities in Parkinson's disease – The results from national United Kingdom epidemiological study cosmos cFaculty of Medicine, University of Lisbon, Neurology, Lisbon, Portugal dUniversity Hospital of Toulouse, Neurology, Toulouse, France M. Minara, J. Dragasekb, P. Valkovicac eUniversity of Padova, Neurology, Padova, Italy aFaculty of Medicine, Comenius University, Second Department of fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Neurology, Bratislava, Slovakia Portugal bFaculty of Medicine, Pavol Jozef Safarik University, First Department of Psychiatry, Košice, Slovakia c Objective Slovak Academy of Sciences, Institute of Normal and Pathological To evaluate the impact of disease severity on the efficacy of Physiology, Centre of Experimental Medicine, Bratislava, Slovakia opicapone (OPC) in levodopa-treated Parkinson's Disease (PD) patients with motor fluctuations. OPC, a once-daily COMT inhibitor, Background proved effective in the treatment of motor fluctuations in PD patients The burden of comorbidities in Parkinson's disease (PD) is in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. significant. They decrease patients' quality of life and also affect a life expectancy. The aim of our study was to disclose prevalence and Methods impact of comorbidities among patients with PD in our population. Patient-level data from matching treatment arms in BIPARK-I and II were combined in placebo (PLC) and OPC-50 mg groups. The Methods studies had similar designs (primary efficacy endpoint: change from In a multi-center, cross-sectional, observational study COSMOS, baseline in patient diaries-based absolute OFF-time) and eligibility clinicians gathered demographical and clinical data from 737 criteria [1,2]. An exploratory post-hoc analysis was performed to patients diagnosed with idiopathic PD.^ evaluate the influence of disease severity (assessed by modified b ≥ fi Hoehn and Yahr, H&Y^ 2.5 vs 2.5) on ef cacy outcomes (i.e., change Results from baseline in OFF-/ON-time). Applied linear regression's slope From overall participants, 53.50% had at least one psychiatric was statistically tested for deviation from zero and linearity. comorbidity; the most prevalent were depression (26%), sleep disorders (23%), cognitive impairment (13%), anxiety-related disor- Results ders (12%) and apathy (7%). In addition, 93.50% had at least one In total, 1027 patients were randomized to BIPARK-I and II and nonpsychiatric comorbidity; the most prevalent were hypertension 522 patients took a dose of study medication; Full Analysis Set (68%), ischemic heart disease (43%), musculoskeletal disorders comprised 517 [PLC (n = 255); OPC-50 mg (n = 262)]. Two PLC (39%), high blood lipids (33%) and diabetes (20%). The prevalence patients with H&Y = 4 were excluded from disease severity analysis of both psychiatric and nonpsychiatric comorbidities increased with as there were no OPC-50 mg matched-patients. For OPC-50 mg, b PD progression (p ^.001, and p =^.001, respectively). Adequate ARTICLE IN PRESS

214 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx treatment of comorbidities was connected with signiﬁcantly better Patients with PD made signiﬁcantly more errors than healthy – effectiveness of therapy (OR 2.781, 95% CI 1.070 7.229, p =^.036). controls in comprehension of reversible sentences.

Conclusion Conclusion With PD progression, the number of both psychiatric and There are ^multiple communication impairment associated with nonpsychiatric comorbidities is on the increase. If not treated PD even in mild and moderate stage. Problems can be present at properly, they lead to deterioration of response to therapy. That's sentence comprehension, as well in lexical retrieval of words in why is essential to search for any comorbidity to provide patients speech production, and difﬁculties can appear also at speech with early and adequate therapy to avoid further worsening of comprehensibility in everyday communication. quality of life. Research was supported by^grant APVV No.15-0155 doi:10.1016/j.jns.2019.10.1193 doi:10.1016/j.jns.2019.10.1194

WCN19-0495 WCN19-0500

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

Communication impairment in patients with mild to moderate Utilization of monotherapy and combination therapies in ad- Parkinson's disease without dementia vanced Parkinson's disease patients during levodopa-carbidopa intestinal gel treatment from the cosmos study Z. Cséfalvaya, J. Markováa, L. Micianovaa, A. Kusnirovab, Z. Kosutzkab, I. Strakab, M. Hajdukcd, M. Kralovad, P. Valkovicb A. Fasanoa, J.C. Parrab, T. Gurevichc, R. Jechd, N. Kovácse, S. Perf,J. aComenius University, Communication Disorders, Bratislava, Slovakia Szászg, L. Bergmannb, A. Johnsonb, O. Sanchez-Soliñob, Z. Tangb,L. bComenius University, 2nd Department of Neurology, Bratislava, Vela-Desojoh Slovakia aMorton and Gloria Shulman Movement Disorders Clinic, The Edmond J cComenius University, Department of Psychology, Bratislava, Slovakia Safra Program in Parkinson's Disease, University of Toronto, Toronto dComenius University, Department of Psychiatry, Bratislava, Slovakia Western Hospital, Division of Neurology- UHN, Toronto, Canada bAbbVie Inc., Neurology, North Chicago, USA c Background Tel Aviv Sourasky Medical Center, Movement Disorders Unit, Neuro- Parkinson's disease (PD) is viewed as a motor disorder, but there logical Institute, Tel Aviv, Israel d are also a variety of communication impairments associated with Charles University, Department of Neurology, Center of Clinical this disorder. Problems appear even early on in the early stages of Neurosciences, 1st Faculty of Medicine, Prague, Czech Republic ^ e illness. Beside of problems in speech intelligibility, both language University of Pécs, Neurology, Pécs, Hungary f production and comprehension impairments can negatively inﬂu- Karolinska Institutet, Department of Clinical Neuroscience, Stockholm, Sweden ence communication in patients with PD.^ gUniversity of Medicine and Pharmacy Tîrgu Mureș, Emergency Clinical Objective County Hospital Mures, Tîrgu Mureș, Romania h To compare speech comprehensibility, lexical retrieval and Hospital Universitario Fundación Alcorcón, Department of Neurology, Madrid, Spain sentence comprehension in PD patients and healthy controls (HC).^

Subjects and methods Objective ﬁ We enrolled 84 patients with idiopathic PD without dementia^(47 COSMOS (NCT03362879) is the rst study fully dedicated to men, H&Y stage 2.0 ± 0.5 and total MDS-UPDRS part III 31 ± 11,18) collecting real-world data assessing add-on PD medication use with and sex, age and education-matched 82 HC (50 men). Every patient levodopa-carbidopa intestinal gel (LCIG) during long-term therapy in with PD in our study was in the early or starting moderate stage of a large, advanced Parkinson's disease (aPD) patient cohort. The the disease. Speech Intelligibility Test (T-ZROD), Naming Test, and percentages of aPD patients treated with LCIG as a monotherapy or Sentence Comprehension Test were used to assess individuals in with add-on PD medications and the reasons for changes in add-on both groups. PD medications will be assessed.

Results Methods We found signiﬁcant differences in speech comprehensibility Main inclusion criteria were aPD patients treated for at least 12 (intelligibility in context) measured by oral reading semantically ^months (M) by the same physician since LCIG initiation. Patients non-predictable randomly selected sentences were found. were grouped into LCIG monotherapy(LCIG the only treatment for Object, action and total naming performance did not differ across PD), LCIG monotherapy during infusion hours(allowing add-on PD groups, however, there was a signiﬁcantly larger delay in naming of medications after LCIG infusion was stopped), and those on LCIG objects and actions in the PD group.^ +add-on therapy.^ ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 215

Results WCN19-0504 409 LCIG treated aPD patients from 49 clinics in 14 countries were included. Patient demographics/baseline characteristics are Poster shift 03 - autonomic nervous system disorders/history of presented in Table 1. Mean LCIG treatment duration was 35.7 (range:12.0–139.3) months with 9.7% of patients(n = 39) on 24 h/ neurology/motor neuron disease/movement disorders/MS & day LCIG infusion. Preliminary analyses indicate that the percentage demyelinating diseases/neuromuscular disorders/vestibular dis- of patients on LCIG monotherapy and LCIG monotherapy during orders and nutritional disorders or intoxications infusion hours increased from LCIG initiation to M12, while the percentage of patients on LCIG+add-on therapy decreased at M12 Copper deficiency myelopathy together with low zinc levels - The (Table 2). Across groups, a majority of patients discontinued the use impossible combination? of add-on PD medication at LCIG treatment initiation. Safety analyses are ongoing. K. Laua, H.Y. Monb, S. Karc, T. Aungc aDepartment of Neurology, Hull, United Kingdom Conclusions bElderly Care Medicine, Hull Royal Infirmary, Hull, United Kingdom Analyses are ongoing; however, preliminary data indicate that cHull Royal Infirmary, Hull, United Kingdom during routine clinical practice LCIG treatment can be initiated and used long-term in aPD patients with many patients well-controlled In this case report we present a patient who presented with a using LCIG monotherapy. For patients not on monotherapy, LCIG rapid onset gait abnormality and fine action tremors in his hands provides important reductions in add-on PD medications. during a short holiday in South Africa. He also reported weight loss and diarrhea intermittently for three years prior to the presentation. Despite multiple first-line investigations, the cause of his neurolog- doi:10.1016/j.jns.2019.10.1195 ical impairment was not elicited. It was his MRI spine was performed ARTICLE IN PRESS

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

that copper deﬁciency was considered. This patient had a very low WCN19-0527 level with zinc deﬁciency. He was given copper supplements. We will discuss and revisit the causes of posterior column abnormalities, together with the other manifestations of impaired Poster shift 03 - Autonomic nervous system disorders/history of copper metabolism. There is also a section on how abnormal zinc neurology/motor neuron disease/movement disorders/MS & metabolism can lead to neurological abnormalities. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1196 Clinical assessment of motor rehabilitation in patients with multiple sclerosis

WCN19-0511 M. Salokhiddinov, A. Umarov Tashkent Medical Academy, Neurology, Olmazor, Uzbekistan Poster shift 03 - autonomic nervous system disorders/history of Background neurology/motor neuron disease/movement disorders/MS & Rehabilitation in multiple sclerosis is one of the main methods of demyelinating diseases/neuromuscular disorders/vestibular dis- reducing the manifestations of movement disorders, movement orders and nutritional disorders or intoxications coordination disorders, balance, walking, correction of increased muscle tone, pain syndrome Serum CHI3LI and CHI3L2 levels in detecting disease activity and prognosis in multiple sclerosis patients Aim To analyse and assess the use of various rehabilitation methods in N. Taşdemir, B. Değirmenci the complex treatment of patients with MS patients Dicle University, Neurology, Diyarbakır, Turkey Methods Objective The study included 44 patients aged 21–53 years of the main Recent studies examined that chitinase is important in inflam- group with a diagnosis MS in the period of exacerbation with the matory processes and tissue regeneration. In this study, we aimed to leading syndrome of cerebellar ataxia and received drug hormonal compare CHI3L1 and CHI3L2 levels in healthy subjects and patients treatment and classes in specially created stabilizing platforms. with multiple sclerosis during exacerbation and remission periods and whether correlate with disease activity and prognosis and to Results The total duration of exercise is 45–50 min; classes are held 3 contribute to the diagnosis and treatment of MS.^ times a week. Autogenic training and psycho-correctional classes are Material and methods conducted individually. In the main period, such social work Blood sample was taken from control-group and from patients methods are used as club communication, classes in interests, fi holding of all-arched events on holidays. After completion of the diagnosed with de nite^MS, once during the attack period and once at least one month after attack in the remission period. CHI3L1 and rehabilitation course in all patients there was improvement in well- CHI3L2 levels of MS patients measured during attack and remission being in the form of a decrease in feeling dizzy and unsteady when periods were compared with each other and with control-group. The walking. Objective methods for diagnosing the equilibrium state studied parameters were compared with the duration of disease, (test stability of standing, assessment of cerebellar and pyramidal attack frequency, sex, family history, Expanded Disability Status Scale functional systems on the disability scale on Kurtzke) and computer (EDSS), the applied treatment and Magnetic Resonance Imaging stabilometry data significantly confirmed the improvement in patient resistance (MRI) lesion load.^

Results Conclusion CHI3L1 levels were signiﬁcantly higher in patients with MS attack The study showed positive results on the application of methods than the control group and MS patients in the remission period. motor rehabilitation during the exacerbation of MS on background of drug and hormonal therapy. Developing novel and more effective ^CHI3L2 levels were lower in patients with MS attack and MS rehabilitation approaches, therefore, requires an improved under- remission than the control group.^ standing of brain plasticity that can be exploited in recovery Conclusion interventions. MS, a very important immune-based systemic disease, was thought to involve the CHI3L1/CHI3L2 axis in the pathogenesis, but other mechanisms were also thought to contribute to the disease pathogenesis. doi:10.1016/j.jns.2019.10.1198 doi:10.1016/j.jns.2019.10.1197 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 217

WCN19-0528 I. Strakaa, M. Minára,M.Škorvánekb, K. Danterovác, M. Groﬁkd,J. Benetinc, P. Valkovičae aComenius University Faculty of Medicine, 2nd Department of Neurol- Poster shift 03 - Autonomic nervous system disorders/history of ogy, Bratislava, Slovak Republic neurology/motor neuron disease/movement disorders/MS & bP.J. Safarik University Faculty of Medicine, Department of Neurology, demyelinating diseases/neuromuscular disorders/vestibular dis- Košice, Slovak Republic orders and nutritional disorders or intoxications cSlovak Medical University, Department of Neurology, Bratislava, Slovak Republic d Short-term central adaptation in benign positional paroxysmal Comenius University Jessenius Faculty of Medicine, Department of vertigo Neurology, Martin, Slovak Republic eSlovak Academy of Sciences, Center of Experimental Medicine, Institute S.Y. Choia, C. Kwang-Dongb of Normal and Pathological Physiology, Bratislava, Slovak Republic aPusan National University Hospital, Department of Neurology, Busan, Republic of Korea Introduction bPusan National University, Department of Neurology, Busan, Republic Non-adherence to pharmacotherapy in Parkinson’s disease (PD) of Korea is associated with worsen clinical state and poor quality of life (QoL). Majority of PD patients takes three daily doses of dopaminergic Objective drugs in minimum. Therefore, it is very important to know predictors To elucidate the cause, associated factors and clinical implications of low level of adherence to pharmacotherapy to PD drugs. of the spontaneous reversed nystagmus (SRN) in benign paroxysmal positional vertigo (BPPV), we analyzed the nystagmus in posterior Methods canal (PC) and horizontal canal (HC) BPPV. We included 124 cognitively intact patients (72 men) with idiopathic PD, taking at least three daily doses of PD drugs. We used Methods validated Slovak versions of questionnaires and scales: Morisky 8- ’ From 2017 to 2018, 182 consecutive patients with PC (n = 119) Item Medication Adherence Scale (MMAS-8), Parkinson s Disease and HC (n = 63) BPPV (men = 64, mean age ± SD = 63.9 ± 13.9) Questionnaire-8 (PDQ-8), Geriatric Depression Scale (GDS), Non- were prospectively recruited. The SRN was deﬁned as the following Motor Symptom Assessment Scale for PD (NMSS), 9-Item Wearing- direction-changing nystagmus after the initial positional nystagmus off Questionnaire (WOQ-9), and MDS-UPDRS III (motor examina- without change of a head position during Dix-Hallpike or head-roll test. tion) and IV (motor complications).

Results Results The SRN was observed in 56 (56/119, 47.1%) in PC and 43 (43/63, In our cohort of PD patients, 33.9% reported high level, 29.8% 68.3%) in HC BPPV. The maxSPV of initial positional nystagmus in PC- reported medium level, and 36.3% reported low level of adherence. BPPV with SRN was greater than that without SRN (56.2 ± 39.3°/sec The rate of adherence correlated with disease duration, and total ﬁ vs. 40.2 ± 34.4°/sec). The duration and Tc of nystagmus of PC-BPPV scores of PDQ-8, NMSS, WOQ-9 and MDS-UPDRS IV. We identi ed with SRN were shorter than these without SRN (p = 0.016 and that only NMSS predicted low level of adherence. From detailed 0.034). In HC-BPPV, the maxSPV of ipsilesional geotropic nystagmus analysis of NMSS domains and questions in multivariate regression was also greater on the group with SRN than those without SRN analysis, we found out that excessive daytime sleepiness (p = (114.3 ± 56.8°/sec vs. 59.3 ± 41.8°/sec, p = 0.003). The estimated 0.029), anhedonia (p = 0.002), and forgetfulness (p = 0.005) were 2 cutoff value of maxSPV for spontaneous reversal is 85.5°/sec in PC predictors for low level of adherence (R = 0.602, R = 0.363). BPPV and 56.7°/sec in HC BPPV. The SRN did not affect to the treatment outcomes in PC- and HC-BPPV. Conclusion In clinical practice, it is very important to identify non-adherent Conclusion patients and provide suitable interventions for improving the rate of The SRN is common in both PC- and HC-BPPV. The velocity adherence and, conclusively, for improvement of clinical state and QoL. storage system may contribute these short-term adaptive responses This research was supported by VEGA 1/0490/16, VEGA 1/0704/17, with telescopic strategy to the acute abnormal vestibular asymmetry. and grant of Ministry of Health of the Slovak Republic 2018/32-LFUK-6. doi:10.1016/j.jns.2019.10.1200 doi:10.1016/j.jns.2019.10.1199

WCN19-0534 WCN19-0529 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 Noisy galvanic vestibular stimulation improves body balance in Predictors of the low level of adherence to pharmacotherapy in healthy adults and patients with bilateral vestibulopathy patients with Parkinson’s disease taking three or more doses of dopaminergic medications C. Fujimotoab, M. Kinoshitab, M. Yagia, T. Yamasobab, S. Iwasakib aTokyo Teishin Hospital, Department of Otolaryngology, Chiyoda-ku, Tokyo, Japan ARTICLE IN PRESS

218 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx bGraduate School of Medicine- The University of Tokyo, Department of WCN19-0572 Otolaryngology and Head and Neck Surgery, Bunkyo-ku, Tokyo, Japan

Poster shift 03 - Autonomic nervous system disorders/history of Noisy galvanic vestibular stimulation (nGVS) is a procedure that applies electrical current as zero-mean current noise to the vestibular neurology/motor neuron disease/movement disorders/MS & system through electrodes placed over the bilateral mastoids. An demyelinating diseases/neuromuscular disorders/vestibular dis- optimal level of nGVS facilitates the processing of subthreshold stimuli orders and nutritional disorders or intoxications in neural systems, such as the autonomic, motor or postural control systems. As for the postural control system, stability in standing Neuroprotective and neurotrophic efficacy of Ginkgo biloba posture and gait performance were improved during the application of extracts against 3-nitropropionic acid-induced oxidative stress imperceptible nGVS level in healthy subjects and in patients with in a rat model of Huntington's disease bilateral vestibulopathy (BV). The proposed mechanism behind these ameliorating effects is stochastic resonance, in which the existence of A. Kumar, S. Farhar an optimal amount of noise can enhance subthreshold signals in a A Central University JMI, Biosciences, Delhi, India non-linear system. We recently found that a 30-min application of nGVS led to a post-stimulation ameliorating effect of the postural Huntington's disease (HD) is a progressive neurodegenerative stability that lasts for several hours in healthy elderly adults and BV disorder. Ginkgo biloba extracts (GBE), a phytoestrogen, has been patients, even after the cessation of the stimulus. nGVS is a promising widely used for treating coronary heart disease and neurological candidate for a novel treatment of refractory postural instability due to disease. The present study examined the potential neuroprotective vestibulopathy. Further studies are needed to increase the evidence and neurotrophic efficacy of GBE against 3-nitropropionic acid (3- level of the therapeutic effects of nGVS. NP)-induced oxidative stress in a rat model of HD and explored the mechanisms of action. doi:10.1016/j.jns.2019.10.1201 60 Male SD rats were pretreated with GBE (25 and 50 mg/kg b.w.) orally prior to the intraperitoneally (i.p.) administration of 3-NP (12 mg/kg b.w.) for 15 days. Nimodipine (12 mg/kg, po) was used as positive control drugs. The body weight, grip strength and behavior WCN19-0560 were monitored within 5th, 10th and 15th day after 3-NP treatment. Then the animals were sacrificed, neuronal damage in striatum was Poster shift 03 - Autonomic nervous system disorders/history of estimated using Nissl staining. Memory (Morris water maze), antioxidants enzymes, acetylcholinesterase (AChE) activity, neurology/motor neuron disease/movement disorders/MS & cycloxygenase-2 (COX-2) and inducible nitric oxide synthase (iNOS) demyelinating diseases/neuromuscular disorders/vestibular dis- expression were analyzed in rat model of HD. orders and nutritional disorders or intoxications Present results shown that administration of 3-NP resulted in a marked reduction in the body weight, memory, grip strength Cadmium activates the noncanconical AKT –GSK3Β signaling to locomotion activity and significantly increased oxidative stress, AChE impair the dopaminergic dysfunctions in rats activity, COX-2 and inducible iNOS expression. GBE (25 and 50 mg/ kg) treated animals exhibited a significant improvement in behav- R. Guptaa, R.S. Drb, V.K.K. Dra ioural, biochemical, histological alterations and oxidative stress aCSIR – Indian Institute of Toxicology Research, Developmental parameters in comparison to only 3-NP treated animals. Present Toxicology Laboratory, Lucknow, India results shown dose-dependently improved 3-NP-induced behavioral, bAIIMS, Department of Biochemistry, Bhopal, India biochemical, and enzymatic changes. Similar effects were obtained with the positive control drugs nimodipine. Cadmium, a neurotoxic heavy metal has been reported to cause Study suggests neuroprotective, neurotrophic and memory Parkinson’s and motor dysfunctions, yet the mechanism how Cd enhancing effects for GBE in a rat model of HD. causes the motor dysfunction remains elusive. The Study has been carried out to identify the mechanism involved in Cd induced doi:10.1016/j.jns.2019.10.1203 dopaminergic alterations. A four weeks treatment was given to Rats with cadmium (5.0 mg/kg, bw, p.o, 28 days) and quercetin (25.0 mg/kg, bw, p.o., 28 days) alone and simultaneously. Western blotting and IHC studies suggest that treatment of rats to cadmium WCN19-0588 causes the Selective decrease in the non canonical signaling molecules β Arrestin, AKt and GSK 3β. The statistical analysis Poster shift 03 - Autonomic nervous system disorders/history of suggests that cadmium exposure down regulates the dopamine D2 receptors and TH levels. The altered level of CREB was of to the neurology/motor neuron disease/movement disorders/MS & down regulation of GSK3b on cadmium treatment. Besides of the demyelinating diseases/neuromuscular disorders/vestibular dis- classical signaling. The altered motor activity and motor coordina- orders and nutritional disorders or intoxications tion on cadmium treatment was due to activation of non canonical signaling. Synchronous facial diplegia due to Herpes Simplex Virus 1 The Results of the present study potentially revealed that reactivation following Diabetic Ketoacidosis Noncanconical AKt –GSK3β Signaling is responsible for cadmium induced motor dysfunction and it also provide an quercetin might be S. Farooq, M. Khan, H. Kamalboor, S. Al Rukn a potential candidate drug for the novel therapeutic intervention Rashid Hospital, Neurology, Dubai, United Arab Emirates such dysfunctions. We describe a 35 year old lady who presented with a one day doi:10.1016/j.jns.2019.10.1202 history of difficulty in opening her mouth and an inability to close her eyes. There was an associated impairment in taste sensation. Her ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 219 past history was significant for a recent diagnosis of Type 1 Diabetes traumatic dizziness (3.1%), vestibular paroxysmia (2.7%), stroke Mellitus (DM 1) discovered when she developed Diabetic (2.2%), drug-induced (1.3%), multiple sclerosis (0.45%) and other Ketoacidosis (DKA). She was managed for this at another hospital disorders (3.1%). In 4.5% of patients no cause could be established. and was discharged home on insulin just five days prior to her neurological presentation. Conclusion Her neurological examination was significant only for a bilateral Vestibular migraine is one of the most common cause of dizziness facial weakness, which was of lower motor neuron type and Herpes and vertigo. This is probably the largest study of Vertigo patients in Simplex Virus 1 (HSV-1) IgM antibodies were positive. Blood sugar Dubai population. The etiological spectrum of vertigo seen in this was well controlled on insulin. study is similar to the spectrum reported from other countries. Bilateral facial palsy or facial diplegia is a rare clinical entity and synchronous involvement (involvement of the contralateral side doi:10.1016/j.jns.2019.10.1205 within 30 days) is even rarer. Commonly implicated etiologies are infectious and immune mediated. While there is a single reported case of unilateral facial palsy in a known diabetic patient presenting with DKA, we report the first case of synchronous bilateral facial WCN19-0595 palsy in a patient with newly diagnosed DM 1 presenting with DKA. Our patient had serum HSV-1 IgM positive suggesting a recent infection with the virus. Poster shift 03 - Autonomic nervous system disorders/history of Ours is a rare case of synchronous bilateral facial palsy in a neurology/motor neuron disease/movement disorders/MS & patient with newly diagnosed DM 1 presenting with DKA. Our demyelinating diseases/neuromuscular disorders/vestibular dis- proposed theory for pathogenesis is a HSV-1 reactivation in an orders and nutritional disorders or intoxications immunocompromised state of DKA. AQP4-IGG plays a potential role in retinal microstructure damage doi:10.1016/j.jns.2019.10.1204 OF NMOSD

Z. Wang, Q. Wang, Q. Guo, X. Qi The Sixth Medical Center of Chinese PLA General Hospital, Neurology, WCN19-0590 Beijing, China

Poster shift 03 - Autonomic nervous system disorders/history of Objective neurology/motor neuron disease/movement disorders/MS & To investigate the role of aquaporin 4 antibody (AQP4-IgG) in retinal microstructure damage of NMOSD. demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Methods Forty patients with neuromyelitis optica spectrum disorders Etiological spectrum of vertigo and dizziness: Clinic-based study (NMOSD), each having at least one episode of optic neuritis (ON), from a specialty medical center in Dubai were retrospectively studied. All NMOSD patients were subdivided into two subgroups based on AQP4-IgG status: 25 NMOSD patients V. Pawar (37 ON eyes) with AQP4-IgG seropositive (Ab+/NMOSD group), 15 Aster DM Healthcare, Neurology, Dubai, United Arab Emirates NMOSD patients (22 ON eyes) with AQP4-IgG seronegative (Ab-/ NMOSD group). In addition, 10 health controls (20 healthy eyes) Introduction and objective matched for age and sex (HC group) were analyzed. Spectral domain Dizziness is a common complaint in neurological outpatients. optical coherence tomography (SD-OCT) was used to quantify Vestibular disorders are the most common cause of dizziness. There peripapillary retinal nerve fiber layer (RNFL). are few studies quantifying the frequency of vestibular disorders in Dubai population. The aim of this study is to report the etiological Results spectrum of vertigo and dizziness from a vertigo specialty clinic in Median visual acuity in ON eyes of Ab+/NMOSD was worse than Dubai. Ab-/NMOSD (P=0.02). There were no significant differences between Ab+/NMOSD and Ab-/NMOSD in aspects of age, sex, disease Material and methods duration, ON episodes, longitudinal extensive transverse myelitis The study was conducted at Vertigo clinic, equipped with Video- (LETM) ratio, NMOSD specific intracranial lesions ratio, positive oculography (VOG). All the consecutive patients suffering from dizziness autoimmune antibody ratio (all P b 0.05). The thickness of temporal, visiting the Vertigo clinic in the last three months were included in the superior, nasal, inferior and average RNFL in ON eyes of NMOSD were study. Detailed history taking and neurotological examination was thinner than eyes in HC group (P b 0.05). The thickness of superior performed in every patient. VOG, Neuroimaging studies and other and inferior RNFL in ON eyes of Ab+/NMOSD were thinner than Ab-/ investigations were done when it was felt appropriate. NMOSD after adjusting for confounding factors (P b 0.05).

Results Conclusions During the 3-month study period, 222 patients [141 males and 81 RNFL can be used to evaluate injury of retinal microstructure of females, mean age 38 years, range 19–83 years] were enrolled in the NMOSD patients. AQP4-IgG seropositive NMOSD suffers severer study. The most frequent (Fig.1) causes were vestibular migraine damage of RNFL implying the potential role of AQP4-IgG in this (43.2%)- [deﬁnite:44.8% probable:55.2%] and BPPV (19.8%). This was pathological process. followed by Meniere’s disease (7.2%) and persistent-postural-phobic dizziness (4.9% alone and 6.7% co-morbid). Other causes included: doi:10.1016/j.jns.2019.10.1206 psychiatric causes (4%), acute unilateral vestibulopathy (3.1%), post- ARTICLE IN PRESS

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

WCN19-0596 WCN19-0597

Switching entacapone ‘non-responders’ to open-label opicapone: Switching from double-blind entacapone or placebo to open- Change in absolute off-time following the 1-year extension label opicapone: change in OFF-/ON-time from patients who BIPARK-I study ended the 1-year BIPARK-I extension on opicapone 50 mg

J. Ferreiraa, A. Leesb, W. Poewec, O. Rascold, A. Santose, D. Magalhaesf, J. Ferreiraa, A. Leesb, O. Rascolc, W. Poewed, F. Ikedoe, D. Magalhaesf,F. F. Rochaf, P. Soares-da-Silvae Rochaf, P. Soares-da-Silvae aFaculty of Medicine- University of Lisbon, Neurology, Lisbon, Portugal aFaculty of Medicine- University of Lisbon, Neurology, Lisbon, Portugal bNational Hospital for Neurology and Neurosurgery, Neurology, London, bNational Hospital for Neurology and Neurosurgery, Neurology, London, United Kingdom United Kingdom cMedical University of Innsbruck, Neurology, Innsbruck, Austria cUniversity Hospital of Toulouse, Neurology, Toulouse, France dUniversity Hospital of Toulouse, Neurology, Toulouse, France dMedical University of Innsbruck, Neurology, Innsbruck, Austria eBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, eBIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Portugal Portugal fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, fBIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, Portugal Portugal

Objective Objective To evaluate the efficacy of opicapone (OPC) in levodopa-treated To evaluate the efficacy of opicapone (OPC) in levodopa-treated Parkinson’s Disease (PD) ‘non-responders’ patients who switched Parkinson’s Disease (PD) patients who switched from placebo (PLC) from placebo (PLC) or entacapone (ENT) to OPC in BIPARK-I open- or entacapone (ENT) to OPC and ended the 1-year extension on OPC- label part. OPC, a once-daily COMT inhibitor, proved effective in the 50mg. OPC, a once-daily COMT inhibitor, proved effective in the treatment of motor fluctuations in PD patients in two large, pivotal, treatment of motor fluctuations in PD patients in two large, pivotal, multinational trials (BIPARK-I and II) [1,2]. multinational trials (BIPARK-I and II) [1,2].

Methods Methods After completing BIPARK-I double-blind part, PLC- and ENT-patients After completing BIPARK-I double-blind part, PLC- and ENT- switched to a 1-year open-label extension under OPC-treatment. This patients switched to a 1-year open-label extension on OPC- post-hoc analysis investigated the change from open-label baseline in treatment. This post-hoc analysis investigated the change from absolute OFF-time in ‘non-responders’ PLC and ENT ‘switchers’.Non- open-label baseline in absolute OFF-/ON-time in PLC and ENT responders, as assessed by Patient Global Impression of Change (PGI-C), ‘switchers’ that ended the 1-year extension on OPC-50 mg. A linear were any subjects who had no improvement by double-blind endpoint. mixed-effect model repeated measurement (MMRM) with region as A linear mixed-effect model repeated measurement (MMRM) with factor and baseline as covariate was applied. region as factor and baseline as covariate was applied. Results Results In total, 199 patients switched from PLC (n = 99) or ENT (n = In total, 199 patients switched from PLC (n = 99) or ENT (n = 100) to 1-year OPC open-label extension. After 1-year treatment 100) to 1-year OPC open-label extension. From these, ~45% were with OPC, the overall mean OFF-time was decreased by -33.8 min PGI-C ‘non-responders’ for both groups. After 1-year treatment with relative to open-label baseline. For subjects who received PLC or ENT OPC, for PLC-patients defined as ‘non-responders’ at double-blind in the double-blind period and ended the 1-year extension taking endpoint, switching to OPC resulted in a statistical significant OPC-50mg, the switch to OPC resulted in a statistically significant additional reduction of OFF-time (−83.8 min, p = 0.0003). Likewise, reduction of OFF-time (−54.9 min, p = 0.0081 and −68.2 min, p = switching ENT ‘non-responders’ to open-label OPC resulted in a 0.0025) and increase of ON-time (+71.7 min, p = 0.0007 and +53.3 statistical significant additional reduction of OFF-time (−45.3 min, min, p = 0.0195). Patients originally allocated to OPC-50 mg in the p = 0.0399). ‘Non-responders’ patients (~23%) originally allocated to double-blind phase showed mainly maintenance of treatment effect. OPC-50mg in the double-blind phase also showed greater response but not statistically significant. Conclusion Patients switching from entacapone to opicapone and ended the Conclusion 1-year extension taking opicapone 50 mg, achieved a significant Switching entacapone ‘non-responders’ to open-label opicapone incremental benefit in reduced OFF-time and increased ON-time. resulted in a statistical significant reduction of OFF-time. References References [1] J.J. Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [1] J.J. Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [2] A.J. Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. [2] A.J. Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. doi:10.1016/j.jns.2019.10.1207 doi:10.1016/j.jns.2019.10.1208 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 221

WCN19-0599 doi:10.1016/j.jns.2019.10.1209

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-0601 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Poster shift 03 - Autonomic nervous system disorders/history of Opicapone as a levodopa sparing agent: Pooled analysis of neurology/motor neuron disease/movement disorders/MS & BIPARK-I and II double-blind trials demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications R. Hausera, O. Rascolb, W. Poewec, J. Ferreirad, A. Leese,O. Klepitskayaf, G. Liangf, A. Santosg, D. Magalhaesh, F. Rochah,P. Detection of duplication of PMP22 gene by ﬂuorescent in situ Soares-da-Silvag hybridization (ﬁsh) in Charcot Marie tooth type 1 in public aUniversity of South Florida, Neurology, Tampa, USA hospital of south of Chile bUniversity Hospital of Toulouse, Neurology, Toulouse, France c Medical University of Innsbruck, Neurology, Innsbruck, Austria M. Fuentealbaa, C. Veasb, C. Jarab, F. Condezab, A. Gonzalezc d Faculty of Medicine- University of Lisbon, Neurology, Lisbon, Portugal aUniversity of Concepcion, Specialties, Concepcion, Chile e National Hospital for Neurology and Neurosurgery, Neurology, London, bUniversity of Concepcion, Specialties, Concepción, Chile United Kingdom cUniversity of Concepcion, Public Health, Concepción, Chile fNeurocrine Biosciences- Inc, Neurosciences, San Diego, USA g BIAL - Portela & Co SA, Research & Development, S. Mamede Coronado, Introduction Portugal The genotypic diagnosis in CMT is a public health need. In our h BIAL - Portela & Co SA, Global Parkinson Disease, S. Mamede Coronado, country, a genetic study is carried out in a single laboratory (INTA) Portugal with restricting of access to public patients.

Objective Objective Evaluate, in levodopa-treated Parkinson’s Disease (PD) patients, To determine presence of duplication of the PMP22 gene in the ability of opicapone (OPC) to act as levodopa-sparing agent. OPC, patients with CMT1 by FISH of the Neurology Unit of the Hospital a once-daily COMT inhibitor, proved effective in the treatment of Regional de Concepcion. CMT1 it is produced by a duplication of motor fluctuations in PD patients in two large, pivotal, multinational 1.5mb on chromosome 17p11.2detectable by FISH and correlated trials (BIPARK-I and II) [1,2]. with "classic" phenotype (onset development of symptoms in the first 2 decades, slow and progressive course, characteristic orthope- Methods dically alterations and decrease in the speed of nerve conduction of Patient-level double-blind data from BIPARKs were combined for arms OPC-50mg. The studies had similar designs (primary efficacy endpoint: change from baseline in patient diaries-based absolute Method OFF-time) and eligibility criteria [1,2]. An exploratory post-hoc 16 patients are included M38% F62% with average age 38(16–63), analysis was performed to evaluate motor response (OFF- and ON- clinically grouped i 4 CLASSIC PENOTHYPE OF CMT1, 7 other time change and UPDRS III) and quality of life (assessed by PDQ-39) HEREDITARY NEUROPATHIES and 5 DEMYELINATING NEUROPA- in levodopa-treated PD patients that reduced levodopa. THIES ACQUIRED

Results From the 517 Full Analysis Set patients, 262 were randomized to OPC-50mg and 41 (16%) had a daily levodopa reduction during the double-blind adjustment period. Mean levodopa dose at baseline was ~840 mg and patients presented ~6.5 h of mean OFF-time. By the end of adjustment period, levodopa was reduced, on average, by ~23% (~200mg) either due to dopaminergic adverse-events (n=30) or proactively (n=11, 27%). Despite dose reduction, levodopa- reduced patients had an absolute mean OFF-time decrease and ON- time increase of N2 h (-131 min and + 125min, respectively) by the end of double-blind period. Additionally, UPDRS parts II and III and PDQ-39 improved from baseline by -2, -3 and -3 points, respectively.

Conclusion Findings suggest that opicapone could act as levodopa-sparing agent and still improve both quality of life and motor response of levodopa-treated PD patients.

References

[1] J.J. Ferreira, et al., Lancet Neurol. 15 (2) (2016) 154–165. [2] A.J. Lees, et al., JAMA Neurol. 74 (2) (2017) 197–206. ARTICLE IN PRESS

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

In FISH assay, presence of more than 40% nuclei with 3 signals cSamsung Medical Center, Department of Plastic Surgery, Seoul, Republic of the PMP22 gene and 2 corresponding to the centromere of of Korea chromosome 17 was considered positive. We found 4 positive cases, 3 in CMT1 group and 1 in OTHER Tumors of the peripheral nerves are composed of neurogenic HEREDITARY NEUROPATHIES (case of Hereditary neuropathy with tumors and non-neurogenic tumors. Neurogenic tumors include the Liability to pressure palsies and none in DEMYELINATING NEUROP- schwannoma, the Tumors of the peripheral nerves are composed of ATHIES ACQUIRED neurogenic tumors and non-neurogenic tumors. There were few Three counter samples (1Positive/2Negative) were analyzed in reports of focal mono-neuropathy caused by peri- or intra-neural INTA using MLPA and FISH having concordance in everyone case. leiomyoma. In this case, we report a case of radial neuropathy caused by intra-neural leiomyoma in 37-year-old male who experienced Conclusion sudden onset of weakness in his right upper extremity. Intraneural Technique of FISH is reliable in the detection of the duplication leiomyomas are very rare, but can develop in peripheral nerves. responsible for CMT1 and increasing the number of cases it is feasible Physicians should include intraneural leiomyoma as a possible to validate the method in our place and implement this genetic diagnosis in a case of a well-demarcated intraneural mass causing diagnosis in our public care system. focal mono-neuropathy. MR imaging can help differentiate doi:10.1016/j.jns.2019.10.1210 intraneural leiomyoma from other benign PNSTs in its homogeneous internal signal intensity, absence of split fat sign or target sign, and presence of hypo-intensity rim around the mass in T1-weighted and T2 weighted image.

WCN19-0604 doi:10.1016/j.jns.2019.10.1211

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- WCN19-0611 orders and nutritional disorders or intoxications Poster shift 03 - Autonomic nervous system disorders/history of Radial neuropathy caused by intraneural leiomyoma: A case neurology/motor neuron disease/movement disorders/MS & report demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications B. Leea, Y. Choib, B. Jeonc, D. Sunga aSamsung Medical Center, Department of Physical Medicine And Neurological immune-related adverse events treated with im- Rehabilitation, Seoul, Republic of Korea mune check point inhibitors: A single center experience bSamsung Medical Center, Department of Pathology and Translational Genomics, Seoul, Republic of Korea ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 223

S. Kamada, A. Hanazono, Y. Sanpei, M. Sugawara, K. Iijima been found in N10% patients with sporadic ALS. Whether mutations Akita University School of Medicine, Department of Neurology, Akita, affect dysphagia remains unknown. The aim of this study was to Japan compare clinical features and progression of dysphagia in limb-onset ALS (N = 10) between patients with genetic mutations (N = 5, 2 Background patients, p62/SQSTM1; 1, VCP; 1, SOD1; and 1, FUS) and those without Immune checkpoint inhibitors (ICIs), anti-CTLA4 and anti-PD1 mutations (N = 5), using videofluoroscopic examination (VF). have dramatically changed the treatment landscape for patients with Similarly, we performed VF in bulbar-onset ALS (N = 7) with cancer. ICIs target T-cell regulatory pathways to enhance antitumor ATXN8OS mutations (N = 2) and without mutations (N = 5). VF CD8 T-cell immunity. On the other hand, ICIs are known to have evaluation was based on the scale established by the Japanese distinctive adverse events, named as "immune-related AEs (irAEs)". Society of Dysphagia Rehabilitation, which can separately evaluate As ICI use becomes more common, neurologists would receive more oral (Or) and pharyngeal (Ph) phases in addition to total scores (To) consultations about neurological irAEs. Myasthenia gravis (MG) is a and on internationally used scales, Dysphagia Outcome Severity relatively well-known neurological immune-related AE, but other Scale and Penetration-Aspiration Scale. Physical conditions were neurological irAEs are known, for example, myopathy, encephalitis, evaluated with the revised ALS-functional rating scale (FRS). We also and polyneuropathy. Our aim is to investigate the incidence and calculated their change scores (c) and scores/disease duration. In clinical manifestations of neurological irAEs at our institution. limb-onset ALS, the change score of Ph (cPh)/year, cTo/year, and cPh/ cFRS were significantly lower in patients with genetic mutations (p b Methods 0.05, Mann-Whitney U test), suggesting that patients with mutations We examined the medical records of all patients treated with ICIs had significantly slower progression and milder dysphagia than at Hospital of Akita University School of Medicine between January those without mutation. In bulbar-onset ALS, patients with ATXN8OS 2015 to April 2019 and performed retrospective review. History, mutations exhibited various progression and severity of dysphagia. cancer type and stage, clinical presentation, neurological examina- Despite the small number of patients enrolled, and different mutated tion, therapeutic course of irAEs, prognosis of cancer were extracted. genes analyzed together, our results may suggest that severity and progression of dysphagia is associated with gene mutations. Results We identified 204 patients treated with ICIs (nivolumab, doi:10.1016/j.jns.2019.10.1213 pembrolizumab, or ipilimumab). 2.5 percent (5/204) developed Neurological irAEs. These include generalized MG with myopathy in 2 patients, ocular MG in 1 patient, generalized myopathy in 1 patient, and rhabdomyolysis in 1 patient. One generalized MG WCN19-0626 patient and generalized myopathy patient were successfully treated with corticosteroid. The other generalized MG patient and ocular MG Poster shift 03 - Autonomic nervous system disorders/history of patient were cured without steroid, only through the discontinuation of immune checkpoint inhibitor. neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Conclusion orders and nutritional disorders or intoxications Prompt diagnosis and appropriate treatment of neurological irAEs will contribute to improving management of patients with advanced Rare and serious types of central nervous system demeylinating cancer. diseases and MS types doi:10.1016/j.jns.2019.10.1212 B. Rajendran, G. Ram Kauvery Hospital, Chennai, India

Multiple sclerosis (MS) is an inﬂammatory condition causing WCN19-0624 demyelination and axonal loss in the central nervous system (brain and spinal cord). The primary brain insult (“sclerosis”) and its “ ” Poster shift 03 - Autonomic nervous system disorders / history of clinical features are dissemination in space , affecting different anatomical sites, and “dissemination in time”, appearing episodically neurology /motor neuron disease / movement disorders / MS & over time. demyelinating diseases / neuromuscular disorders / vestibular The common types of MS includes disorders and nutritional disorders or intoxications - Relapsing and remitting type (RRMS) Clinical features and progression of dysphagia in bulbar-onset or - Secondary progressive MS (SPMS) limb-onset amyotrophic lateral sclerosis between patients with - Primary progressive MS ( PPMS) genetic mutations and those without mutations The sub-division is based more on clinical characteristics and ﬁ C. Isonoa, M. Hiranob, K. Fukudaa, M. Samukawab, K. Saigohb,Y. not speci c biological pathophysiology. Nonetheless, they Nakamurac, S. Kusunokib provide an organized framework for diagnosis and long-term aKindai University Faculty of Medicine, Rehabilitation Medicine, Osaka- management. sayama, Japan Multiple Sclerosis was initially thought to be a rare condition in bKindai University Faculty of Medicine, Neurology, Osaka-sayama, Japan countries close to the equator such as India. However that has cSakai City Medical Center, Neurology, Sakai, Japan changed now and we do see cases of Multiple Sclerosis in this part of the world. And occasionally we do also come across patients with evidence Amyotrophic lateral sclerosis (ALS) exhibits dysphagia associated of demyelination in the CNS which is resistant to treatment and has with intractable pneumonia or malnutrition. Genetic mutations have ARTICLE IN PRESS

224 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx a rapidly progressive course which even leads to death. These rare PC in our patient suggest a possibility of partial APV selectively types of MS includes Marburg variety and tumefactive MS. affecting the utricle and PC. We have three such patients who presented with rapidly progressive neurological deﬁcits and showed such a rampant course, doi:10.1016/j.jns.2019.10.1215 in spite of various anti-inﬂammatory agents used including Rituximab. This presentation is to highlight the fact that demyelinating disease of the CNS could be quite dangerous from the onset and rare WCN19-0641 types of MS should be considered early in such cases.

Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1214 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-0635 Is CIDP underdiagnosed among DM patients? A study of CIDP cases in a metropolitan hospital in Penang, Malaysia Poster shift 03 - Autonomic nervous system disorders/history of a b c d e neurology/motor neuron disease/movement disorders/MS & G.B. Eow , K.J. Goh , L.S. KoK , J. Naper , C.K. Ong aNeurology Department, Penang, Malaysia demyelinating diseases/neuromuscular disorders/vestibular dis- bUniversity Malaya Medical Centre- UMMC- Malaysia, Neurology, orders and nutritional disorders or intoxications Petaling Jaya, Malaysia cGleanegles Medical Centre- Penang, Nephrology, Georgetown, Malaysia Vertical diplopia as a main manifestation of acute peripheral dUniversity College Dublin- Bellﬁeld- Dublin, Medical, Bellﬁeld, Ireland vestibulopathy eGleanegles Medical Centre, Medical, Georgetown, Malaysia

a b c d S. Lee , K. Choi , S.H. Kim , I.S. Moon Background a Pusan National University Hospital, Neurology, Busan, Republic of Diabetes Mellitus (DM) has a higher incidence of CIDP than the Korea general population. The cases are under reported. Unlike diabetic b Pusan National University Hospital, Neurology, Seo-gu, Republic of polyneuropathy, DM-CIDP is treatable with intravenous immuno- Korea globulin (IVIG), corticosteroids and plasma exchange. cDong-A University Hospital, Neurology, Busan, Republic of Korea d Daedong Hospital, Neurology, Busan, Republic of Korea Objectives To study the demography of CIDP patients and the subgroup Background population of CIDP with DM and their response to IVIG The diagnosis of acute peripheral vestibulopathy (APV) is occasionally challenging due to atypical manifestations particularly Methods in APV patients sparing horizontal canal function. We report a This is a cross-sectional retrospective study from patient of patient presenting with acute vertical diplopia as a main manifesta- neurology clinics in Penang Hospital. We include patients with tion of APV. diagnosis of CIDP based on INCAT 2001 criteria. The subset of CIDP with DM and their response to IVIG was Case report further analysed based on subjective and objective improvement in A 63-year-old female presented vertical diplopia and mild mean change in MRC sum score and nerve conduction study imbalance for 3 days. There was no spontaneous nystagmus or head parameters. Clinical and neurophysiological evaluations were pref- tilt. Bedside horizontal HITs were normal. She veered to the left ormed before and after IVIG therapy. while tandem walking. Hess screen tests showed a skew deviation with hypertropic left eye, and fundus photography revealed Results extorsion (20°) of the right eye and intorsion (2°) of the left eye. 80 patients have CIDP. 56.3% of patients were male (n=45), while The patient had rightward subjective visual vertical (SVV) tilt with 43.7 % were female (n=35). 38.8 % were Chinese (n=31), 37.5% binocular viewing (10.0°). Video HITs disclosed decreased were Malay (n=30), 23.8% were Indian (n=19). Age range – 16-81. vestibulo-ocular reflex gain with corrective catch-up saccades of The mean age was 52.49 ±15.46 SD, Among the patients with CIDP, the right posterior canal (PC), while bithermal caloric tests and air- 45% had DM (n=36), 38.8% were treated with IVIG (n=31). Among conducted cervical VEMPs tests were symmetrical. Brain MRIs the patients treated with IVIg, 14 has DM. 14 out of the 36 of DM including diffusion-weighted images with 3mm thin section, with CIDP were given IVIg and improved. which were performed at 3 and 10 days after the onset of symptoms, were unremarkable. The symptoms rapidly resolved 3 Conclusion days later, and video HITs, ocular torsion, and SVV tilt returned to Our study highlights the importance of identifying treatment normal. responsive CIDP with underlying DM neuropathy. DM-CIDP patients have excellent response to immunosuppressive therapy. Conclusion The findings suggest that IVIG therapy is beneficial in DM with CIDP Our patient developed acute vertical diplopia as a main manifes- as they have a significantly better response to IVIG than Idiopathic CIDP. tation of APV. Neuro-otological evaluation showed findings suggestive of selectively involving unilateral PC and utricle. Normal results doi:10.1016/j.jns.2019.10.1216 of initial and follow-up MRIs, and decreased VOR gain of unilateral ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 225

WCN19-0654 Parkinson’s disease (PD) is well-known as a progressive and degenerative disease of nervous system. The degeneration of dopaminergic neurons in substantia nigra, and reduction in amount Poster shift 03 - Autonomic nervous system disorders/history of of neurotransmitter dopamine available in striatum relate symptoms neurology/motor neuron disease/movement disorders/MS & of this disease. It is hypothesized that a drug delivery system that demyelinating diseases/neuromuscular disorders/vestibular dis- provides controlled and sustained release of PD drugs would afford orders and nutritional disorders or intoxications better management of PD. Hollow microcapsules composed of PMMA (polymethyl methacrylate) and poly(caprolactone)(PCL) are Post bariatric surgery neurological complications - A single center prepared through a modified double-emulsion technique. They are experience loaded with three PD drugs, i.e., levodopa (LD), carbidopa (CD), and entacapone (ENT), at a ratio of 4:1:8. M. Khan, Y. Malik, S.K. Habibullah, A. Al Madani Microcapsules were prepared through a double emulsion (W1/O/ fi Rashid Hospital, Neurology, Dubai, United Arab Emirates W2) solvent evaporation method with modi cations to produce hollow microshpheres. Microcapsules were then spray coated along with ENT. Shape and surface morphology were studied using SEM & Background TEM. Average particle size and polydispersity index were determined Obesity epidemic has now hit most countries of the world and by optical microscopy, drug entrapment, CLSM, Buoyancy tests and bariatric surgery is now an established treatment for it. Neurological in-vitro drug release was studied. deficits arising from malnutrition are known complications of LD and CD are localized in both hollow cavity and PMMA/PCL shell, bariatric surgery. Our aim was to determine the proportion of while ENT is localized in PMMA/PCL shell. Release kinetics of hydropho- bariatric surgery patients who end up with neurological complica- bic ENT is observed to be relatively slow as compared to other tions at our center and the factors that predict their development. hydrophilic drugs. It is further hypothesized that encapsulating ENT into PCL as a surface coating onto these microcapsules can aid in accelerating Methods its release. Now, these spray-coated hollow microcapsules exhibit similar We carried out a retrospective medical record based review of all release kinetics, according to Higuchi’srate,forallthreedrugs. bariatric surgery cases at our center between 2009 and 2017. All Results suggest that multiple drug encapsulation of LD,CD, and those who had been admitted or seen in outpatient clinic with a ENT in gastric floating microcapsules could be further developed for neurological complication, deemed to be secondary to nutritional in-vivo evaluation for management of PD. deficiency by the treating neurologist, were identified. doi:10.1016/j.jns.2019.10.1218 Results A total of 670 bariatric surgery procedures were carried out during the 9 year period. A total of 18 patients had re-presented to the hospital with a neurological complication. 12 cases were of polyneuropathy, 6 were mononeuropathies and 2 patients had WCN19-0669 Wernicke’s encephalopathy. Mean age of the cases was 32 years fi (SD 7.5). 60% of the patients presented within the rst 4 months of Poster shift 03 - Autonomic nervous system disorders/history of surgery. Almost all had suffered from protracted vomiting post neurology/motor neuron disease/movement disorders/MS & surgery and had vitamin deficiencies. demyelinating diseases/neuromuscular disorders/vestibular dis- Conclusion orders and nutritional disorders or intoxications The rate of neurological complications at our center was low (2.6%). Most cases were of polyneuropathy. Post-operative Secondary hyperparathyroidism quadriplegia mimicking vomiting was the common risk factor identified. Special attention Guillain-Barré syndrome, a case report should be paid to vitamin supplementation in patients who suffer from post bariatric surgery vomiting in order to avoid these A. Elkady, Y. Salah, A. Zakaria complications. Mataria Teaching Hospital, Neurology Department, Cairo, Egypt doi:10.1016/j.jns.2019.10.1217 Introduction Neuromuscular disorder as initial manifestation of secondary hyperparathyroidism (SHPT) was reported, it could be associated with a variety of clinical presentations. WCN19-0664 Case report A 62 year-old woman, obese, known diabetic for 15 years, Poster shift 03 - Autonomic nervous system disorders/history of presented with proximal weakness and pain in her right then left neurology/motor neuron disease/movement disorders/MS & lower limb, then progressed to the trunk and both upper limbs over demyelinating diseases/neuromuscular disorders/vestibular dis- 10 days until she became quadriplegic suggestive of GBS. Neurolog- orders and nutritional disorders or intoxications ical examination showed symmetric weakness in her lower extremities (1/5) more than her upper extremities (3/5). Reflexes were -1 Sustained release triple drug loaded colloidosomes for manage- all over except for the ankle reflex which was absent bilaterally. NCV ment of Parkinson’s disease showed delayed peripheral sensorimotor response with delayed F wave, but H reflex was present. Her initial laboratories were normal M. Bhargavaa, S. Bhargavab including CSF, except mild elevated creatine kinase (CK), alkaline aGTB Hospital, R&D, Kanpur, India phosphatase (ALP) and hypophosphatemia. After 4 plasmapharesis bSigna College of Pharmacy, Pharmacy, Kanpur, India sessions, there was no improvement. Hence, extended metabolic ARTICLE IN PRESS

226 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx profile was done showing significant elevation of parathyroid (13/45) achieved NEDA-4. 51% (23/45) were now on second-line hormone, CK, ALP, severe hypophosphatemia and kidney functions DMT (fingolimod 47%; alemtuzumab 2% and natalizumab 2%) impairment. EMG revealed myopathic changes in the proximal muscles, parathyroid SPECT-CT scans was negative, while serum Conclusion vitamin D was b 4 ng/ml. As a result, the diagnosis of SHPT NEDA-4 has the potential to capture the impact of DMT therapies complicated by hypophosphatemia leading to rhabdomyolysis and in RRMS. a-BVL may be a key domain to consider when assessing the muscle weakness was made. A dose of 600,000 IU cholecalciferol was effectiveness of the first line DMT. Considering NEDA-4 status, administered with correction of hypophosphatemia. Not only did clinicians may select a second-line DMT earlier in the disease course this show mild instant improvement, but surprisingly she made a to control subclinical disease activity of RRMS. complete recovery over the next 2 months. doi:10.1016/j.jns.2019.10.1220 Conclusion Ascending weakness mimicking GBS could occur in patients with SHPT and is likely to improve following treatment of the cause. WCN19-0682 doi:10.1016/j.jns.2019.10.1219

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-0680 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

Poster shift 03 - Autonomic nervous system disorders/history of Clinical heterogeneity and diagnostic challenges in poems syn- neurology/motor neuron disease/movement disorders/MS & drome and variant: A case series from Southeast Asia demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications Y.H. Lau, F.L. Hiew Hospital Kuala Lumpur, Neurology Department, Kuala Lumpur, Serial decline of no evidence of disease activity-4 status in early Malaysia disease stages of multiple sclerosis POEMS syndrome is a rare paraneoplastic disorder associated a a b a a C. Guevara , M. Martinez , P. Orellana , C. Silva-Rosas , V. Diaz ,J.De with an underlying plasma cell dyscrasia presenting with poly- b b Gazia , C. Garrido neuropathy, organomegaly, endocrinopathy, monoclonal protein, a Universidad de Chile, Neurology, Santiago, Chile and skin changes. Diagnosis can be challenging especially among b Universidad de Chile, Radiology, Santiago, Chile those with atypical presentation. The diagnosis of POEMS was based on published criteria. We Background reviewed the clinical, laboratory and imaging characteristics of 4 In relapsing-remitting multiple sclerosis (RRMS), no evidence of patients in Kuala Lumpur Hospital from year 2012-2018. Clinical disease activity-3 (NEDA-3) is defined as no relapses, no disability spectrum and disease variant were determined. progression and no MRI activity. NEDA-4 status is defined as meeting Three had POEMS syndrome and 1 had Castleman disease variant. all NEDA-3 criteria plus having an annualized brain volume loss (a- Mean age of onset was 40 (SD 11.5), with male predominant (75%). BVL) of ≤ 0.4%. All had refractory chronic demyelinating polyneuropathy to standard CIDP treatment as common presentation. Paraprotein IgA lambda Objective from monoclonal plasma cell-proliferative disorder were found in 2 To determine the impact of adding a-BVL to NEDA-3 (NEDA-4). patients. Sclerotic bone lesion (100%) was the most common major criteria fulfilled. Organomegaly (75%) was the most common minor Methods criteria fulfilled, followed by extravascular volume overload (oe- Forty-five patients were prospectively followed at baseline, dema, 50%, pleural effusion 25%), papilledema (25%), thrombocytosis months 12 and 24, at the University of Chile Hospital. SIENA (25%) and acrocyanosis (25%). Finger clubbing was seen in 50% of the software was used to assess a-BVL. patients. The only surviving patient with Castleman variant had improvement of neuropathy following IV rituximab and was in Results remission following autologous bone marrow transplant. Rapidly The patients had a mean age of 33.0 years (18-57), disease progressive disease was observed in one patient, with clinical duration of 1.9 years (0.4-4), EDSS score of 1.3 (0-4) and 67% were deterioration to completely bedbound within a year. female. 73% were on first-line DMT (interferons (53%), glatiramer In conclusion, POEMS syndrome is a rare disorder and should be acetate (11%), teriflunomide (9.0%)) and 18% were on second-line suspected in atypical cases of CIDP particularly when there is DMT (fingolimod). treatment resistance. Extensive investigation is often needed to At 12 months, 58% (26/45) of patients achieved NEDA-3. An a- meet diagnostic criteria for POEMS and rapid referral to haematology BVL of N0.4% was observed in 52.0% (21/45) (mean: 0.45%). Adding team for definite treatment is warranted. a-BVL (threshold of 0.4%) the proportion of patients achieving NEDA- 4 was 36% (16/45). doi:10.1016/j.jns.2019.10.1221 At 24 months, 48% (22/45) of patients achieved NEDA-3. An a-BVL of N0.4% was observed in 49 % (22/45) (mean: 0.53%). 28% ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 227

WCN19-0686 M.K. Acharyaa, S. Banerjeeb, A. Panditc, G. Gangopadhayc aNeuromedicine, Kolkata, India bBangur Institute of Neurosciences, Neuromedicine, Kolkata, India Poster shift 03 - Autonomic nervous system disorders/history of cBangur Institute of Neurosciences, Neuromedicine, Kolkata, India neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- Background orders and nutritional disorders or intoxications Clinical, radiological and laboratory data of the cases of long segment myelitis (LSM) who fail to respond to steroid pulse or recur Determinants of caregiver burden in Parkinson’s disease after initial response are lacking.

K. Ananda, M. Chandrab, A. Junejac, P. Kathuriab Methods aDr RML Hospital, Neurology, New Delhi, India We included patients of LSM, who either failed to respond to steroid bDr. RML Hospital & PGIMER, Psychiatry, New Delhi, India pulse therapy or recurred while on maintenance steroid therapy after cDr. RML Hospital & PGIMER, Neurology, New Delhi, India initial response. Their clinical, radiological and laboratory data were recorded. CSF TB PCR, Anti-aquaporin4-Ab, ANA and Serum ACE level, Background VDRL, ANA and ANCA were tested in all cases. IV Immunoglobulin Parkinson's disease (PD) is a neurodegenerative disorder with (IVIG), Plasmapheresis or four weekly pulse of rituximab (375 mg/m2/ significant impact on the quality of life of patients and their dose) were tried either alone or in combination. Patients were followed caregivers. The present study aims to study the burden on caregivers up clinically for the rest of the study period. in PD using Zarit Caregiver Burden Inventory (ZCBI). Results Methods Among the sixteen patients (eleven steroid non-responders fi fi Eighty patients of Parkinson’s disease and their primary care- and ve having new/recurrent clinical de cits) MRI showed lesions givers who fulfilled inclusion and exclusion criteria were recruited in cervical cord in four, dorso-lumber segment in nine, three had for the study after institutional ethical clearance. Diagnosis of PD was both and in two cases there were brain involvement. Most common established using UK Brain Bank criteria; severity/staging of CSF abnormality was elevated protein followed by lymphocytic Parkinson’s disease was done by Movement Disorder Society - pleocytosis. Aquaporin4-IgG was positive in four. Seven patients Unified Parkinson’s disease rating scale (MDS-UPDRS-III). Caregiver received IVIG, plasmapheresis was done in two, another four patients burden was assessed with the Zarit Caregiver Burden Inventory were treated with rituximab and combination of treatment was tried (ZCBI). in two. Motor power improvement was documented in three patients treated with immunoglobulin and/or plasmapheresis. Results One patient responded to empirical ATD. Rituximab was given to Eighty patients of Parkinson’s disease with mean age of 61.48 ± aquaporin positive cases only and none showed any further 6.71 were enrolled in the study. Most of them were male patients relapse till date. No major treatment related side effects were (70%). An equal number of primary caregivers were enrolled. Most of documented. them were middle aged (52.26 ± 6.80) females (71.3%) and spouses of patients (80%). Caregiver burden was significantly related to the Conclusion age of the patient (p value b 0.001), duration of illness (p value b Treatment of acute attacks of LSM, not responding to steroids and 0.007) and Hoehn and Yahr stage of Parkinson’s disease (p value b that of neuromyelitis optica with rituximab shows good results. 0.000). While there was no significant relation found between doi:10.1016/j.jns.2019.10.1223 caregiver burden and age of caregiver or type of relationship with patient.

Conclusions Caregiver burden in PD is high and related to duration and WCN19-0691 severity of illness. Caregiver burden is not related to age of caregiver or type of relationship with the patient. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & doi:10.1016/j.jns.2019.10.1222 demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-0690 Clinical condition and prognosis of inpatients with Fukuyama congenital muscular dystrophy in specialized institutions for muscular dystrophy in Japan Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & K. Ogataa, T. Murakamia, M. Suzukia, T. Takahashib, S. Kuruc, T. Saitod demyelinating diseases/neuromuscular disorders/vestibular dis- aNational Hospital Organization Higashisaitama National Hospital, orders and nutritional disorders or intoxications Department of Neurology, Hasuda, Japan bNational Hospital Organization Sendai-Nishitaga Hospital, Department Steroid failure in primary/recurrent long segment myelitis: What of Neurology, Sendai, Japan c else? National Hospital Organization Suzuka National Hospital, Department of Neurology, Suzuka, Japan ARTICLE IN PRESS

228 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx dNational Hospital Organization Osaka Toneyama Medical Center, Results Department of Neurology, Toyonaka, Japan Before treatment, HFMSE scores were 4, 2 and 7 points respectively. The RULM scores before treatment were 14, 18 and 16 Fukuyama congenital muscular dystrophy (FCMD) is the second points respectively. The HFMSE scores after nusinersen treatment for most common form of muscular dystrophy in the Japanese 6 months were 4, 2 and 5 points respectively, and the RULM scores population and is caused by mutations in the fukutin (FKTN) gene. were 14, 20 and 16 points. Before treatment, %VC were 18.5%, 52%, We analyzed clinical condition and prognosis of inpatients with 84.5%, and FEV1.0% were 91.4%, 80.9%, 78.1%, respectively. After FCMD registered in the database of wards specialized for muscular treatment, %VC were 17.8%, 62.5%, 84.5%, and the FEV1s rate was dystrophy established in 27 national hospitals. From 1999 to 2013, 95.4%, 95.8%, 79.2%, respectively. In Cases 2 and 3, waning was 135 patients with FCMD were registered. Mechanical ventilation observed in the repetitive nerve stimulation test before treatment, (MV) was introduced to 94 patients (69.7%), and the mean age at MV but it disappeared after treatment. introduction was 25.1 ± 6.1 years (4~35). Among 94 patients with MV, 47 had progressed to tracheostomized positive pressure Conclusion ventilation (TPPV). Tube feeding (TF) was introduced to 97 patients Although no muscle strengthening effect was found in skeletal (71.9%), and the mean age at TF introduction was 25.5 ± 5.6 years muscle, respiratory function was improved. The disappearance of (17~37). There were 53 cases of death reported in the database. The waning after treatment suggested that nusinersen improves the mean age of death was 27.37.3 years (5.4~43.6). The causes of death function of the neuromuscular junction. included 15 heart failures, 12 respiratory failures, 8 pulmonary infections, 6 sudden deaths. In our database, clinical progression of FCMD seemed slower than in the Japanese national registry of FCMD reported in Ishigaki et al, 2018. This difference might due to variation doi:10.1016/j.jns.2019.10.1225 of clinical course among FCMD patients, for which any modiﬁer other than FKTN gene mutation should be accountable. doi:10.1016/j.jns.2019.10.1224 WCN19-0705

Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & WCN19-0703 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 & Recognition and prevalence of non motor symptoms of demyelinating diseases/neuromuscular disorders/vestibular dis- Parkinson’s disease and their awareness; study using non motor orders and nutritional disorders or intoxications symptoms questionnaire in 50 patients

The effects of nusinersen in SMA patients more than 50 years N. Yaqoob, A. Herekar after onset Baqai Medical University, Neurology, Karachi, Pakistan

A. Hashiguchi, Y. Higuchi, Y. Tashiro, H. Arata, H. Takashima Objective Kagoshima University Hospital, Neurology- Neurological Disease Center, The aim of this study was to explore the prevalence of non motor ’ ’ Kagoshima, Japan symptoms of Parkinson s disease and the patient s awareness of these symptoms by using Non Motor Symptoms, (NMS) screening questionnaire consisting of 30 items. Purpose Improvements in muscle strength have been seen in children Place and duration of study with SMA. On the other hand, there are few patients receiving treatment in elderly adult patients over 50 years old, and the effect is Baqai Medical University, Karachi, Pakistan from March 2017 to January 2018. unknown. To conﬁrm the nucinersen effect in SMA patients more than 50 years after onset. Patients & methods ﬁ Methods 50 patients were evaluated using the de nitive version of Non Motor Symptoms (NMS) questionnaire, in the Out Patient Depart- Three SMA patients more than 50 years after onset were evaluated before and after nusinersen treatment. The muscle ment (OPD), highlighting the frequency of non motor symptoms in males and females patients of Parkinson’s disease. strength was evaluated with HFMSE (Hammersmith functional motor scale-expanded) and RULM (Revised upper limb module). Respiratory function was assessed by %VC (% vital capacity) and Results FEV1.0% (forced expiratory volume in one second, %). In electro- All 50 patients had more than one symptom. Symptoms encoun- physiological examination, nerve conduction examination and tered in patients were feeling anxious and frightened 42 (84%), feeling repetitive nerve stimulation test were performed. sad, low and blue 39 (78%), unpleasant sensation in legs 35 (70%), unexplained pains 35 (70%), feeling light headed and dizzy 33 (66%), ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 229 sense of urgency to pass urine 31 (62%), unexplained change in weight Conclusions 28 (56%), difficulty getting to sleep 26 (52%), dribbling saliva 22 (44%), Results of our study confirm glial cells damage in PD. difficulty concentrating 20 (40%), constipation 30 (60%), problem Additionally, a positive correalation between csf and serum levels remembering things 30 (60%). Feeling anxious and frightened was of S100B among parkinsonian patients, confirm BBB damage in PD the most common symptom in males (84%) and feeling sad, low and and suport the concept of S100B being a biomarker of BBB blue in females (100%). 46 patients were unaware of any relationship permeability. between Parkinson’s disease and these non motor Symptoms.

Conclusion For Parkinson’s disease to be properly managed, non motor doi:10.1016/j.jns.2019.10.1227 symptoms should be comprehensively assessed and addressed to ensure that the patients are informed about their association with Parkinson’s disease. WCN19-0730 Keywords: Parkinson’s disease, Motor Symptoms, NMS questionnaire Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1226 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications

WCN19-0715 Comparison of clinical characteristics, therapeutic responses and outcomes in myelin-oligodendrocyte-glycoprotein-mog associated nmosd versus AQP4 associated NMOSD in a consecutive Poster shift 03 - Autonomic nervous system disorders/history of cohort of Sri Lankan patients neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular dis- B. Senanayakea, M. Aravinthana, P. Thakshana, J. Jitprapaikulsanb,U. orders and nutritional disorders or intoxications Ranawakac, M. Riffsyd, J. Wijesekerae, S. Pittockf aNational Hospital of Sri Lanka, Institute of Neurology, Colombo, Sri Increased serum and CSF levels of S100B reflect glial cells Lanka degeneration in PD bMayo Clinic, Department of Laboratory Medicine and Pathology, Phoenix, USA c E. Papuć, K. Rejdak University of Kelaniya, Department of Medicine, Kelaniya, Medical University of Lublin, Department of Neurology, Lublin, Poland Sri Lanka dDr. Neville Fernando Teaching Hospital, Department of Neurology, Introduction Colombo, Sri Lanka e There is increasing evidence that glial cells degeneration is Medway NHS Foundation Trust, Neurology, Kent, United Kingdom f involved in the initiation and progression of PD in addition to neural Mayo Clinic, Center for MS and Autoimmune Neurology, Rochester, structures. USA S100B is a calcium binding protein, mainly expressed and produced in astrocytes. It has been shown that S100B is only Introduction expressed by a subtype of mature astrocytes that ensheath blood Neuromyelitis optica spectrum disorder(NMOSD) has been vessels. S100B is also a candidate peripheral biomarker of blood– reported in association with aquaporin-4(AQP4)-IgG positivity. brain barrier (BBB) permeability. Seronegative cases were later identified myelin-oligodendrocyte- glycoprotein(MOG)-IgG positivities. The clinical courses of these 2 Methods groups differ. Subjects were 28 patients with PD and 28 healthy controls. All PD and control subjects underwent S100B assessment in serum and Objective csf. Both groups were matched for gender but not for age (PD 65.18 To compare the clinical phenotypes and outcomes of AQP4-IgG ± 8,64 vs controls 48.14 ± 13.71 years; p N 0.05). S100B levels and MOG-IgG positive NMOSD patients. in serum and csf were assessed using commercially available ELISA kit. Method Sri Lankan patients fulfilling revised NMOSD diagnostic criteria in Results a CNS inflammatory demyelinating disorders(IDDs) cohort were We found statistically significant differences in serum levels of included. Serostatus and clinical information were collected. S100B protein between PD and healthy controls groups (24,65 ± 11.94 vs 14,47 ± 7,24 pg/ml; p b 0.05). Results Csf levels of S100B protein in PD patients were also significantly Among 810 IDDs patients, 71 patients were identified, of whom higher than in healthy controls (133,52 ± 54.72 vs 114,86 ± 120.69 37 were AQP4-IgG positive and 34 were MOG-IgG1 positive. MOG- pg/ml; p b 0.05). IgG1 positive were females 20/34(58.8%) while AQP4-IgG positives No correlation was found between serum and csf levels of S100B patients were 32/37(86.4%) (P = 0.009). AQP4-IgG positive NMOSD among healthy control subjects. Statistically significant positive presented with isolated transverse myelitis(TM) in 12/34(32.4%), correlation was found between serum and csf levels of S100B among isolated optic neuritis(ON) in 8/34(21.6 %), and TM with ON in 8/34 PD patients (p = 0.003). (21.6%) whereas MOG-IgG positive NMOSD presented with TM with ARTICLE IN PRESS

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

ON in 9/37(26.4%), encephalitis with ON in 8/37(23.5%), and isolated WCN19-0743 ON in 6/37(17.6%). Area postrema syndrome (APS) was seen in the AQP4-IgG positive (8, 21.6%) compare with MOG-IgG positive patients (1, 2.9%) (P = 0.018). Eighty-seven percent of MOG-IgG1 Poster shift 03 - Autonomic nervous system disorders/history of positive and 97.2% of AQP4-IgG positive were treated during the ﬁrst neurology/motor neuron disease/movement disorders/MS & clinically encountered attack. Maintenance oral immunosuppres- demyelinating diseases/neuromuscular disorders/vestibular dis- sants were used in 47% and 94% in MOG-IgG1 positive and AQP4-IgG orders and nutritional disorders or intoxications positive patients, respectively. Considering the mobility outcomes, 86% of MOG-IgG1 positive and 94% of AQP4-IgG positive NMOSD are Ischemic monomelic neuropathy: A rare complication after currently walking unsupported. brachiocephalic arteriovenous ﬁstula

Conclusions H. Elhasina, A. Hassanb, N. Solimanb, M. Szolicsc ON is more common in MOG-IgG positive patients than AQP4-IgG aTawam Hospital, Neurology, Al Ain, United Arab Emirates positive NMOSD whereas APS is rare in MOG-IgG NMOSD patients. bTawam Hospital, Neurology, Al Ain, United Arab Emirates Mobility outcomes of AQP4-IgG positive NMOSD are better than cTawam Hospital, Neurology, Al Ain, United Arab Emirates previous reports. Background doi:10.1016/j.jns.2019.10.1228 Ischemic monomelic neuropathy (IMN) is a rare neuropathy of the distal nerves due to ischemia secondary to acute loss of proximal ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 231 arterial supply as a result of shunting or non-compressive occlusion. Methods The main features of IMN are multiple distal focal mononeuropathies Patients with type 2 diabetes mellitus (T2DM) with or without with axonal injury. symptoms of neuropathy were retrospectively enrolled. Demo- graphic data, clinical laboratory data, MNSI score, and NCS results Case report were collected for analysis; DPN was diagnosed based on the MNSI A 71 years old female patient with history of end stage renal score (≥ 3.0) and abnormal NCS results. disease on hemodialysis and type 2 diabetes. Presented with right hand persistent pain and reduced hand function that started 24 h Results after formation of right brachiocephalic arteriovenous (AV) fistula. The final analyses included 198 patients (115 men and 83 Doppler scan was done suggesting of steal syndrome. Patient women) with a mean age of 62.6 ± 12.7 years. The mean MNSI then underwent MILLER banding procedure for the fistula. score was 2.8 (range, 0.0–9.0), and 69 patients (34.8%) were Despite good vascular supply, the right hand pain did not improve diagnosed with DPN. The MNSI score was positively correlated with and was partially responding to gabapentin and codeine+ the median motor nerve latency and negatively correlated withthe paracetamol. median motor, ulnar sensory, peroneal, tibial, and sural nerve On examination: The right hand was warm and the capillary refill conduction velocities (NCVs). When the patients were categorized was satisfactory, palpable radial pulse with no signs of tissue into quartiles according to MNSI score, peroneal nerve conduction ischemia. She was unable to flex the wrist and fingers of the right velocity was significantly lower in the second MNSI quartile than in hand and she had clawing of right hand. She had allodynia and the first MNSIquartile (p = 0.001). A multivariate analysis revealed hyperalgesia in the distribution of right median, ulnar nerves. that the peroneal NCV was independently associated with MNSI Nerve conduction study showed absent sensory responses from score after adjusting for age, sex, and glycosylated hemoglobin A1c the right median, ulnar and radial nerves. The right median and ulnar (HbA1c) levels. motor responses were significantly attenuated suggestive of a severe axonal neuropathy and peripheral neuropathy secondary to diabetes. Conclusions She was referred to vascular surgery for fistula ligation. The present results indicate that a decrease in peroneal NCV was responsible for early sensory deficits in T2DM patients. Conclusion IMN is a potentially rare devastating complication of arteriove- doi:10.1016/j.jns.2019.10.1230 nous access for hemodialysis which can result in permanent disability. doi:10.1016/j.jns.2019.10.1229 WCN19-0756

Poster shift 03 - Autonomic nervous system disorders/history of WCN19-0748 neurology/motor neuron disease/movement disorders/MS & 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 & Bifacial weakness with paresthesia - Variant of Guillain-Barre demyelinating diseases/neuromuscular disorders/vestibular dis- syndrome: A case report orders and nutritional disorders or intoxications S.M. Al Alawia, R. Renganathanb The relations of variables of nerve conduction studies to clinical aShamma Masoud AL Alawi, Neurology, Abu Dhabi, United Arab symptoms scores in patients with type 2 diabetes Emirates; bMedical, Neurology, Abu Dhabi, United Arab Emirates J.H. Park Inje University Sanggye Paik Hospital- Inje University College of Al Alawi S1, Alduhoori A2, Renganathan R3. Medicine, Departments of Neurology, Seoul, Republic of Korea 1R3 Medical Resident, 2R6 Medical Resident, 3,Consultant Neurol- ogist, AAH Objectives Diabetic peripheral polyneuropathy (DPN) presents a various Background degree of symptoms from wide spectrum of nerve damages. Nerve Guillain-Barre syndrome (GBS) is an acute post-infectious conduction studies (NCSs) are considered to be the gold standard for polyneuropathy usually presenting with progressive weakness of nerve damage, but they are often dissociated with subjective limbs. About 30–60% have facial palsy. symptoms in patients with DPN. The aim of this study was to investigate the NCS manifestations according to symptoms quanti- Case description ﬁed by the Michigan Neuropathy Screening Instrument (MNSI) A 40 year old female with history of hypertension, presented with symptom scores. numbness of lower and upper limbs and right facial weakness, ARTICLE IN PRESS

232 Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx slurred speech and difficulty chewing. She denied any significant as axonal GBS with Hadden’s and one shifted to AIDP with Rajabally’s weakness in limbs. She had recent h/o flu-like illness. Examination criteria. One patient classified as inexcitable form with Hadden’s showed LMN-type facial weakness bilaterally (RNL), are flexia and criteria moved to axonal. In summary, serial NCS revealed subtype reduced pinprick sensation in distal limbs. Power in limbs was change in 15% of patients with Hadden’s criteria and 10% with normal. CT brain was normal. CSF showed raised protein with Rajabally’s criteria. So single study might still be insufficient even normal cells. Autoimmune screen and serum ACE were negative. after Rajabally’s modification but a larger study is needed. Anti-glycolipid antibodies were not done. Nerve conduction studies showed bilateral facial mononeuropathies & acute demyelin- doi:10.1016/j.jns.2019.10.1232 ating predominantly motor polyneuropathy with secondary axonal loss, supporting the diagnosis of ‘facial diplegia with paresthesia variant’ of GBS. She was treated with a course of IVIG and physiotherapy. She developed neuropathic pain in limbs which WCN19-0772 responded to Gabapentin. One week after discharge, she received ^ 2nd course of IVIG. Her facial weakness showed about 70–80% improvement when last seen in the clinic. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Discussion demyelinating diseases/neuromuscular disorders/vestibular dis- Bifacial weakness with paresthesia is a rare variant of GBS first orders and nutritional disorders or intoxications described by Ropper in 1994. Our patient had early diagnosis and fi was treated appropriately with fairly signi cant improvement Morphine-induced myoclonus in an 85-year old male: A case clinically. report

Conclusion D.L. Que, M. Baltazar, L. Laxamana, M.C. Macrohon-Valdez, A. Ng When patients present with bifacial weakness, it is important to St. Luke's Medical Center, Institute for Neurosciences- Section of Adult consider GBS in the differential diagnosis. Also, it is important to Neurology, Quezon City, Philippines. recognize that GBS variants can present without any motor weakness in limbs. ^Morphine, the prototype of opioid, has been known for its doi:10.1016/j.jns.2019.10.1231 effectivity in pain control. However, metabolism of morphine produces substrates that could be neurotoxic and hence making the drug dangerous at high concentrations and with chronic use. Morphine undergoes glucuronidation in the liver, producing morphine-6-glucuronide and morphine-3-glucuronide. Once a threshold WCN19-0766 concentrations of morphine-3-glucuronide is reached, neurotoxicity effects may manifest. Myoclonus and seizure are among these. Poster shift 03 - Autonomic nervous system disorders/history of Metabolism of opioids may be affected by the patient's clinical neurology/motor neuron disease/movement disorders/MS & condition, including the age, creatinine clearance and liver function.^ We discuss a case of an 85-year-old male who had a chronic demyelinating diseases/neuromuscular disorders/vestibular dis- ^ intake of high doses of Morphine with an impaired renal function, orders and nutritional disorders or intoxications and who later developed myoclonus. We reviewed the metabolism of Morphine and how its metabolites could have caused neuro- Electrodiagnosis of Guillain-Barré syndrome subtypes: Single and excitatory effects which later led to the myoclonic movements of the serial nerve conduction study (NCS) patient. The patient improved with Diazepam, Piracetam, Valproic, Clonazepam and Levetiracetam. He was eventually sent home with Z.P. Tun, O. Ohnmar, W.M. Thit resolution of the myoclonus and spasms and maintained only on University of Medicine 1, Department of Neurology- Yangon General Levetiracetam 500 mg twice a day. Hospital, Yangon, Myanmar, Burma doi:10.1016/j.jns.2019.10.1233 We aimed to describe the electrodiagnosis of Guillain-Barré syndrome (GBS) subtypes and their changes in serial NCS. We analyzed 40 GBS patients admitted to Yangon General Hospital, Myanmar over 1 year in 2018. NCS was performed twice serially at WCN19-0777 least 2 weeks apart and subtypes were classified according to Hadden’s and Rajabally’s electrodiagnostic criterias. Out of 40 patients, 60% were male and 40% were female. Mean age was 35.4 Poster shift 03 - Autonomic nervous system disorders/history of years. First NCS was done 11.3 ± 4.6 days after onset and second NCS neurology/motor neuron disease/movement disorders/MS & 35.1 ± 7.7 days after onset. Comparing single and serial NCS, demyelinating diseases/neuromuscular disorders/vestibular dis- Hadden’s criteria diagnosed AIDP in 45% vs 52.5%, axonal GBS in 35% orders and nutritional disorders or intoxications vs 32.5%, equivocal in 15% vs 12.5% and inexcitable form in 5% vs ’ 2.5% and Rajabally s criteria diagnosed AIDP in 45% vs 52.5%, axonal Sporadic case of bulbar amyotrophic lateral sclerosis in elderly GBS in 42.5% vs 35%, equivocal in 12.5% vs 12.5%. 6 patients (15%) patient following cholecystectomy changed classification with Hadden’s and 4 patients (10%) with Rajabally’s criteria after second study. From axonal group, two B. Moscua, I. Roman-Filipb, C.C. Roman-Filipb patients shifted to AIDP and one patient to equivocal form with both aAcademic Emergency Hospital, Neurology, Sibiu, Romania criterias. From equivocal group, one was reclassified as AIDP and one b Lucian Blaga University, Faculty of Medicine, Sibiu, Romania^ ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 233

Introduction 3 years history of multiple falls, along with difficulty in swallowing Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenera- and speaking that resulted in a weight loss of 71 kg. In addition, he tive disease. 70% of the affected individuals develop the spinal form of the suffered an apparent cognitive decline and some psychiatric disease, while 30% develop bulbar changes. 85^% of the individuals with disturbances, on top of his baseline obsessive-compulsive behavior. spinal form of the disease develop bulbar changes as a result of disease He was initially misdiagnosed and investigated for Huntington's progression. Bulbar form of ALS is associated with a poorer prognosis disease, Huntington-like diseases, and malnutrition but the (average survival rate less than two years) and lower quality of life. workup denied these diagnoses. The patient underwent several investigations including laboratory investigations, a brain MRI, and Case presentation electrodiagnostic studies. His lab tests revealed red blood cell We report the case of a 76 years old female presenting with acanthocytosis of more than 5% on the peripheral smear, in addition dysphonia and mild dysphagia following laparoscopic cholecystectomy to elevated lactate dehydrogenase and creatinine phosphokinase. His with general anesthesia (Jan. 2017). Past medical history was not brain MRI was unremarkable. Nerve conduction studies and significant. Immediate neurological examination reveals dysphonia electromyography performed on all four limbs and paraspinal and moderate dysphagia, present pharyngeal reflex and no significant muscles revealed findings suggestive of chronic polyneuropathy. In motor impairment. Blood tests (biochemistry, HIV serology, hepatitis view of the absence of targeted treatment for neuroacanthocytosis, B, C and syphilis testing, CK, Ab antiMuSK, Ab-antiACH, Ab the prognosis was explained to the family and the importance antineuronals, neoplasic markers) were negative. Cerebral CT and of follow-up was emphasized to optimize symptomatic fi MRI reveals a mild cortical atrophy without signi cant changes. ENG management.^ and needle guided EMG examination was performed, revealing specific changes with moderate axonal degeneration of the right doi:10.1016/j.jns.2019.10.1235 peroneus muscle, rich spontaneous potentials and decreased recruitment. Possible bulbar form of ALS is diagnosed and Riluzole (2*5 mg/ day) treatment was started. The patient developed general muscular weakness and atrophy, swallowing difficulties and finally respiratory WCN19-0785 insufficiency, dying 22 months after initial diagnosis.

Conclusion Poster shift 03 - Autonomic nervous system disorders/history of Our case reported shows particular bulbar form of ALS in an neurology/motor neuron disease/movement disorders/MS & elderly patient following general anesthesia associated with a severe demyelinating diseases/neuromuscular disorders/vestibular dis- prognosis and signiﬁcant impairment. orders and nutritional disorders or intoxications doi:10.1016/j.jns.2019.10.1234 Steroid resistant recurrent transverse myelitis in an elderly female with good response to plasma pheresis: A case report

D.L. Que, L. Damian WCN19-0782 St Luke's Medical Center Quezon City, Institute for Neurosciences, Quezon City, Philippines^ Poster shift 03 - Autonomic nervous system disorders/history of fl neurology/motor neuron disease/movement disorders/MS & Transverse myelitis is a rare disease involving in ammation of the spinal cord. It is estimated to have an incidence of 1.34 to 4.6 per demyelinating diseases/neuromuscular disorders/vestibular dis- million. It is more common in females and has a bimodal peak at orders and nutritional disorders or intoxications 10–19 years old and 30–39 years old. It is monophasic but may become recurrent in some. Diagnostic criteria for the spectrum of Neuroacanthocytosis, a rare and challenging diagnosis diseases involving transverse myelitis is still evolving. Studies have shown an association between recurrent transverse myelitis, neuro- a a b Y. Abdelmajid , N. Altalai , M. Atnasous myelitis optica spectrum and autoimmune antibodies associated a Rashid Hospital- Dubai Health Authority, Neurology, Dubai, United with systemic lupus erythematosus (SLE) and other systemic Arab Emirates autoimmune conditions. Management options for recurrent trans- b Saudi German Hospital, Neurology, Dubai, United Arab Emirates^ verse myelitis include steroids, plasmapheresis, immunosuppressive antimetabolites and monoclonal antibody. We are presented now Neuroacanthocytosis syndromes are a heterogeneous group of with an interesting case of recurrent transverse myelitis in an elderly genetically defined neurological disorders characterized by progres- female presenting with progressive weakness and numbness of the sive degeneration of the basal ganglia, along with red blood cell bilateral lower extremities. She tested positive for autoimmune acanthocytosis. Despite its likelihood of being under-diagnosed, its antibodies anti-ssA and anti-dsDNA. She had disease progression reported prevalence is approximately 1–5 per 1,000,000 individuals. despite management with intravenous steroids, hence was consid- Its onset is usually by the third or fourth decade of life, and it's ered steroid-resistant. The patient responded well with six sessions classically manifested by orolingual action-induced dystonia, gener- of plasmapheresis. alised chorea, vocal tics, and stereotypies. Other manifestations include dysphagia, dysarthria, peripheral neuropathy, cognitive doi:10.1016/j.jns.2019.10.1236 decline, areflexia, and parkinsonism. We herein report a rare case of neuroacanthocytosis in a 40 years old male who presented with a ARTICLE IN PRESS

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

WCN19-0788 Objective ^ To determine protective factors of monophasic course of demyelination diseases in children with long-term follow-up period. Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & Material and methods demyelinating diseases/neuromuscular disorders/vestibular dis- Case series: 75 children with the ﬁrst demyelinating event. Age orders and nutritional disorders or intoxications 2–17 years old (median is 12 years). Duration of the follow-up period was 0,5–17 years (median is 4.3 years.). Clinical signs, Demyelinating diseases in childhood: Associations with monoph- magnetic resonance imaging (MRI) data were estimated as prognos- asic course tic factors. For this aim we used two-sample survival analysis.

K. Nevmerzhitskayaa, A. Sergeevbc, S. Pavlovad, M. Sergeevac Results fi aUral State Medical University, Department of Neurology- Neurosurgery 33 patients (44%) were identi ed to have a monophasic course of fi and Genetics, Yekaterinburg, Russia demyelination disease. The nal diagnoses were: optic neuritis (n = bUral Federal University named after the first President of Russia B.N. 18), acute disseminated encephalomyelitis (n = 9), transverse ≤ Yeltsin- Institute of Radio-electronics and IT, Department of Information myelitis (n = 6). The younger age ( 10 years old), clinical presenta- fi Technology and Automation, Ekaterinburg, Russia tion with optic neuritis, normal brain MRI or poor-de ned, tumor- cInstitute of Industrial Ecology UB RAS, Laboratory of Physics and like lesions on MRI. The curves of the cumulative proportion without fi Ecology, Yekaterinburg, Russia relapse (Kaplan-Meier) are presented in the gure. dUral Federal University, Laboratory of Neurotechnology, Yekaterinburg, Russia Conclusion ^ We suggest that only long duration of follow-up after the first demyelinating event the provides a clear prognosis of the disease Background course. But revealed factors may determine fair prognosis of Monophasic demyelination diseases have favorable outcome in demyelination diseases. comparison with polyphasic disorders. doi:10.1016/j.jns.2019.10.1237 ARTICLE IN PRESS

Abstracts / Journal of the Neurological Sciences (2019) xxx–xxx 235

WCN19-0812 This research was supported by noncommercial grant 15–0155 from Slovak Agency for Science and Research.

Poster shift 03 - Autonomic nervous system disorders/history of doi:10.1016/j.jns.2019.10.1238 neurology/motor neuron disease/movement disorders/MS & demyelinating diseases/neuromuscular disorders/vestibular disorders and nutritional disorders or intoxications WCN19-0814 Cognitive and affective theory of mind deficits in Parkinson's disease Poster shift 03 - Autonomic nervous system disorders/history of neurology/motor neuron disease/movement disorders/MS & M. Kralovaa, K. Sujanskab, Z. Csefalvayc, J. Markovac, M. Papayovad,A. Kusnirovad, Z. Kosutzkad, P. Valkovicd, M. Hajduke demyelinating diseases/neuromuscular disorders/vestibular dis- aFaculty of Medicine Comenius University, Clinic of Psychiatry, Brati- orders and nutritional disorders or intoxications slava, Slovak Republic bFaculty of Arts- Comenius University, Department of Psychology, Radiological characteristics of neuromyelitis optica spectrum Bratislava, Slovak Republic disorder in Kuwait: A tertiary neurology centre experience cFaculty of Education- Comenius University, Department of Communi- cation Disorders, Bratislava, Slovak Republic I. Moustafaa, S. Faroukab, E. Abdelnabic, J. Al-Hashelad, R. Alroughanie dFaculty of Medicine- Comenius University, Second Clinic of Neurology, aIbn Sina Hospital, Department of Neurology, Kuwait City, Kuwait Bratislava, Slovak Republic bFaculty of Medicine- Al-Minia University, Department of Neurology, Al- eFaculty of Medicine- Faculty of Arts- Comenius University, Clinic of Minia, Egypt Psychiatry, Department of Psychology, Bratislava, Slovak Republic cIbn Sina Hospital, Department of Radiology, Kuwait City, Kuwait dHealth Sciences Centre- Kuwait University, Department of Medicine, Background Kuwait City, Kuwait – e Recent studies have highlighted impairment in social cognitive Amiri Hospital, Department of Neurology, Sharq, Kuwait^ abilities in patients with Parkinson's disease. The hallmark of the social cognition is Theory of Mind (ToM), ability to infer about Abstract mental states (beliefs, desires) of other people. Cognitive impairment Background in fronto – striatal circuits is related to difficulties in understanding Neuromyelitis optica spectrum disorder (NMOSD) is an autoim- of other people minds. mune demyelinating disorder of the central nervous system that was previously thought to be a subtype of multiple sclerosis (MS). We Objective describe radiological characteristics of a case series of NMOSD fi The aim of the study was to assess de cits in cognitive and patients from the largest tertiary neurology centre in Kuwait.^ affective ToM in Parkinson's disease. Secondary aim was to evaluate, whether deficits in ToM are related to cognitive impairment. Methods This is a case series study of NMOSD patients who were seen at Subjects and methods the Multiple Sclerosis clinic, Ibn Sina Hospital from January 2017 to Sample consisted of 88 patients with Parkinson's disease and 30 January 2019. We describe their magnetic resonance imaging (MRI) healthy individuals. Mean age of patients was M = 62.19 and SD = characteristics. 9.69. Comic Strip Task (Cognitive ToM), Reading the Mind in the Eye Test (Affective ToM) and neurocognitive battery was administered. Results Our study identified 32 patients who fulfilled the 2015 interna- Results tional NMOSD diagnostic criteria. Twenty-six females and 6 ^males We found significant differences across groups in Comic Strip were identified of whom 22 patients tested positive for AQP4 Task (t = −4.171, p b .001, d = −0.79) and Eyes Test (t = −4.313, antibodies in the serum while 10 were seronegative. The mean age p b .001, d = −1.10). Comic Strip Task was related to several was 35.4 and mean age at onset was 28.5 years with an average neurocognitive domains. We found weaker associations for Eyes disease duration of 7 years. There was a strong female predominance test. Both tests were moderately to strongly related to working with a ratio of 4.3:1. We identified MRI features of the cohort and memory task. compared it with those reported in other worldwide studies.

Conclusions Interpretation We found deficits in both domains of ToM in patients with This is the first study from Kuwait describing the radiological Parkinson's disease. Neurocognitve impairment characteristic for characteristics of NMOSD. We highlighted the similarities and patients was related to social – cognitive abilities. That's very differences of our cohort from other worldwide cohorts reported in important finding, because such non-motoric symptoms have even the literature. greater impact on patients´ quality of life than motoric symptoms, doi:10.1016/j.jns.2019.10.1239 even in the early stages of the disease.^