JNS-14028; No. of Pages 40 ARTICLE IN PRESS Journal of the Neurological Sciences (2015) xxx–xxx Contents lists available at SciVerse ScienceDirect Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns Movement Disorders 834 of the disease, the absence of segregation in many families so far WFN15-1473 described, and the evidence of expansions also in healthy subjects. Movement Disorders Objective: Here we describe two members of a large Italian family Effects of zonisamide for overall non-motor symptoms with (Fig. 1), second cousins once removed, with a peculiar disease Parkinson’s disease patients phenotype. Patients: The first patient (red arrow) was a 55-years-old man M. Abe, M. Koga, M. Kawai, T. Kanda. Clinical Neuroscience and Neurology, Yamaguchi University School of Medicine, Yamaguchi, Japan whose father suffered from a late-onset very mild ataxia. He went to our attention because of a 20-year-history of slowly progressive Background: Non-motor symptoms in Parkinson's disease (PD) are cerebellar ataxia, L-dopa-responsive parkinsonism, and orthostatic fi considered as an important factor related to the quality of life in PD hypotension. The second patient (black arrow) had speech dif cul- patients. However, treatment strategies for these symptoms have yet ties and gait disturbance from the age of 17 years, and disabling been established. Zonisamide was reported to improve motor paroxysmal myoclonic tremor of the head at the age of 19 years. functions of PD and have various effects on nervous systems. The Neurophysiological evaluation excluded a cortical origin of the present study investigated the efficacy of zonisamide in non-motor tremor. Cerebellar atrophy was present on MRI in both patients. Further ten membersPROOF of the family have been reported to be affected symptoms of PD. Methods: 20 patients with PD participated in this study and were with cerebellar ataxia. treated with zonisamide (25 mg/day) for 12 weeks. When subjects Results: Molecular genetic analysis excluded SCA1-2-3-6-7-10-12- entered in this study, written informed consent was acquired from 17-36 and DRPLA and demonstrated a SCA8 pathological expansion. each subject. General symptoms were evaluated using the Unified PD Conclusion: The segregation between the disorder and the CTA/CTG Rating Scale (UPDRS). Non-motor symptoms were measured by Non expansion in so distant relatives supports the pathological role of fi Motor Symptom Scale (NMSS), which consists of 9 subdomains. The SCA8 mutation. Our description con rms intrafamilial heterogeneity study protocol got the approval of the Institutional Review Board. of clinical features. Results: Median UPDRS part III score significantly decreased from 25.5 points at baseline to 20.6 points at 12 weeks (p = 0.004). In addition, NMSS total score showed significant improvement from 55.9 +/- 51.6 to 44.3 +/- 50.4 (p = 0.044). Within each NMSS subdomain, significant amelioration was observed in mood/cognition (from 11.9 +/- 18.0 to 7.3 +/- 12.8, p = 0.020) and attention/ memory (from 7.3 +/- 8.9 to 4.8 +/- 7.6, p = 0.021) domains. Conclusions: This study suggests that zonisamide improves non- motor symptoms, especially psychical and cognitive symptoms in PD patients. RRECTEDdoi:10.1016/j.jns.2015.08.903 doi:10.1016/j.jns.2015.08.902 839 836 WFN15-0362 WFN15-0696 Movement Disorders Movement Disorders Polineuropathy and B12 deficiency in levodopa/carbidopa Intrafamilial phenotypic variability in Spinocerebellar ataxia type 8 intestinal gel – etiology and management -our experience A. Antenoraa,S.Pelusoa,M.Espositoa,A.DeRosaa,A.Bruscob,A.Fillaa,G. C. Baetua, I. Buragaa, M. Buragab, V. Petrea. aNeurology, Colentina De Michelea. aDepartment of Neurosciences Reproductive and Hospital, Bucharest, Romania; bPhisiology, Carol Davila University of Odontostomatological Sciences, University of Naples Federico II, Naples, Medicine and Pharmacy Bucharest, Bucharest, Romania b UNCO Italy; Department of Medical Sciences, University of Torino, Torino, Italy Background: Duodenal infusion of levodopa/carbidopa intestinal Background: Spinocerebellar ataxia type 8 (SCA8) is an autosomal gel(LCIG) is an effective treatment option for advanced Parkinson's dominant ataxia related to expanded CTA/CTG triplet repeats on disease(PD). chromosome 13q21. The pathological role of these expansions is We found polyneuropathy in a number of patients treated with currently debated because of the genetic and clinical heterogeneity LCIG (DUODOPA). 0022-510X/$ – see front matter. ARTICLE IN PRESS 2 Abstracts / Journal of the Neurological Sciences (2015) xxx–xxx Is this a side effect of therapy or is independent from neurologic 841 disease? WFN15-0807 We have 28 patients treated with Duodopa since January2012;an Movement Disorders important percent of them developed polyneuropathy after initiating Hemodynamics in patients with idiopathic Parkinson’s disease this therapy. J. Baika,H.Mab. aNeurology, Sanggye Paik Hospital Inje University, Seoul, Objective: To determine the etiologic factors and the correct b Korea; Neurology, Sacred Heart Hospital Hallym University, Anyang, Korea management in order to prevent this affection. Material and methods: We studied 28 patients with advanced PD in Background: Arterial stiffness is an independent predictor of all-cause treatment with LCIG;They were examined by EMG(Electromyography) and cardiovascular mortality, fatal and nonfatal coronary events, and serum level of B12 before treatment and 6 and 12 months after Duodopa symptomatic strokes. The aim of the present study was to explore the treatment. The objectives of this study were to evaluate the risk of arterial stiffness by measurement of the augmentation index (AIx) in neuropathy in patients with Parkinson's disease and to evaluate the role subjects with Parkinson’s disease and to evaluate the relationship of levodopa exposure as a potential risk factor. between these indexes and other clinical and laboratory variables. Results: We found some cases of severe sensorimotor poly- Methods: AIx was measured by applanation tonometry (SphygmoCor, neuropathy with both subacute and chronic onsets, rarely associated Atcor, Australia). Applanation tonometry was used to record the radial with vitamin B12 imbalance. We did not observed severe changes in artery pressure waveform continuously, and mean values of the 2 fl vitamin B12 level. This may re ect a rare complication or a severe screens of pulse waves of good quality were used for analysis. side effect. Results: Forty patients with Parkinson’s disease and 90 control Conclusion: We describe 28 PD patientswho developed axonal subjects were enrolled for the study. The mean age was 66.4 years. fi polyneuropathy and vitamin B(12) de ciency. Wereview the potential The baseline characteristics were well balanced among the groups. etiologic factors, and discuss about the algorithm for themanagement While there were no significant differences among the groups for and prevention of this simptomatology. Our research suggest a hemodynamic indices, peripheral PP and aortic PP were significantly relationshipbetween levodopa/carbidopa intestinal gel and PN. lower in IPD group than control group. (P = 0.031, P = 0.001). fi Keywords: Parkinson disease,neuropathy ,B12 de ciency According to the univariate analysis, AIx was significantly correlated doi:10.1016/j.jns.2015.08.904 with aortic pulse pressure (r = 0.324; P b 0.001) and peripheral pulse pressure (r = 0.193; P = 0.025). Conclusion: In this study we asked whether there is a difference in 840 terms of hemodynamicPROOF indexes between subjects with Parkinson’s WFN15-0371 disease and control. From this evidence of lower peripheral and Movement Disorders aortic PP in IPD group, we conclude that subjects with Parkinson’s Complications related to levodopa/carbidopa intestinal gel disease could have a lower risk of developing cardiovascular event. treatment –colentina hospital experience C. Baetua, I. Buragaa, M. Buragab, G. Mihailescua, V. Petrea. aNeurology, doi:10.1016/j.jns.2015.08.906 Colentina Hospital, Bucharest, Romania; bPhisiology, Carol Davila University of Medicine and Pharmacy Bucharest, Bucharest, Romania 842 Background: Enterally administered levodopa/carbidopa gel WFN15-1496 (Duodopa)(LCIG) is used for the treatment of advanced Parkinson's Movement Disorders disease (PD) in patients with motor fluctuations and dyskinesias. Post ischemic stroke status dystonicus, a case report However, it is not free of complications. I. Barcellos, E.D. Padovani, A.D. Carpiné, B.C. Elamide, L. Filla, C.A. Our clinic started to use LCIG in December 2012.So far we have Twardowschy. aNeurology, Hospital Universitário Cajuru, Curitiba, Brazil treated 28 patients. Although generally considered safe, PEG (percutaneous endo- Introduction: Status Dystonicus (SD) is a life-threatening rare scopic gastrostomy ) tube placement can be associated with many movement disorder characterized by episodes of frequent or potential complications. This paper describes a variety of PEG tube continuous severe generalized dystonic spasms. It’s considered a related complications and strategies for complication avoidance. underdiagnosed entity and it treatment remains a challenge. Objective: - to report complications that we observedRRECTED in our patients Case report: A 44-years old woman was admitted with a 6-hour history with advanced Parkinson's disease treated with continuous infusion of of loss of consciousness and generalized stiffness. She had a previous intraduodenal levodopa/carbidopa(LCIG)