TY -的T1 -成人Opsoclonus-Myoclonus综合症(P04.045) JF -神经学乔-神经病学SP - P04.045 LP首页 - P04.045詹姆斯六世- 78 - 1补充AU -克洛斯盟Rajeev Kumar AU - j . Ahlskog盟约瑟夫松本AU -肖恩Pittock AU -万带兰列侬AU - j . d . Bartleson盟凯萨琳McEvoy AU -艾伦Aksamit盟安德鲁·麦肯Y1 - 2012/04/25 UR - //www.ez-admanager.com/content/78/1_Supplement/P04.045.abstract N2 -目的:描述成人Opsoclonus-Myoclonus综合症的临床过程和结果(OMS)。背景OMS是幼儿认识到自身免疫性疾病,常与神经母细胞瘤。成人OMS的描述是有限的。设计/方法:回顾性研究(1990 - 2011),电话面试和血清学鉴定的梅奥Clinic-Rochester成人OMS患者(考试结果局限于opsoclonus-myoclonus;发作时期≥18年)。结果:OMS被确定在21个成年人(女性,11)。发作时期中值为47岁(范围,27 - 78);平均随访,43个月(范围1 - 187)。初期症状包括:头晕,14;平衡困难(肌阵挛所致),14;恶心和/或呕吐、10; vision abnormalities (caused by opsoclonus), 6; tremor/tremulousness, 4. Myoclonus distributions: extremities, 15 patients (upper and lower, 13; upper-only, 1; lower-only, 1) craniocervical, 8 (hyperkinetic dysarthria, 5) and trunk, 4. Extensive oncological evaluations documented carcinoma in 3 patients (breast, 2; small-cell lung, 1); none had a paraneoplastic antibody identified. A flu-like prodrome was reported in 6 patients; no infectious etiology was identified. Immunotherapies were initiated in 18 patients (median of 1-month post-symptom-onset; continued for a median of 2-weeks; range, 1-52): corticosteroids and IVIg, 10; steroids or IVIg, 4; steroids and plasmapheresis, 2; steroids, IVIg and plasmapheresis, 2; mycophenolate, 1. Outcomes at ≤1 month: improvement with immunotherapy, 16 (symptomatic therapies [usually clonazepam] were additionally helpful in 14); rapid neurological decline and death, 2 (neither had cancer identified). A further patient received clonazepam only and improved. There was no follow-up for the remaining two patients. Outcomes among 16 patients with ≥6 months surveillance were: neurological remission, 12 (2 relapsed early, requiring ≥ 6 months immunotherapy); mild symptoms, 2 (1 had breast cancer); minimal improvement, 1 (symptom-onset to diagnosis duration, 8 months); death from small-cell carcinoma, 1 (no neurological follow-up).Conclusions: Adult-onset OMS was uncommon, and a cause rarely identified. Cancer should be excluded. Early (and extended if necessary) immunotherapy may facilitate optimal outcomes.Disclosure: Dr. Klaas has nothing to disclose. Dr. Kumar has received personal compensation for activities with Novartis, Glaxo-Smith-Klein, Inc., Teva Neuroscience, Lundbeck Research USA, Inc., Boehringer-Ingelheim Pharmaceuticals, Inc., Allergan, Inc. and Appied Magnetics as a speaker and/or consultant. Dr. Ahlskog has nothing to disclose. Dr. Matsumoto has nothing to disclose. Dr. Pittock has received research support from Alexion Pharmaceuticals, Inc. Dr. Lennon has nothing to disclose. Dr. Bartleson has nothing to disclose. Dr. McEvoy has nothing to disclose. Dr. Aksamit has nothing to disclose. Dr. McKeon has nothing to disclose.Wednesday, April 25 2012, 07:30 am-12:00 pm ER -