成人Opsoclonus-Myoclonus综合征(P04.045)

文摘

摘要目的:描述成人opsoclonus-myoclonus综合症的临床过程和结果(OMS)。

背景OMS是公认的在儿童自身免疫性疾病,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),电话采访和血清学评价的梅奥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;视觉异常(斜视眼阵挛所致),6;地震/发抖,4。肌阵挛分布:四肢,15例(上部和下部,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).

结论:成人OMS少见,很少了。癌症应该被排除在外。早期(必要时和扩展)免疫治疗可能促进最佳的结果。

披露:克洛斯博士没有披露。Kumar博士已经与诺华收到个人补偿活动,Glaxo-Smith-Klein, Inc . Teva神经科学,Lundbeck公司研究它是一家美国公司,勃林格殷格翰集团制药、Inc .)、爱力根公司和应用磁学作为一个演讲者和/或顾问。Ahlskog博士没有披露。松本博士没有披露。Pittock博士已经收到Alexion制药研究支持,公司列侬博士没有披露。Bartleson博士没有披露。McEvoy博士没有披露。Aksamit博士没有披露。麦克科恩博士没有披露。