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[眼阵挛-肌阵挛综合征。临床及眼震电图检查结果与病程研究]

[Opsoclonus and myoclonus syndrome. Clinical and electronystagmography findings with course studies].

作者信息

Leopold H C

出版信息

Fortschr Neurol Psychiatr. 1985 Feb;53(2):42-54. doi: 10.1055/s-2007-1001952.

Abstract

This review is based on the clinical experience with 7 patients and a total of 171 additional cases described in literature. Clinical features are presented of this "benign" cerebellar encephalopathy, which occurs preferably in association with viral infections, neuroblastomas or rarely carcinomas. Numerous descriptions of single cases stress a heterogeneous variety of other diseases which possibly may cause this syndrome. Special emphasis is put on the electronystagmographic documentation of eye movements with follow up of the spontaneous recovery over several years. The eye movement characteristics of opsoclonus allow for a clear differentiation from other forms of acquired ocular oscillations, such as ocular flutter, ocular dysmetria, square wave jerks, macro-saccadicoscillations, lightning eye movements, pendular nystagmus, obliquus superior myokymia and voluntary nystagmus. Differential diagnosis of the underlying diseases is particularly dependent on the age of onset. Histopathological findings as well as the hitherto suggested immunologic pathogenesis, therapeutic regimen and the prognosis are discussed critically on the basis of the literature.

摘要

本综述基于对7例患者的临床经验以及文献中描述的另外171例病例。本文介绍了这种“良性”小脑性脑病的临床特征,其更常与病毒感染、神经母细胞瘤相关,或很少与癌相关。众多单病例描述强调了可能导致该综合征的其他多种不同疾病。特别强调了通过眼震电图记录眼球运动,并对数年的自发恢复情况进行随访。眼阵挛的眼球运动特征有助于与其他形式的后天性眼球震颤,如眼扑动、眼球辨距障碍、方波急跳、大扫视振荡、闪电样眼球运动、钟摆样眼球震颤、上斜肌肌阵挛和随意性眼球震颤进行明确区分。潜在疾病的鉴别诊断尤其取决于发病年龄。基于文献对组织病理学发现以及迄今提出的免疫发病机制、治疗方案和预后进行了批判性讨论。

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