橄榄桥小脑萎缩和多系统萎缩  

Olivopontocerebellar atrophy and multiple system atrophy

作　　者：张振馨[1,2] 高宗恩[1,2] 王建明[1,2] 谭铭勋 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院神经科 [2]胜利油田中心医院神经内科

出　　处：《中华内科杂志》1998年第4期265-268,共4页Chinese Journal of Internal Medicine

摘　　要：目的探讨橄榄桥小脑萎缩（ＯＰＣＡ）的本质及与其他神经元变性病的关系。方法ＯＰＣＡ５２例，家族性５例，散发性４７例（其中单纯ＯＰＣＡ１２例，伴ＳｈｙＤｒａｇｅｒ综合征１７例，伴帕金森综合征８例，伴ＳｈｙＤｒａｇｅｒ综合征和帕金森综合征１０例）。比较分析其临床特点。结果发现ＯＰＣＡ和其他系统神经元萎缩的组合中，累及系统的种类和数量不一，不同系统受累的各种症状出现次序、频度、表现形式和严重度不同，但有一定的规律性。结论ＯＰＣＡ本质的不均一性。Objective In order to explore the entity of olivopontocerebellar atrophy (OPCA), the relationship of OPCA with progressive neurodegenerative diseases and the application of the term multiple system atrophy (MSA) clinically. Methods We studied the clinical features of 52 patients with OPCA, including 5 patients with familial OPCA (fOPCA) and 47 patients with sporadic OPCA (sOPCA). sOPCA consisted of “pure” OPCA (12 cases) and OPCA plus parkinsonism (8 cases), OPCA plus Shy Drager syndrome (17 cases) and OPCA plus parkinsonism as well as Shy Drager syndrome (10 cases), being characterized clinically by combinations of cerebellar, pyramidal, extrapyramidal and autonomic involvement. Results It is found that on clinical ground, sOPCA is distinct from fOPCA. The former is not uniform in the presence or absence of the additional signs of autonomic failure or and extrapyramidalsigns, but sOPCA is characterized by the show the regularity of encompassment of multiple system neurodegeneration in category, form, and order. Conclusion It is suggested that OPCA is a heterogeneous disorder, but the different neurodegenerative diseases included in this entity might share a common cause.

关 键 词：橄榄体 桥脑 小脑萎缩 病因 遗传性共济失调 

分 类 号：R744.702[医药卫生—神经病学与精神病学]