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[进行性小脑萎缩背景下的橄榄脑桥小脑萎缩(一项临床解剖学研究)]

[Olivopontocerebellar atrophy in the context of progressive cerebellar atrophies (a clinico-anatomical study)].

作者信息

Ionel C

机构信息

Clinica de Neurologie, Spital Colentina Bucuresti.

出版信息

Rev Med Interna Neurol Psihiatr Neurochir Dermatovenerol Neurol Psihiatr Neurochir. 1990 Jan-Mar;35(1):51-60.

PMID:2237007
Abstract

The study of 15 cases of progressive cerebellar atrophies, and especially of the olivopontocerebellar atrophy, that was investigated both clinically and anatomically, has attempted to evidence particularities and correlations existing between these two types of atrophy. Olivopontocerebellar atrophy appears to be an abiotrophy of the cerebellum, considered to be spontaneous, sporadic, and sometimes with a hereditary familial background. It is a systemic disease, predominantly of the neocerebellum and involving the cerebellopedal system. It is at the opposite end of Holmes-type atrophies, and of the cerebello-olivary atrophies of the young (I. T. Niculescu, Th. Hornet, 1936) which mainly involve the paleocerebellum. The disease has a polymorphous symptomatology, it has a slow, progressive evolution with mostly cerebellar signs, with extrapyramidal phenomena and psychical disturbances due to lesions of the telencephalic pathways, and sometimes of the spinal, cerebellar and bulbar proprioceptive afferences, with spinal and bulbar involvement (Cezar Ionel, 1949, 1972).

摘要

对15例进行性小脑萎缩病例,尤其是橄榄脑桥小脑萎缩病例进行了临床和解剖学研究,试图揭示这两种萎缩类型之间存在的特殊性和相关性。橄榄脑桥小脑萎缩似乎是小脑的一种营养障碍,被认为是自发的、散发性的,有时具有遗传家族背景。它是一种全身性疾病,主要累及新小脑并涉及小脑脚系统。它与霍姆斯型萎缩以及年轻人的小脑橄榄萎缩(I.T.尼古列斯库、Th.霍内特,1936年)处于相反的一端,后者主要累及旧小脑。该疾病具有多形性症状,呈缓慢、进行性发展,主要表现为小脑体征,伴有由于端脑通路病变引起的锥体外系现象和精神障碍,有时还伴有脊髓、小脑和延髓本体感觉传入障碍,伴有脊髓和延髓受累(塞扎尔·约内尔,1949年、1972年)。

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