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先天性及散发性小脑颗粒层萎缩伴继发性脑桥小脑变性(作者译)

[Congenital and sporadic atrophy of the granular layer of the cerebellum with secondary pontocerebellar degeneration (author's transl)].

作者信息

Lapresle J, Annabi A

出版信息

Rev Neurol (Paris). 1980;135(12):885-93.

PMID:7466115
Abstract

The primary interest of the case reported here of congenital atrophy of the granular layer of the cerebellum (Norman type) lies in the presence of an associated pontocerebellar degeneration, interpreted to be secondary (transsynaptic and retrograde) to the granular involvement and due to the length (42 years) of the evolution. Secondly, the sporadic character of this case is not in favor of a genetic disorder. By analogy with Herringham and Andrewes cat ataxia due to a perinatal virus infection (Margolis and Kilham), it can be hypothesized that granular cerebellar atrophy in man is related to an injury incurred at a "favorable" moment in histogenesis, that is, between the 3rd and 6th month of fetal life.

摘要

本文报道的先天性小脑颗粒层萎缩(诺曼型)病例的主要关注点在于存在相关的脑桥小脑变性,其被解释为继发于(跨突触和逆行性)颗粒层受累,并且是由于病程长达42年。其次,该病例的散发性特征不支持遗传性疾病。通过与因围产期病毒感染导致的赫林厄姆和安德鲁猫共济失调(马戈利斯和基勒姆)相类比,可以推测人类小脑颗粒层萎缩与在组织发生的“有利”时刻,即胎儿生命的第3至6个月之间遭受的损伤有关。

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