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成人未被重视的小脑发育不全:一名38岁男性的病例报告

Unappreciated agenesis of cerebellum in an adult: case report of a 38-year-old man.

作者信息

Leestma J E, Torres J V

机构信息

The Chicago Institute of Neurosurgery and Neuroresearch, Illinois 60614, USA.

出版信息

Am J Forensic Med Pathol. 2000 Jun;21(2):155-61. doi: 10.1097/00000433-200006000-00013.

Abstract

An unexpected finding at autopsy of almost complete agenesis of the cerebellum in an apparently functional, mentally subnormal 38-year-old man who died as the result of an accidental electrocution is reported. The posterior fossa was normal in appearance despite nearly complete absence of the cerebellum. A number of syndromes of cerebellar atrophy or dysgenesis have been reported, but congenital agenesis is considered a very rare condition. It does not resemble most common cerebellar malformations or acquired conditions, especially in an adult, who apparently had reasonable motor and coordinative function. The relevant literature is reviewed.

摘要

据报道,一名38岁男性因意外触电死亡,尸检时发现小脑几乎完全发育不全,而该男子生前功能正常但智力低于正常水平。尽管小脑几乎完全缺失,但后颅窝外观正常。此前已报道过一些小脑萎缩或发育异常的综合征,但先天性发育不全被认为是一种非常罕见的情况。它与大多数常见的小脑畸形或后天性疾病不同,尤其是在一名成年人身上,而该成年人显然具有合理的运动和协调功能。本文对相关文献进行了综述。

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