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[成人异染性脑白质营养不良]

[Adult metachromatic leukodystrophy].

作者信息

Wang L

机构信息

Dept of Neuropatholog in PLA General Hospital Beijing.

出版信息

Zhonghua Shen Jing Jing Shen Ke Za Zhi. 1990 Dec;23(6):354-5, 385.

PMID:2098247
Abstract

An adult case of metachromatic leukodystrophy confirmed by characteristic findings of the brain and superficial sural nerve biopsies, but with absence of deficiency of arylsulfatase A activity in the leucocytes, was reported. Ultrastructurally, typical membrane-bound inclusions were found in white matter and Schwann cells. The long course of thirty years and late onset of illness were discussed.

摘要

报告了一例成人异染性脑白质营养不良病例,经脑和腓肠神经活检的特征性发现确诊,但白细胞中芳基硫酸酯酶A活性无缺乏。超微结构上,在白质和施万细胞中发现了典型的膜结合包涵体。讨论了三十年的病程和疾病的晚发情况。

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