[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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