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成人异染性脑白质营养不良一例中包涵体物质的电子显微镜研究;肾脏活检、周围神经和脑白质的观察结果

Electron microscopic investigation of inclusion material in a case of adult metachromatic leukodystrophy; observations on kidney biopsy, peripheral nerve and cerebral white matter.

作者信息

Joosten E, Hoes M, Gabreëls-Festen A, Hommes O, Schuurmans Stekhoven H, Slooff J L

出版信息

Acta Neuropathol. 1975 Dec 8;33(2):165-71. doi: 10.1007/BF00687542.

Abstract

The fine structural characteristics of storage products in peripheral nerve, kidney and cerebral white matter, from a case of adult metachromatic leukodystrophy are described. There were pronounced differences from the fine structural aspects in late infantile cases. A large proportion of the inclusions did not exhibit a unit membrane. An hypothesis is proposed to clarify the delayed manifestation of this type of metachromatic leukodystrophy until childhood.

摘要

本文描述了一例成人异染性脑白质营养不良患者外周神经、肾脏和脑白质中储存产物的精细结构特征。与晚期婴儿型病例的精细结构方面存在明显差异。大部分包涵体未显示单位膜。提出了一个假说来解释这种类型的异染性脑白质营养不良在儿童期之前延迟出现的表现。

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