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[影响中枢和周围神经系统的髓鞘疾病]

[Myelin diseases affecting both the central and the peripheral nervous system].

作者信息

Hagen K, Mellgren S I, Bovim G

机构信息

Nevrologisk avdeling, Norges teknisk-naturvitenskapelige universitet, Trondheim.

出版信息

Tidsskr Nor Laegeforen. 1998 Sep 30;118(23):3600-2.

PMID:9820004
Abstract

Myelin is synthesized by oligodendrocytes in the central nervous system and by Schwann cells in the peripheral nervous system. A combined dysmyelinating/demyelinating process in the central nervous system and in the peripheral nervous system occurs only rarely. A brief review of these conditions is given in this article. Occasionally, coexistence of multiple sclerosis and chronic inflammatory demyelinating polyneuropathy is reported. Cases of multiple sclerosis, hypertrophic polyneuropathy, and central nervous system demyelination in Miller-Fisher syndrome and Guillain-Barré syndrome have also been reported. Concurrent dysmyelination in the central nervous system and peripheral nerve demyelination are common in metachromatic leukodystrophy, Krabbe disease and adrenoleukodystrophy. A Norwegian family with adult-onset demyelinating disease affecting both the peripheral and central nervous system has recently been described. The mode of inheritance is probably autosomal dominant, but its exact nature remains unknown.

摘要

髓磷脂由中枢神经系统中的少突胶质细胞和周围神经系统中的施万细胞合成。中枢神经系统和周围神经系统联合出现的髓鞘形成异常/脱髓鞘过程极为罕见。本文对这些病症进行简要综述。偶尔会有关于多发性硬化症与慢性炎症性脱髓鞘性多发性神经病并存的报道。也有关于米勒-费希尔综合征和吉兰-巴雷综合征中多发性硬化症、肥厚性多发性神经病及中枢神经系统脱髓鞘病例的报道。在异染性脑白质营养不良、克拉伯病和肾上腺脑白质营养不良中,中枢神经系统同时存在髓鞘形成异常和周围神经脱髓鞘的情况很常见。最近描述了一个挪威家族,其成年期发病的脱髓鞘疾病同时累及周围和中枢神经系统。遗传方式可能为常染色体显性遗传,但其确切性质仍不清楚。

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