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罕见脱髓鞘疾病。一种弥漫性播散性硬化症。

Unusual demyelinating disease. A form of diffuse-disseminated sclerosis.

作者信息

Tanaka J, Garcia J H, Khurana R, Kamijyo Y, Viloria J E

出版信息

Neurology. 1975 Jun;25(6):588-93. doi: 10.1212/wnl.25.6.588.

Abstract

A young man, in good health until the age of 22, developed progressive personality changes, lethargy, motor diffuculities, urinary bladder dysfunction, and convulsions. Spinal fluid abnormalities included monocytic pleocytosis and selective increase of gamma globulins. The clinical features and the structural lesions in the central nervous system are reminiscent of disseminated-diffuse sclerosis. Oligodendrocytes appeared normal in number and showed nonspecific abnormalities. Comparison of the structural lesions found in this case with those described in seven reports of demyelination indicates that the separation of this syndrome, as a distinct entity, may not be justified. We suggest that such cases be classified as myelinoclastic disorders, in the same category with multiple sclerosis and its variants.

摘要

一名22岁前身体健康的年轻男子出现了进行性人格改变、嗜睡、运动障碍、膀胱功能障碍和惊厥。脑脊液异常包括单核细胞增多和γ球蛋白选择性增加。中枢神经系统的临床特征和结构病变使人联想到播散性弥漫性硬化症。少突胶质细胞数量看起来正常,但有非特异性异常。将该病例中发现的结构病变与七篇脱髓鞘报告中描述的病变进行比较表明,将这种综合征作为一个独特的实体区分开来可能没有道理。我们建议将此类病例归类为髓鞘破坏障碍,与多发性硬化症及其变体属于同一类别。

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