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13个月女童的纤维蛋白样脑白质营养不良(亚历山大病)。病例报告。

Fibrinoidal leucodystrophy (Alexander's disease) at 13 months girl. A case report.

作者信息

Marszał E, Kałuza J, Adamek D, Jamroz E

机构信息

Department of Neurology Second Pediatrics Clinic of Silesian Medical Academy, Katowice.

出版信息

Patol Pol. 1992;43(4):193-5.

PMID:1287550
Abstract

The case of Alexander's fibrinoidal leucodystrophy that was presented below is an exemplar of extremely rare degenerative disease of the CNS. On the grounds of the clinical course of the disease it seems that our case can be reckoned as an early childhood form of Alexander's disease. An interesting difference that pays attention is the marked hydrocephalus. In the most cases of Alexander's disease the volume of ventricular system is normal however most of authors expresses that it sometimes can be slightly and insignificantly enlarged.

摘要

以下所呈现的亚历山大纤维样脑白质营养不良病例是中枢神经系统极为罕见的退行性疾病的一个典型例子。基于该疾病的临床病程,我们的病例似乎可被视为亚历山大病的幼儿型。一个值得关注的有趣差异是明显的脑积水。在大多数亚历山大病病例中,脑室系统的容积是正常的,然而大多数作者表示,它有时可能会轻微且不显著地扩大。

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