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[X染色体连锁肾上腺脑白质营养不良的脑部磁共振成像]

[Cerebral MRT in x chromosome linked adrenoleukodystrophy].

作者信息

Kujat C, Halb A, Hermes M, Scherb T, Hadidi H, Henkes H, Dewes W

机构信息

Funktionsbereich Kernspintomographie, Universitätskliniken des Saarlandes, Homburg/Saar.

出版信息

Bildgebung. 1991;58(4):205-7.

PMID:1797249
Abstract

Adrenoleukodystrophy (ALD) is a rare lipid storage disease, characterized among other symptoms by a progressive demyelinization of white matter. Changes are well demonstrated by MRT: Leukencephalopathy begins symmetrically in the occipital lobes of the cerebrum and involves furthermore the splenium of the corpus callosum, the nucleus dentatus and the descending tracts. As neurologic signs may precede signs of adrenal insufficiency, MRT may be important to suspect the diagnosis of ALD, which generally is diagnosed by biochemical tests.

摘要

肾上腺脑白质营养不良(ALD)是一种罕见的脂质贮积病,其特征症状包括白质进行性脱髓鞘。磁共振成像(MRT)能很好地显示这些变化:脑白质病始于大脑枕叶,呈对称性,进而累及胼胝体压部、齿状核和下行束。由于神经症状可能先于肾上腺功能不全的症状出现,MRT对于怀疑ALD的诊断可能很重要,ALD通常通过生化检查来确诊。

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