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肾上腺脑白质营养不良症,一种动态进展性疾病:两例病例的脑磁共振成像

Adrenomyeloneuropathy, a dynamic progressive disorder: brain magnetic resonance imaging of two cases.

作者信息

Mo Yuan-Heng, Chen Ya-Fang, Liu Hon-Man

机构信息

Department of Medical Imaging, National Taiwan University Hospital, 7 Chung-Shan South Road, 100 Taipei, Taiwan.

出版信息

Neuroradiology. 2004 Apr;46(4):296-300. doi: 10.1007/s00234-003-1096-8. Epub 2004 Mar 4.

Abstract

Adrenomyeloneuropathy (AMN) is a phenotype variant of X-linked adrenoleukodystrophy. We present two patients with adult-onset AMN who were initially suspected to have demyelinating disorders radiologically and finally diagnosed on the basis of laboratory data. The brain magnetic resonance images showed abnormal signal intensity at pyramidal tracts and cerebellar hemisphere bilaterally with abnormal enhancement after contrast medium administration. Review of the literature shows that the brain magnetic resonance findings of adrenomyeloneuropathy may include normal brain, tract demyelination, white matter demyelination, or brain atrophy. Disease progression was demonstrated by follow-up imaging.

摘要

肾上腺脑白质营养不良神经病(AMN)是X连锁肾上腺脑白质营养不良的一种表型变异。我们报告了两名成年发病的AMN患者,他们最初在影像学上被怀疑患有脱髓鞘疾病,最终根据实验室数据得以确诊。脑部磁共振成像显示双侧锥体束和小脑半球信号强度异常,注射造影剂后有异常强化。文献回顾表明,肾上腺脑白质营养不良神经病的脑部磁共振成像表现可能包括脑部正常、传导束脱髓鞘、白质脱髓鞘或脑萎缩。随访影像学检查证实了疾病的进展。

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