BRAIN MRI AND ELECTROPHYSIOLOGIC ABNORMALITIES IN PRECLINICAL AND CLINICAL ADRENOMYELONEUROPATHY

被引:56
作者
AUBOURG, P
ADAMSBAUM, C
LAVALLARDROUSSEAU, MC
LEMAITRE, A
BOUREAU, F
MAYER, M
KALIFA, G
机构
[1] HOP ST VINCENT DE PAUL, DEPT PEDIAT NEUROL, F-75674 PARIS 14, FRANCE
[2] HOP ST VINCENT DE PAUL, DEPT PEDIAT RADIOL, F-75674 PARIS 14, FRANCE
[3] HOP ST ANTOINE, DEPT NEUROPHYSIOL, F-75571 PARIS 12, FRANCE
关键词
D O I
10.1212/WNL.42.1.85
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We describe the neurophysiologic abnormalities in 17 patients with clinical adrenomyeloneuropathy (AMN), an adult phenotype of X-linked adrenoleukodystrophy (ALD). These abnormalities also allowed the early recognition of the AMN phenotype in three asymptomatic boys with biochemically proven ALD. In 14/16 adult AMN patients, there were cerebral white matter abnormalities on MRI which were severe and comparable with cerebral ALD in four of the patients.
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页码:85 / 91
页数:7
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