脾
磁共振成像
皮质脊髓束
医学
高强度
病理
肾上腺脑白质营养不良
白质
病变
放射科
内科学
磁共振弥散成像
过氧化物酶体
受体
作者
Florian Eichler,Asif Mahmood,D. J. Loes,Lena Bezman,Doris Lin,Hugo W. Moser,Gerald V. Raymond
出处
期刊:Archives of neurology
[American Medical Association]
日期:2007-05-01
卷期号:64 (5): 659-659
被引量:56
标识
DOI:10.1001/archneur.64.5.659
摘要
Background
An inherited disorder, X-linked adrenoleukodystrophy (X-ALD) is known to cause progressive inflammatory demyelination. Objective
To analyze the adult pattern of disease progression in X-ALD. Design, Setting, and Patients
We retrospectively assessed magnetic resonance (MR) images obtained in adult patients who had developed cerebral disease between January 1, 1985, and December 31, 2005. We identified 103 adult patients with X-ALD with lesions on their MR images. Of these, 56 had serial MR examinations at least 1 year apart and were included in this study. Main Outcome Measure
Progression of X-ALD lesions on MR images. Results
On initial presentation, 17 patients with X-ALD had corticospinal tract lesions without splenium or genu involvement, 24 had symmetric corticospinal tract lesions with additional involvement of the splenium or genu, and 15 did not have corticospinal tract involvement but had other white matter lesions. In 18 of 21 patients with progressive lesions, corticospinal tract involvement preceded or occurred concurrently with progressive inflammatory demyelination. Conclusions
Brain MR imaging abnormalities in adults with X-ALD progress slower than those reported in childhood. The involvement of the corticospinal tracts is prominent and may at times represent a variant course of progressive inflammatory demyelination.
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