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肾上腺脑白质营养不良的磁共振成像

MR imaging of adrenoleukodystrophy.

作者信息

Uchiyama M, Hata Y, Tada S

机构信息

Department of Radiology, Jikei University School of Medicine, Tokyo, Japan.

出版信息

Neuroradiology. 1991;33(1):25-9. doi: 10.1007/BF00593329.

Abstract

We performed MRI in 7 cases with adrenoleukodystrophy (ALD), including 3 cases with childhood ALD, 3 cases with adrenomyeloneuropathy (AMN) and one symptomatic heterozygote. The symptomatic heterozygote was the mother of the patient with AMN. High-field-strength (2.0T) MRI was used in two cases with childhood ALD. In all 3 cases of childhood ALD, MR showed widespread lesions in both cerebral hemispheres. Areas of low intensity in the diencephalon or striatum on heavily T2-weighted images at 2.0T were seen in 2 cases with childhood ALD. Cerebral lesions confined to the internal capsule, cerebral peduncle and optic radiation in the adult onset ALD including AMN and symptomatic heterozygote, have not been reported and this confinement may indicate an early stage of the disease.

摘要

我们对7例肾上腺脑白质营养不良(ALD)患者进行了磁共振成像(MRI)检查,其中包括3例儿童型ALD、3例肾上腺脊髓神经病(AMN)和1例有症状的杂合子。该有症状的杂合子是AMN患者的母亲。2例儿童型ALD患者使用了高场强(2.0T)MRI。在所有3例儿童型ALD中,磁共振成像显示双侧大脑半球有广泛病变。2例儿童型ALD患者在2.0T的重T2加权图像上可见间脑或纹状体的低强度区域。在包括AMN和有症状杂合子的成人型ALD中,局限于内囊、大脑脚和视辐射的脑部病变尚未见报道,这种局限性可能表明疾病处于早期阶段。

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