Neurology:强直性肌营养不良1型

2018-10-27 zyx整理 神经科病例撷英拾粹

51岁女性,表现为进行性远端肌无力和肌萎缩2年。临床和电生理提示肌强直。 MRI可见整个白质受累的双侧散在和融合性异常高信号,主要累及前颞叶和外囊

51岁女性,表现为进行性远端肌无力和肌萎缩2年。临床和电生理提示肌强直。 MRI可见整个白质受累的双侧散在和融合性异常高信号,主要累及前颞叶和外囊(图)。DMPK基因检测提示1个等位基因中超过50个CTG重复,证实为强直性肌营养不良1型(DM1)的诊断。 DM1是见于成人的最常见肌营养不良。尽管晚期DM1,CADASIL和线粒体肌病的MRI可能相似,但每种疾病的临床病史都有很大差异。


(图:A:T2/FLAIR:双侧外囊高信号[箭头];B:T2/FLAIR:前颞叶白质对称融合性高信号[箭头];C-D:T1增强:未见病灶强化)

原始出处:Zhang B1, Yang Y1, Wu H1, et al. Teaching NeuroImages: Myotonic dystrophy type 1. Neurology. 2018 Sep 25;91(13):e1269-e1270. 

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    2018-12-14 yinhl1978
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    2018-10-31 zywlvao
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    2018-10-27 183****7028

    学习

    0

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