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平山病:肌萎缩侧索硬化症的一种罕见临床变异型

Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis.

作者信息

Aundhakar Swati C, Mahajan Sanket K, Chhapra Daanish A

机构信息

Department of Medicine, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India.

出版信息

Adv Biomed Res. 2017 Jul 28;6:95. doi: 10.4103/2277-9175.211797. eCollection 2017.

Abstract

Hirayama's disease is a rare clinical variant of amyotrophic lateral sclerosis where distal muscles are involved more compared to proximal muscles and vice-versa occurs only in 10% cases and so it is differentiated from O'Sullivan McLeod syndrome which involves only small muscles of single limb. Here, we present a case of Hirayama's disease where disease achieved a plateau after 3 years with no further progression. His electrophysiological studies, and clinical picture, and magnetic resonance imaging findings were consistent with a diagnosis of Hirayama's disease.

摘要

平山病是肌萎缩侧索硬化症的一种罕见临床变异型,其中远端肌肉比近端肌肉受累更多,反之仅在10%的病例中出现,因此它与仅累及单肢小肌肉的奥沙利文·麦克劳德综合征相鉴别。在此,我们报告一例平山病病例,该疾病在3年后达到平台期,未进一步进展。他的电生理研究、临床表现和磁共振成像结果均符合平山病的诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96a9/5549545/be058e810474/ABR-6-95-g001.jpg

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