《中国康复理论与实践》

《中国康复理论与实践》 ›› 2009, Vol. 15 ›› Issue (01): 21-23.

• 专题 • 上一篇    下一篇

12例平山病临床分析

邸军1a;李娜1b;李秋香1b;赵哲1b;沈宏锐1b;崔建岭1c;胡静1b   

  1. 1.河北医科大学第三医院,a.骨伤科;b.神经肌肉病科;c.放射科,河北石家庄市 050051
  • 收稿日期:2008-10-20 出版日期:2009-01-01 发布日期:2009-01-01
  • 通讯作者: 李娜

Hirayama Disease: 12 Cases Report

DI Jun，LI Na，LI Qiu-xiang，et al   

  1. Department of Orthopedics and Neuromuscular Disease，The Third Hospital of Hebei Medical University，Shijiazhuang 050051，Hebei，China
  • Received:2008-10-20 Published:2009-01-01 Online:2009-01-01

PDF (PC)

被引次数

14

摘要： 目的 分析平山病临床、电生理与颈MRI影像学特征。方法 回顾性分析12例平山病的临床、电生理、颈MRI和骨骼肌活检病理资料。结果和结论 患者均为男性，青春期发病，单/双侧上肢远端无力伴肌萎缩，无感觉障碍和锥体束征。肌电图以颈7-胸1支配区为中心呈神经源性异常。颈MRI示3例下颈髓轻度萎缩，屈颈位12例程度不等下颈髓受压扁平、前移，硬膜囊后间隙增宽，硬膜外新月形或条状高信号,部分可见血管流空影；增强扫描4例，新月形信号影有强化。肱二头肌活检3例，组化染色病理分析1例示轻度神经源性异常，2例正常。

关键词: 平山病, 肌电图, 核磁共振

Abstract: Objective To investigate the clinical, electrophysiological and magnetic resonance imaging (MRI) characteristics of Hirayama disease. Methods 12 cases with Hirayama Disease were analyzed retrospectively. Results and Conclusion All the patients were male, onset at adolescence, with muscle weakness and atrophy on one or two upper limbs, but without sensory disorder or pyramidal sign. The EMG showed neurogenic abnormalities in the areas dominated by C7-T1 nerve in all the patients. Cervical cord MRI found slight atrophy of lower cervical cord in 3 cases in routine position，compressed and displacement forward in flexion position in 12 cases，with posterior epidural space widening，lunular or striped high signal and voids of vessel in some patients. Enhancement scanning in 4 cases showed lunular sign enhanced. Biceps muscles biopsies were performed in 3 cases, the pathology Results appear neuropathic change in 1 cases, another 2 cases were normal.

Key words: Hirayama disease, electromyogram, magnetic resonance imaging