摘要
目的 探讨青少年上肢远端肌萎缩症的临床解剖及MRI特点,为该病的早期诊断和治疗提供依据.方法 对13例确诊的青少年上肢远端肌萎缩症患者的临床表现、临床解剖、神经电生理特征及影像学资料进行回顾性分析.结果 青少年上肢远端肌萎缩症平均发病年龄18.6岁,表现为局限于手和前臂的肌萎缩,前臂呈斜坡样;肌电图显示患者双侧上肢远端肌肉均呈神经源性损害,受损节段多在下颈髓前角细胞;屈颈MRI均见下段颈髓前移、变平,硬脊膜向前移位,硬脊膜外间隙增宽,硬脊膜外间隙内半月形信号影;与正常自愿者比较,在C6椎体上沿水平,MRI自然位和前屈位可显示出青少年上肢远端肌萎缩症患者脊髓前后径明显缩小(P＜0.05). 结论 青少年上肢远端肌萎缩症可能为下颈段脊髓病变,神经电生理及影像学检查有助于其早期临床诊断与鉴别诊断,患者应早期佩带颈托和避免长时间屈颈位.
Abstract
Objective To study clinical characteristics of juvenile muscular atrophy of the distal upper extremity, for early diagnosis and treatment. Methods The clinical data, neuro-electrophysiological features, clinical anatomy and MRI features of 13 patients diagnosed by the criteria of Hirayama Disease were retrospectively analyzed. Results The mean age of onset was about 18.6 years old. Hirayama disease was characterized by muscular atrophy in the hand and forearm. The brachioradialis was spared (oblique amyotrophy). EMG indicated that the impairment of spinal anterior cells was limited to the arm relevant segments and both sides were involved. During neck flexion, the spinal cord was placed forward and flattened. The diameter of cervical spine cord in the sixth cervical vertebra along the horizontal in juvenile muscular atrophy of the distal upper extremity was smaller than that in normal volunteers significantly (P<0.05). Conclusions Juvenile muscular atrophy of the distal upper extremity may be a special type of cervical myelopathy. The neuro-electrophysiological check and the neck flexion MRI check are valuable to the clinical diagnosis and differential diagnosis. Cervical collar therapy is recommended the sooner the better and avoiding long time neck flexion position is very important for the patients.
NETL
NSTL
维普
万方数据