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目的探讨单肢肌萎缩(MMA)/平山病患者的临床、肌电图及颈椎MRI特征。方法 2009年5月至2014年5月就诊本科,符合诊断标准的患者,连续登记并详细记录及分析人口学资料、临床、电生理和颈椎自然位和屈曲位MRI资料。结果 (1)共41例,男39例、女2例,发病年龄14~24岁、平均年龄(16.87±2.62)岁。病程1~121月、平均病程(22.13±26.25)月。双上肢均有临床症状者6例(14.6%),症状局限单侧者35例(85.4%);单侧者左12例(29.3%)、右23例(56.1%)。冷麻痹22例,指震颤9例,手麻木4例;41例均有手固有肌萎缩,均无感觉障碍;(2)症状侧尺和正中神经运动潜伏期延长,小指展肌、拇短展肌运动波幅减低,小指展肌/拇短展肌波幅比值减小;(3)症状侧针肌电图显示异常自发活动者的出现率,在第一骨间肌和小指展肌为100%、拇短伸肌90.1%、拇短展肌86.3%、肱桡肌16.8%、肱二头肌13.8%;在仅限于单侧症状的35例患者中,无症状侧手固有肌也显示异常自发活动者占51.4%;(4)32例患者完成颈椎MRI检查。自然位时32例均显示颈2-颈7椎体后方下缘连线与椎体相交;均显示下段颈髓萎缩变扁平,其中位于C5-C7节段14例,C5-C6节段6例,C6-C7节段7例,C5-T1节段5例;屈颈位时15例显示硬膜腔后壁前移,移位的硬膜后方可见硬膜外占位,内有流空信号,恢复自然位后占位消失。9例显示髓内可疑T_2异常高信号。结论 MMA/平山病主要见于青少年男性;电生理表现为低位颈髓前角细胞病变,且无症状侧可显示临床下神经源性损害;小指展肌/拇短展肌波幅比值减小,是有鉴别意义的电生理指标;屈颈位颈椎MRI对于诊断很重要。结合临床、神经电生理及影像表现,有助于更全面认识本病。
Objective To investigate the clinical, electromyographic and cervical MRI features of patients with mono-muscular atrophy (MMA) / psoriasis. Methods From May 2009 to May 2014, patients who met the diagnostic criteria were enrolled and recorded in detail. Demographic data, clinical, electrophysiological and cervical natural-flexion and MRI data of flexion were recorded and analyzed in detail. Results (1) A total of 41 cases, 39 males and 2 females, the age of 14 to 24 years old, mean age (16.87 ± 2.62) years. Duration of 1 ~ 121 months, the average duration (22.13 ± 26.25) months. There were 6 cases (14.6%) with clinical symptoms in both upper extremities and 35 cases (85.4%) with unilateral unilateral symptoms. Left unilateral patients were 12 (29.3%) and right (23 cases) were 56.1%. Cold paralysis in 22 cases, finger tremor in 9 cases, 4 cases of numbness in hand; 41 cases of hands have muscular atrophy, no sensory disturbances; (2) Symptoms of lateral and median nerve motor latency, the little finger abductor muscle, (3) The EMG of symptomatic side showed that the incidence of abnormal spontaneous activity in the first interosseous muscle and little finger muscle was 100%, thumb 90.1% of short extensor, 86.3% of abductor hallucis, 16.8% of brachioradialis, 13.8% of biceps; in 35 patients who were restricted to unilateral symptoms, asymptomatic lateral muscularis propria also showed abnormal spontaneous activity Accounting for 51.4%; (4) 32 patients completed cervical MRI examination. In the natural position, 32 cases showed the connection of the lower edge of the cervical 2-neck 7 posterior vertebra and the vertebral body. Both showed atrophy and flattening of the lower cervical spinal cord, including 14 cases in C5-C7 segment and 6 in C5-C6 segment , C6-C7 segment in 7 cases, C5-T1 segment in 5 cases; 15 cases of flexion-neck position showed the dural wall anterior dural displacement can be seen after the epidural space, there is a flow empty signal After the restoration of natural bits occupy disappear. 9 cases showed intramedullary suspicious T 2 abnormal high signal. Conclusions MMA / Pyeongshan disease is mainly found in adolescent males. Electrophysiological findings are low anterior cervical spinal cord lesions and asymptomatic side can show clinical neurogenic damage. The amplitude ratio of abductor lessees exhibits a decrease in amplitude Identify the significance of electrophysiological indicators; cervical flexion cervical MRI is very important for the diagnosis. Combined with clinical, neuroelectrophysiological and imaging findings, contribute to a more comprehensive understanding of the disease.