52例肌萎缩侧索硬化症的临床及肌电图分析

目的：探讨肌萎缩侧索硬化（ALS）的临床表现及肌电图（EMG）特点，企为早期诊断提供依据。方法：回顾性研究近3年来收治的52例ALS患者的一般资料、临床表现、EMG及其它实验室检查，分析其发病特点、临床特征及EMG特点。结果：50例为散发病例，2例有ALS家族史。男性36例，女性16例，平均发病年龄54．67岁，平均病程13．69个月。起病形式均为缓慢起病，逐渐加重。几乎均以肢体肌无力、肌萎缩为首发症状，大部分患者肢体远端症状重于近端。主要临床表现为肢体肌和舌肌无力、萎缩及肌束震颤、锥体束征、构音障碍、呛咳及吞咽困难。无感觉障碍。EMG表现为广泛神经原性异常。颈椎磁共振（MRI）示74％的患者合并颈椎病。结论：ALS的诊断主要依靠临床表现及EMG检查。EMG、MRI及一些血液学检查是诊断及鉴别诊断该病的重要辅助检查手段。

Analysis of clinical and EMG in 52 patients with amyotrophic lateral sclerosis

Objective： To analyze the clinical manifestations and EMG characteristics of patients with amyotrophic lateral sclerosis（ALS） and provide the data for the early and accurate diagnosis. Methods..The records of the clnical information in 52 patients with ALS during recent 3 years were retrospectively reviewed. The clnical characteristics and data of EMG and laboratory examination were analysed. Results.. All cases developed sporadically. , with 36 males and 16 femals. The mean onset age was 54. 67 years. The mean course of disease was 13.69 months. The onset of all cases was chronic and progressive. The common onset initial symptoms were muscle weakness and atrophy in limbs. The symptoms of dista limbs were more serious than those of proximal limbs. Cranial nerve nucleus were commonly involved, most common in the hypoglossal nucleus and nerve nucleus. The main clinical manifestations were that limb and tongue muscle weakness, atrophy and fasciculation, pyramidal sign, dysarthria, choking and difficulty swallowing. No sensory disturbances occurred. EMG showed wide region neurogenic abnormal- ities. Some patients with cervical spine MRI showed cervical spondylosis. Conclusion：The ALS diagnosis still depends on clinical manifestation and EMG. EMG, MRI and Blood tests are useful for the diagnosis and differential diagnosis fo ALS.