散发性肌萎缩侧索硬化的临床特征  

Clinical study in 40 cases of sporadic amyotrophic lateral sclerosis and literature review

作　　者：杨云珠[1] 徐仁伵[1] 

机构地区：[1]南昌大学第一附属医院神经内科,南昌330006

出　　处：《江西医药》2009年第4期309-311,共3页Jiangxi Medical Journal

摘　　要：目的探讨散发性肌萎缩侧索硬化(SALS)的临床特征,为早期准确诊断SALS提供依据。方法回顾分析近年来我院收治的40例SALS患者的临床资料,对其发病特点、症状、体征及实验室检查进行统计分析。结果SALS平均发病年龄52.4岁,30岁以前和75岁以后发病少见,男女发病比例5∶3。SALS在临床上隐袭起病居多,但也可呈亚急性起病。上肢远端乏力或肌肉萎缩为最常见的起病症状,其发展一般从起病部位水平或垂直的累及邻近的运动神经元。并发呼吸肌麻痹是SALS最常见的死因。SALS的诊断仍然主要依靠临床表现。EMG检查对确诊SALS的病变范围和诊断无临床表现的隐匿损伤部位有重要意义。MRI检查对鉴别颈椎病及脊髓压迫症、脊髓肿瘤和脊髓空洞症的诊断具有重要的辅助价值。结论SALS以中年发病为主,男性多见,其发展遵循一定的规律。EMG和MRI检查对SALS的诊断和鉴别诊断有重要意义。Objective To analyze the clinical features of sporadic amyotrophic lateral sclerosis（SALS）,and provide clinical data for early diagnosis.Methods We retrospectively reviewed the records of 40 cases of SALS in our hospital during recent years.Clinical characteristics,symptom,sigu and data of laboratory examination on the patients were statistical analyzed.Results The mean age at oneset was 52.4 years,onset before 30 years and 75 years was less.Male to female ratio was 5：3.An insidious onset is very common,but it can also develop subacutely. The hypodynamia or amyotrophy of the upper extremities was the most common initial symptom. SALS develops from the initial location to the near motor neuron vertically or horizontally.Respiratory muscle paralysis was the principal cause of death.The diagnosis of ALS was mainly depend on clinical sign yet.EMG has significant value in the diagnosis and MRI was beneficial to the differential diaguosis.Conclusion SALS mainly onset when middle-aged,males are significantly more than females, and it follows invariably developing regularity. EMG and MRI has important value in the diagnosis and differential diagnosis of ALS.

关 键 词：散发性肌萎缩侧索硬化 临床表现 诊断 

分 类 号：R746.4[医药卫生—神经病学与精神病学]