摘要
目的探讨进行性脊肌萎缩症(PSMA)的临床特点、诊断与鉴别诊断。方法回顾性分析129例PSMA患者的临床资料。结果本组患者均隐袭起病,逐渐加重,男性多见,发病年龄65.9%患者>50岁。首发症状以单侧上肢无力和肌萎缩为多见(65.9%),均表现为下运动神经元损害的症状和体征,51.9%患者出现延髓麻痹症状;肌电图检查均提示神经源性损害;易误诊为颈或腰椎病。结论本病是一组慢性进行性下运动神经元疾病,病变可累及延髓。诊断主要依据临床表现和肌电图。
Objective To investigate the clinical features, diagnosis and differential diagnosis of progressive spinal muscular atrophy （PSMA）. Method The clinical data of 129 cases of PSMA were analysed retrospectively. Results The patients presented with insidious onset, progressive development and male predominance. The age of onset for most patients （65.9%） was over 50 years old. Weakness and atrophy of single upper extremity were the common primary symptoms （ 65.9% ）. All were presented the symptoms and signs of lower motor neuron damage. 51.9% of the patients showed the symptoms of bulbar palsy. Electromyogram （EMG） suggested neurogenic impairment in all the cases. PSMA might be misdiagnosed as cervical or lumbar diseases. Conclusions PSMA is a chronic neuroclegenerative disease that selectively affected the lower motor neuron and bulbar function. The diagnosis of this disease mainly bases on the clinical features and the result of EMG examination.
出处
《临床神经病学杂志》
CAS
北大核心
2006年第6期420-421,共2页
Journal of Clinical Neurology
关键词
进行性脊肌萎缩症
临床特点
progressive spinal muscular atrophy
clinical features
