山西省165例运动神经元病临床特征及误诊分析

目的研究运动神经元病(MND)的临床特征及误诊分析。方法回顾性分析165例MND患者的一般资料、临床症状、误诊、肌电图、辅助检查等,进行统计分析。结果入组患者165例:ALS 122例(73.9%),其中确诊ALS 101例,临床或电生理拟诊ALS 21例;PMA 26例(15.8%);PBP 15例(9.1%);PLS 2例(1.2%)。男女比例为1.84∶1。MND患者发病年龄为20~80岁,平均发病为52.15±10.41岁,男女发病高峰均为50~59岁,二者差异没有统计学意义(P0.05)。山西省MND误诊率为42.42%(70/165),误诊为脑血管病的35.7%(25/70),误诊于基层医院77.1%(54/70)。结论 MND发病男性多于女性,山西省男性患病比例较国内相关报道高,发病年龄集中在50~59岁;分型中ALS是最常见的类型;MND误诊率高,脑血管病误诊居首位,误诊多发生在基层医院;影像学及电生理检查对MND的诊断有重大意义,临床上应积极完善相关辅助检查。

Clinical features and misdiagnosis of motor neuron diseases: an analysis of 165 cases in Shanxi Province, China

Objective To analyze the clinical features and misdiagnosis of motor neuron diseases （MND）. Methods A retrospective analysis was performed on the clinical data of 165 MND patients to investigate general information, clinical symptoms, misdiagnosis, electromyograms, and auxiliary examinations. Results Of the 165 patients, 122（73.9% ） had amyotrophic lateral sclerosis （ ALS）, including 101 confirmed cases and 21 suspected cases （ supported by clinical and electrophysiological examinations）, 26（ 15.8% ） had progressive muscular atrophy, 15 （9.1% ） had progressive bulbar palsy, and 2 （ 1.2% ） had primary lateral sclerosis. The male-to-fe- male ratio was 1.84： 1. The age of patients with MND ranged from 20 to 80 years. The mean age of onset was 52.15 ±10.41 years. The peak age of onset was 50 - 59 years whether in males or females, with no significant difference （ P 〉 0.05 ）. The misdiagnosis rate of MND was 42.4% （70/165） in Shanxi Province. Of the misdiagnosed cases, 35.7% （：25 cases） were misdiagnosed as cerebrovascu- lar diseases, and 77.1% （54 cases） were found in primary hospitals. Conclusions MND is more common in males than in females; the prevalence rate among males in Shanxi Province is higher than related data reported in China. The peak age of onset is 50 -59 years. ALS is the most common type of MND. The misdiagnosis rate of MND is high; MND is usually misdiagnosed as cerebrovascular diseases, and misdiagnosed cases are mainly found in primary hospitals. Imageological and electrophysiological examinations are of great significance for the diagnosis of MND, so we should actively improve the relevant auxiliary examinations.