山西省165例运动神经元病临床特征及误诊分析  

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

作　　者：乔晶晶[1] 张金[1] 郭军红[1] 薛国芳[2] 张晓雷[3] 

机构地区：[1]山西医科大学第一医院,山西省太原市030001 [2]山西医科大学第二医院,山西省太原市030001 [3]山西省人民医院,山西省太原市030001

出　　处：《国际神经病学神经外科学杂志》2014年第1期23-26,共4页Journal of International Neurology and Neurosurgery

基　　金：国家自然科学基金(2013011057-2);2011年高校院基金(09019)

摘　　要：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。结果人组患者165例：ALS122例（73．9％），其中确诊ALS101例，临床或电生理拟诊ALS21例；PMA26例（15．8％）；PBP15例（9．1％）；PLS2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（P〉0．05）。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。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.

关 键 词：运动神经元病 临床特征 误诊 

分 类 号：R744.8[医药卫生—神经病学与精神病学]