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目的 探讨肌电图广泛神经源性损害与肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)诊断之间的关系.方法 对2002年1月至2008年12月北京协和医院运动神经元疾病数据库进行回顾性分析,统计肌电图表现为广泛神经源性损害的疾病种类,总结ALS患者首次就诊时肌电图神经源性损害的分布区域和随诊后的结果,并对影响ALS初诊时肌电图表现的因素进行Logistic 回归分析.结果 在首次就诊时,共有298例患者的肌电图表现为广泛神经源性损害,其中ALS 192例(64.4％),进行性肌萎缩36例(12.1％),肯尼迪病13例(4.4％),平山病10例(3.4％),颈椎病或腰椎病9例(3.0％),脊髓性肌萎缩6例(1.3％),多灶性运动神经病5例(1.7％),ALS叠加综合征5例(1.7％),肌病4例(1.3％),遗传性运动神经病3例(1.0％),运动轴索性周围神经病3例(1.0％),脊髓灰质炎后综合征2例(0.7％),未能确定诊断者10例(3.4％).本数据库中,共有213例患者最后确诊为ALS,其中第1次肌电图检查时,8例(3.8％)表现为2个区域神经源性损害,13例(6.1％)表现为1个区域神经源性损害,经随诊3～24个月后,均发展为广泛神经源性损害.Logistic回归分析显示,ALS肌电图广泛神经源性损害的表现与病程、起病部位、发病年龄以及性别无关.结论 广泛神经源性损害的肌电图改变并非仅见于ALS;在疾病发生后一定时期内,ALS也可以仅有1个或2个区域的神经源性损害.

Objective To explore the correlation between diffuse neurogenic changes on electromyography and diagnosis of amyotrophic lateral sclerosis (ALS).Methods Retrospective study was performed based on database of motor neuron disorders collected from January,2002 to December,2008.The category of disease with diffuse neurogenic changes at the first examination was summarized.The electromyography (EMG) manifestation in ALS patients at the first examination and the results after follow-up were reviewed.The factors affecting EMG manifestation in ALS were analyzed with binary Logistic regression.Results In 298 patients with diffuse neurogenic changes on EMG,192 cases (64.4％ ) were diagnosed of ALS,36 ( 12.1％ ) progressive muscular atrophy,13 (4.4％ ) Kennedy' s disease,10 (3.4％)Hirayama disease,9 ( 3.0％ ) cervical spondylosis combined with lumbar spondylosis,6 ( 1.3％ ) spinal muscular disease,5 ( 1.7％ ) multifocal motor neuropathy,5 ( 1.7％ ) ALS-plus disease,4 ( 1.3％ )myopathy,3( 1.0％ ) hereditary motor neuropathy,3 ( 1.0％ ) axonal motor neuropathy,2(0.7％ ) postpolio syndrome,and 10 (3.4％) with no definite diagnosis.In total 213 patients who were diagnosed with ALS after follow-up,at their first examinations,8 (3.8％) had neurogenic changes in two regions and 13(6.1％ ) had neurogenic changes in one region,and they all developed to diffuse neurogenic changes after follow-up for 3 to 24 months.Logistic regression analysis showed that the EMG change at first examination was not related to duration from onset,symptom location at onset,age at onset and gender.Conclusion Diffuse neurogenic changes on EMG can present in many disease including ALS.Neurogenie changes in one or two regions on EMG can be the manifestation of ALS at early stage.