散发性肌萎缩侧索硬化39例临床分析

摘要

Objective To determine the clinical characteristics of sporadic amyotrophic lateral sclerosis (sALS), to improve the accuracy of diagnosis. Methods We reviewed 39 cases visiting the General Hospital of Chinese People's Armed Po-lice force during June 2009 and June 2014. Results The average morbidity age was (54. 7 ± 10. 7)years-old. They were mostly male patients and the peak of the course was at the 24th month. The risk of paroxysm was greatly increased in individuals who were factory workers and farmers and who used to be in good health. The main clinical manifestations included muscle weakness, muscle atrophy, fasciculations, slurred speech, drinking cough and cognitive dysfunction. About 12. 8% of sALS had cognitive impairment. The cervical cord was supposed to be the predilection site. Conclusion sALS is a middle-aged, male predominant disease. sALS accompanied with cognitive impairment may be related to frontotemporal dementia, Alzheimer's disease and other neurodegenerative disorders in some aspects. An detailed inquiry, a thorough physical check-up and timely clinical examination of electromyogram and discriminating diagnosis are important in diagnosis.%目的：了解散发性肌萎缩侧索硬化( sporadic amyotrophic lateral sclerosis, sALS)的临床特点,以提高临床诊断率。方法回顾性分析2009年6月—2014年6月武警总医院收治的39例sALS的临床资料。结果本组发病平均年龄(54.7±10.7)岁,男性多见,病程高峰在24个月左右。发病人群主要为既往体健人群,临床表现主要为肌无力、肌肉萎缩、肌束震颤、言语不清和饮水呛咳,5例(12.8%)伴随认知功能障碍,颈髓受累最多。结论 sALS以中年发病为主,男性多见,sALS伴认知功能障碍可能与阿尔茨海默病和额颞叶痴呆等其他神经系统变性病存在某种联系；详细问诊,仔细查体,及时行肌电图检查并注意鉴别诊断,有助于诊断。