原发性侧索硬化2例报道及相关文献复习  

Report of two cases of primary lateral sclerosis and review of the literature

作　　者：王凤志[1] 何志义[1] 李蕾[1] 邓淑敏[1] 刘芳[1] 赵奕楠[1] 孟肃[1] 

机构地区：[1]中国医科大学附属第一医院神经内科,辽宁沈阳110001

出　　处：《中风与神经疾病杂志》2013年第3期244-247,共4页Journal of Apoplexy and Nervous Diseases

摘　　要：目的探讨原发性侧索硬化(PLS)的临床诊断、与经典的肌萎缩侧索硬化的关系、神经电生理特点、影像学特点及鉴别诊断。方法分析我科收治的2例PLS患者的临床资料,并复习相关文献。结果 PLS是隐匿起病,进展缓慢的仅累及上运动神经元的神经退行性疾病,很多初诊为PLS的患者经过长期随访最后发展为肌萎缩侧索硬化,PLS的影像学表现多样,神经电生理检查容易早期发现下运动神经元损伤。结论对于疑诊PLS的患者,明确是否具有局限性下运动神经元损伤症状十分重要,对患者进行时间的纵断随访对疾病最后的诊断和预后评估意义重大。Objective To investigate the clinical diagnosis of primary lateral sclerosis （PLS）, and the relationship with the classic amyotrophic lateral sclerosis （ALS）, the neural electrophysiological features, imaging features and differenti- al diagnosis. Methods We analyzed the clinical data of 2 patients with PLS who were admitted to our hospital, and re- viewed of relevant literature. Results PLS is a neurodegenerative disease characterized by involving only the upper motor neuron,occult onset,and the slow progress. Mmany patients diagnosed as PLS after long-term follow-up turned out to be ALS,PLS is various in imaging manifestation, and neural electrophysiological examination is helpful in early detection of lower motor neuron injury. Conclusion For patients suspected with PLS, identifying the existence of localized injury of lower motor neuron symptoms is very important, conducting a longitudinal follow-up is of great significance to the last diag- nosis and prognosis.

关 键 词：原发性侧索硬化 肌萎缩侧索硬化 上运动神经元 下运动神经元 

分 类 号：R744[医药卫生—神经病学与精神病学]