神经丝蛋白与肌萎缩侧索硬化  

Neurofilament and amyotrophic lateral sclerosis

作　　者：蔡正一 崔丽英[1,2] Cai Zhengyi;Cui Liying(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Neuroscience Center,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院神经科学中心,北京100730

出　　处：《中华神经科杂志》2020年第12期1055-1062,共8页Chinese Journal of Neurology

基　　金：中国医学科学院医学与健康科技创新工程项目(2016-I2M-1-004)。

摘　　要：肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种同时累及大脑锥体细胞、脑干运动细胞核、脊髓前角细胞和锥体束的神经系统变性疾病,早期诊断非常困难,目前尚无特效治疗,预后较差,平均寿命3~5年。研究和发现ALS在早期诊断中特异性的生物标志物,对于缩短诊断延迟时间、探讨和阐明发病机制、监测疾病进展和判断预后非常重要。近年的研究表明,神经丝蛋白(neurofilament)在上述方面具有重要的作用,是目前认为最具临床价值和应用前景的ALS生物标志物。因此,本文对神经丝蛋白与ALS的有关研究进行了综述。Amyotrophic lateral sclerosis(ALS)is a neurodegenerative disorder,stimultaneously affecting cerebral pyramidal cells,motor nuclei in the brain stem,anterior horn cells of the spinal cord,and the pyramidal tract.The early diagnosis of ALS is very difficult.At present,it still lacks efficacious therapy.Its prognosis is poor,with the median survival time for about 3-5 years.The study and discovery of ALS-specific biomarkers for early diagnosis are critical to shorten diagnostic delay,explore and elucidate pathogenesis,monitor disease progression and predict prognosis.In recent years,studies have shown that the neurofilament plays an important role in the aspects above,and at present,it is considered as the most clinically valuable and promising biomarker for ALS.Thus,the article provides a review about research on neurofilament and ALS.

关 键 词：肌萎缩侧索硬化 生物学标记 神经微丝蛋白质类 诊断 诊断 鉴别 预后 

分 类 号：R744.8[医药卫生—神经病学与精神病学]