散发性肌萎缩侧索硬化患者骨骼肌病理改变特点及其与疾病进展的相关性  

The relationship between disease progression and skeletal muscle pathological changes in sporadic amyotrophic lateral sclerosis patients

作　　者：朱文佳[1] 欧阳雅声 邸丽[1] 卢岩[1] 王敏[1] 文欣玫 朵建英[1] 栾沁榕 李存江[1] 笪宇威[1] ZHU Wenjia;OUYANG Yasheng;DI Li;LU Yan;WANG Min;WEN Xinmei;DUO Jianying;LUAN Qinrong;LI Cunjiang;DA Yuwei(不详;Department of Neurology,Xuanwu Hospital Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经内科,100053

出　　处：《中国神经免疫学和神经病学杂志》2021年第5期376-380,共5页Chinese Journal of Neuroimmunology and Neurology

基　　金：国家自然科学基金资助项目(82001352、81801255);首都卫生发展科研专项(首发2018-4-2015)。

摘　　要：目的研究散发性肌萎缩侧索硬化(sporadic amyotrophic lateral sclerosis,sALS)患者骨骼肌病理改变特点及其与疾病进展速度的相关性。方法回顾性分析2011年4月至2018年3月首都医科大学宣武医院收治的sALS患者29例,患者均行肌肉活检提示神经源性损害,且经随访诊断符合修订的EI Escorial临床确诊或临床很可能的ALS标准。根据活检部位肌力情况分为症状前期(肌力正常组)及症状期(肌力异常组),比较不同疾病阶段骨骼肌病理特点及Ⅰ和Ⅱ型肌纤维受累情况。根据疾病进展速率中位数,将患者分为缓慢进展组及快速进展组,比较不同疾病进展速率患者间骨骼肌病理改变特点以及神经再生支配代偿程度的差异性。结果在sALS的不同疾病阶段均可见肌纤维角形萎缩呈小组样分布及群组化现象,但两组患者出现不同类型肌纤维小组样萎缩及群组化构成无统计学差异(P>0.05)。不同疾病进展速率组间出现小组样萎缩程度、靶纤维及群组化病理改变的患者分布无统计学差异(P>0.05),但缓慢进展组神经再生支配代偿程度较快速进展组更高(P<0.05)。结论在sALS患者的不同疾病阶段Ⅰ和Ⅱ型肌纤维均存在失神经及神经再生支配的病理改变,不存在选择性肌纤维受累。缓慢进展的sALS患者神经再生支配程度较快速进展患者更高。Objective To investigate the pathological changes of skeletal muscle in sporadic amyotrophic lateral sclerosis(sALS)and its correlation with the rate of disease progression.Methods A retrospective analysis was performed on 29 patients with sALS in Xuanwu Hospital Capital Medical University from April 2011 to March 2018.All patients underwent skeletal muscle biopsy,indicating neurogenic pattern changes.They all met the revised EI Escorial criteria for clinically definite or clinically probable ALS.According to the muscle strength of the biopsy site,the patients were divided into presymptomatic(normal muscle strength)and symptomatic(abnormal muscle strength)groups,the pathological characteristics of skeletal muscle and the involvement of typeⅠandⅡmuscle fibers in different disease stages were compared.According to the median rate of disease progression,the patients were divided into a slow progression group and a rapid progression group.The pathological characteristics of skeletal muscle and the degree of nerve innervation with different disease progression rates were compared.Results In different stages of sALS,angular atrophic muscle fibers were distributed in small groups and showed fiber type grouping,but there was no significant difference in types of atrophic fiber and grouping between the two groups(P>0.05).There was no significant difference in the extent of angular atrophic muscle fibers,target fibers and type grouping among different rates of disease progression(P>0.05),but the compensation of nerve innervation in the slow progression group was higher than that in the rapid progression group(P<0.05).Conclusions There were pathological changes of denervation and innervation in typeⅠandⅡmuscle fibers of sALS patients at different disease stages,and both fiber type were involved.Patients with slowly progressing sALS had a higher level of nerve innervation than patients with rapidly progressing sALS.

关 键 词：肌萎缩侧索硬化 小组样萎缩 靶纤维 群组化 肌萎缩 

分 类 号：R744.8[医药卫生—神经病学与精神病学]