进行性神经性肌萎缩症的临床与检查——附58例分析

Progressive neuromuscular atrophy syndrome:clinical manifestation and examination in 58 cases

目的:研究进行性神经性肌萎缩症的临床及实验室检查特点。方法:对58例进行性神经性肌萎缩症患者的临床表现,肌酶、肌电图、肌肉活组织检查(活检)及腿部肌肉CT扫描结果进行回顾性分析。结果及结论:本病临床呈良性过程,病人仍能保持一定的劳动力;7例肌酶轻度增高;肌电图改变有两类,一类表现为神经源性损害伴传导速度减慢或不能引出,共52例,另一类是神经源性损害伴高电位出现,6例;肌肉活检23例,19例表现为大群性肌萎缩,4例为小群性肌萎缩,其中4例病人出现肌病性改变;6例患者行腿部肌肉CT扫描,均显示肌肉密度减低。对患者的临床表现,肌酶、肌电图、肌活检、CT检查结果进行综合分析有助于进行性神经性肌萎缩症的诊断和对其进行分类。

Objective:To study the characteristic of clinical and laboratory examination of progressive neuromuscular atrophy syndrome. Methods:Data of clinical manifestations,muscle enzyme profile,electromyogram (EMG),muscle biopsy and lower limbs CT scan were analyzed retrospectively in 58 cases with progressive neuromuscular atrophy syndrome. Results and conclusions:The clinical progress of progressive neuromuscular atrophy syndrome was slow and benign. Muscle enzyme increased in 7 ca ses. There were two types of the EMG changes. EMG showed neural damage and slow or disappearance of conduct velocity in 52 ca ses,neural damage and high potential were found in 6 cases. All muscle biopsies were abnormal. 19 cases had muscle atrophy in big crowds,4 cases had muscle atrophy in small crowds. 4 cases had myopathy change. 6 cases done with lower limbs CT scan showed that muscle density decreased. Comprehensive analysis of clinical manifestation,muscle enzyme profile,EMG,pathological findings and CT scan is helpful in diagnosis and classification of progressive neuromuscular atrophy syndrome.