良性单侧下肢萎缩的临床特点

Clinical features of Benign monomelic amyotrophy of lower limb

目的探讨良性单侧下肢萎缩(BMALL)的临床特点。方法回顾分析3例BMALL患者的临床资料。结果 3例患者均为男性,慢性起病,以单侧下肢肌萎缩为主症,肌无力程度轻;血清肌酶水平均正常;肌电图示2例为神经源性损害,1例未见异常;腰椎影像学示3例L3～4、L4～5椎间盘轻度突出。肌肉病理学检查示3例患者均出现肌纤维大小不等和小角化纤维,极小纤维和核内移纤维各2例,同型纤维明显群组化1例。结论 BMALL临床特征为单侧下肢萎缩,神经电生理及肌肉病理学均表现为神经源性损害。

Objective To approach the clinical features of benign monomelic amyotrophy（BMALL）.Method The clinical data of 3 BMALL patients were analysed retrospectively.Result All of the 3 cases were male and chronic onset,mainly manifestation was single side lower limb muscle atrophy.The degree of muscle weakness was light.The level of serum enzyme in the 3 cases were normal.EMG showed that the neurogenic damage in 2 cases,and other one was normal.The lumbar imaging showed that intervertebral disk between L3-4,L4-5 were mild outstanding in the 3 cases.Muscle pathologic examination showed that the size of muscle fibers was inequality and had little keratosic fiber.The minimum and nuclear ingression myofibers were fount out in 2 cases respectively and myogenic grouping in 1 case.Conclusions The clinical feature of BMALL is single side lower limb muscle atrophy.The feature of muscle electrophysiology and pathology are neurogenic damage.