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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 investigate the clinical features of benign unilateral lower limb atrophy (BMALL). Methods The clinical data of 3 patients with BMALL were retrospectively analyzed. Results All the 3 patients were male and had chronic onset. The main symptoms were muscle atrophy of unilateral lower extremity. Muscular weakness was mild. Serum muscle enzymes were normal. Electromyography showed 2 cases of neurogenic damage and 1 case showed no abnormality ; Lumbar imaging showed 3 cases of L3 ~ 4, L4 ~ 5 intervertebral disc mildly prominent. Muscle pathology examination showed that there were 2 cases with small muscle fibers, small keratinized fibers, minimal fibers and nuclear endocervical fibers in 3 cases, and 1 case with identical fibers. Conclusion The clinical features of BMALL are unilateral lower extremity atrophy, neuroelectrophysiology and muscle pathology all show neurogenic damage.