目的:探讨脂质沉积性肌病(LSM)的临床和肌肉病理特点.方法:分析1例LSM患者的临床特点、实验室检查、肌活检资料,并复习相关文献.结果:LSM患者的主要临床特点为四肢近端肌无力和对运动不耐受.实验室检查示血清肌酶中度升高；肌电图多呈肌源性损害；肌肉病理显示肌纤维空泡变性,脂滴明显增多.结论:LSM是一种少见的肌病类型,患者以肢体近端肌无力及对运动不耐受为主要表现,确诊依靠肌肉病理检查,预后相对良好.
Objective To analyze the clinical and pathological features of lipid storage myopathy (LSM). Methods The clinical data, laboratory results and muscle biopsies of a case of LSM were analyzed and having the related literature reviewed. Results Weakness of proximal limb muscles and intolerance to exercise were the prominent clinical manifestations.Serum muscle enzymes were moderately elevated,and electromyogram (EMC) showed myogenic damages. Myopathological changes included vacuole degeneration of muscle fiber and increase of lipid droplets. Conclusions LSM is a rarely seen myopathy. Weakness of proximal limb muscles and intolerance to exercise are the two major clinical manifestations.The definite diagnosis depends on the muscle biopsy, and the prognosis is relatively well.
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