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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 investigate the clinical and muscle pathological features of lipid deposition myopathy (LSM). Methods: One patient with LSM was analyzed for clinical features, laboratory tests and muscle biopsy data, and the related literature was reviewed. RESULTS: The main clinical features of patients with LSM were weakness in the proximal extremities and intolerance to exercise. Laboratory tests showed a moderate increase in serum creatinine; EMG mostly myogenic damage; muscle pathology showed muscle fiber vacuolar degeneration, lipid droplets increased significantly. Conclusion: LSM is a rare type of myopathy. The patients were mainly manifested as muscle weakness of limbs and intolerance to exercise. The diagnosis was based on muscle pathology and the prognosis was relatively good.