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目的 结合肌肉病理改变 ,探讨脂质沉积性肌病 (L SM)的诊断及治疗。 方法 对 15例 L SM患者进行临床及病理分析 ,光镜与电镜观察。 结果 患者均有进行性四肢无力 ,血清肌酶不同程度升高 ,肌肉病理苏丹黑染色及电镜示肌纤维中大量脂质沉积 ,以 型纤维为主。 结论 L SM是一组以肌无力和肌疲劳为主要表现的肌纤维内脂肪代谢障碍病。肌肉病理检查有助于确定诊断。激素治疗有效。
Objective To investigate the pathological changes of muscle and discuss the diagnosis and treatment of lipid deposition myopathy (L SM). Methods The clinical and pathological analysis of 15 cases of L SM patients were observed by light microscope and electron microscope. Results All patients had progressive weakness of the extremities, elevated serum muscle enzymes to varying degrees, Sudan black staining of muscle pathology, and a large amount of lipid deposition in the muscle fibers by electron microscopy. Conclusion L SM is a group of myofibrillar fat dysfunctions, which mainly manifest muscle weakness and muscle fatigue. Muscle pathology helps determine the diagnosis. Hormone therapy is effective.