脂质沉积性肌病的电镜超微病理诊断

代谢性肌病中的脂质沉积性肌病（ lipid storage myopathy，LSM），临床表现缺乏特异性，容易引起误诊。LSM等代谢性肌病的诊断，更多依赖临床表现、生化检查、组织化学及酶组织化学检查等。如果临床取材方法恰当、取材位点准确，通过骨骼肌电镜超微结构观察，可进一步证实肌纤维内是否存在脂质沉积、线粒体结构异常等病变，对线粒体肌病、LSM等肌病的诊断与鉴别诊断具有重要的临床价值；但如果取材不当，则可能导致漏诊，甚至误诊。

Ultrapathological diagnosis of lipid storage myopathy by electron microscopy

The lipid storage myopathy( LSM) of metabolic myopathies lacks specificity in clinical manifestations, and it is easy to cause misdiagnosis. The diagnosis of metabolic myopathies such as LSM depend more on clinical manifestation, biochemical examination, histochemical and enzyme histochemical examination and so on. If the clinical sampling method of skeletal muscle tissue is appropriate, and the sampling site is accurate, it can be further confirmed whether the abnormal ultrastructural pathological changes such as lipid deposition and mitochondrial structures be observed in muscle fibers of the skeletal muscle tissue by means of the transmission electron microscopy, which will have important clinical value in the diagnosis and differential diagnosis of mitochondrial myopathy, LSM and other myopathies. But if the clinical sampling method of the muscle is inappropriate, it may lead to missed diagnosis and even error diagnosis.