６例脂质沉积性肌病经肌肉组化检查确诊，其中２例做了电镜观察。年龄在３～３５岁之间，４例有家族史，均在同辈人发病，属常染色体隐性遗传。肌活检油红Ｏ染色示大量脂滴在肌浆中沉积，Ⅰ型纤维中含量最多，Ⅱ型其次。电镜观察见大量无膜脂滴平行分布于肌原纤维间，线粒体增多，其嵴不清晰。病人皆无全身性症候，故以肉硷缺乏致病可能性最大。６例对强地松疗效均满意。 6 cases of lipid deposition myopathy confirmed by muscle biopsy, of which 2 cases were electron microscopy. The age of 3 to 35 years old, 4 cases of family history, all in the same generation of human, autosomal recessive inheritance. Muscle biopsy oil red O staining showed a large number of lipid droplets deposited in the sarcoplasm, type Ⅰ fiber content, type Ⅱ followed. Electron microscopy showed a large number of lipid droplets without membrane parallel distribution in myofibrils, mitochondria increased, the crest is not clear. Patients have no systemic symptoms, so the possibility of a lack of meat alkaline pathogenesis. Six cases of pine are satisfied with the efficacy.