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目的 观察脂质沉积性肌病的病理形态学特点 ,并探讨其病因及治疗效果。方法 收集经光镜和电镜检查确诊的脂质沉积性肌病 8例 ,8例患者分别于股四头肌或者肱二头肌取活检 ,常规进行组织学和酶组织化学染色 ,光镜下观察 ,超薄切片 ,铅铀双染色 ,电镜下观察。并对 8例患者的临床治疗用药进行分析。结果 8例患者肌纤维内均有空泡及裂隙样改变 ,苏丹黑B及油红O脂肪染色示肌纤维内脂滴明显增加 ,电镜证实大量的脂肪滴堆积在残存的肌纤维中。病变为轻度者有 3例 ,中度有 2例 ,重度有 3例 ,其中 1例合并了糖原沉积病 ,1例合并细胞色素C氧化酶明显降低。使用肾上腺皮质激素治疗 7例有效 ,1例无效 ,合并维生素B2 及其他维生素等药物治疗效果好 ,肉毒碱治疗对系统性缺乏患者有显效。结论 脂质沉积性肌病确诊靠肌肉活检病理形态特征性改变 ,肾上腺皮质激素、肉毒碱、维生素、含有丰富肉毒碱食物综合治疗可获显效 ,由于病因有多种 ,明确某一种病因后可针对病因特定治疗。
Objective To observe the pathological features of lipid-deposited myopathy and explore its etiology and therapeutic effect. Methods Eight patients with lipid-deposition myopathy diagnosed by light microscope and electron microscope were collected. Eight patients were taken biopsy of quadriceps or biceps, histology and enzymatic histochemistry were routinely performed and observed under light microscope , Ultrathin sections, lead-uranium double-stained, observed under electron microscope. Eight patients with clinical medication were analyzed. Results All the 8 patients had vacuoles and fissure-like changes in the muscle fibers. Fat staining of Sudan Black B and Oil Red O showed a significant increase of lipid droplets in the muscle fibers. Electron microscopy confirmed that a large number of fat droplets accumulated in the remnant muscle fiber. Lesions were mild in 3 cases, moderate in 2 cases, severe in 3 cases, of which 1 case of glycogen deposition disease, 1 case of combined with cytochrome C oxidase decreased significantly. The use of corticosteroids in 7 cases effective, 1 case of ineffective, combined with vitamin B2 and other vitamins and other drugs treatment effect, carnitine treatment of patients with systemic deficiency was effective. Conclusions The diagnosis of lipid deposition myopathy is characterized by the characteristic changes of the pathological changes of muscle biopsy. Corticosteroids, carnitine, vitamins, and foods rich in carnitine can be markedly effective. Due to various causes, a definite cause After the specific treatment for the cause.