肌纤维内脂肪滴增多在不同神经肌病的表现规律

Appearance of lipid storage in muscle fiber in neuromuscular disorders

目的总结不同神经肌病的肌纤维内脂肪滴增多的病理改变规律。方法分析 5年内 5 2 3例脂质沉积性肌病、线粒体病、肌炎、肌营养不良、激素性肌病、酒精中毒性肌病和神经原性骨骼肌损害等疾病肌肉活检标本的肌纤维内脂肪滴的改变规律。结果在不同疾病情况下肌纤维内脂肪滴增多均以Ⅰ型肌纤维内增多为主 ,而且男性出现脂肪滴增多的情况多于女性。均匀性显著增多常见于脂质沉积性肌病和线粒体 脂肪 糖原累积病 ;均匀性肌纤维内脂肪滴中度增多除部分脂质沉积性肌病外 ,还常见于激素性和酒精中毒性肌病以及严重营养不良的情况。非均匀性显著增多出现在少数线粒体病、肌炎和肌营养不良患者 ;非均匀性中度增多除出现在炎性肌病、肌营养不良 ,还出现在神经原性骨骼肌损害 ;轻度肌纤维内脂肪滴增多可以出现在许多肌肉、神经或全身系统性疾病。结论许多疾病可以伴随肌纤维脂肪代谢异常 ,性别对疾病状态下的脂肪代谢可能存在很大的影响 ,在不同的神经肌病中 ,脂肪代谢的异常具有不同的表现规律 ,肌纤维内脂肪滴增多的诊断价值需要结合骨骼肌其他病理改变以及临床资料进行综合分析。

ObjectiveTo compare the appearance of abnormal lipid accumulation in skeletal muscle fibers in various neuromuscular disorders by analyzing the results of Oil-Red-O staining of muscle biopsy samples. Methods523 muscle biopsies performed in 1998—2002,consisted of lipid storage myopathy, mitochondrial diseases, inflammatory myopathy, muscular dystrophy, steroid myopathy, alcoholic myopathy and neurogenic muscle atrophy,were studed. ResultsType Ⅰ fibers were selectively involved in lipid storage of muscle fibers.In all diseases, men were more frequently affected than momen in which men to women was 3∶2.Marked uniformity lipid storage in muscle fibers was found most frequently in patients with lipid storage myopathy and mitochondrien-lipid-glycogene storage diseases. Moderate uniformity lipid storage in the muscle fibers was found in patients with steroid myopathy, alcoholic myopathy and malnutrition. Moderate or marked non-uniformity lipid storage in muscle fibers was found in patients with mitochondrial cytopathy, inflammatory myopathy, muscular dystrophy. Moderate non-uniformity lipid storage in muscle fibers was found in patients with neurogenic muscle atrophy. Mild lipid storage in muscle fibers was found in numerous systemic disease and neuromuscular disorders. ConclusionNumerous neuromuscular diseases can associated with abnormal lipid metabolism in muscle fibers, in which the men are more involved than women. The appearance of lipid accumulation in the muscle fibers markedly changes in different neuromuscular diseases. The diagnostic value of lipid storage in muscle fibers should be considered under analysis clinical symptoms and other myopathological findings.