论文部分内容阅读
目的探讨脂质沉积性肌病(lip id storagy myopathy,LSM)的临床及病理特征。方法收集3例经病理检查确诊的LSM,结合相关文献分析其临床表现、病理特征和实验室检查结果。结果临床以近端肌无力、萎缩为主要表现,有运动不耐受特点,肌电图示以肌源性损害为主,可合并神经源性损害,肌酶以CK和LDH升高为主,对激素反应早且明显,病理特征是Ⅰ型肌纤维内大量脂质颗粒沉积。结论LSM临床无特征性表现,确认依赖于肌肉病理学检查。
Objective To investigate the clinical and pathological features of lipid stray myopathy (LSM). Methods Three LSM patients diagnosed by pathology were collected and analyzed for their clinical manifestations, pathological features and laboratory findings. Results The main clinical manifestations of proximal muscle weakness, atrophy as the main features of exercise intolerance, myoelectricity showed mainly myogenic damage, may be associated with neurogenic damage, CK and LDH muscle enzymes mainly to Hormone response early and obvious pathological features of a large number of lipid particles within the type Ⅰ muscle fiber deposition. Conclusion There is no clinical manifestations of LSM, confirm the pathological examination depends on muscle.