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目的:对2例脂质沉积性肌病(LSM)的临床资料予以分析报道。方法:收集2例经病理检查确诊的LSM病例的临床资料,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:LSM临床以肢带肌近端无力为主要表现,有明显的运动不耐受特点,部分受累肌有压痛;肌电图示以肌源性损害为主;肌酶以肌酸激酶轻中度升高为主,对糖皮质激素反应早且明显;病理特征为肌纤维内有大量脂质颗粒沉积,并以Ⅰ型肌纤维为主。结论:LSM以肢带型肌无力综合征为临床表现时极易误诊为多发性肌炎等,确诊依赖于肌肉病理学检查。
Objective: To analyze the clinical data of two cases of lipid deposition myopathy (LSM). Methods: Two cases of LSM diagnosed by pathology were collected, and their clinical manifestations, pathological features and laboratory findings were analyzed in combination with related literatures. Results: LSM clinical manifestations of limb proximal weakness as the main performance, there are obvious characteristics of sports intolerance, part of the muscle involvement of tenderness; EMG showed mainly myogenic damage; creatine kinase mild to moderate Increased mainly glucocorticoid response early and obvious; pathological features of a large number of muscle fiber deposition of lipid particles, and the main type Ⅰ muscle fibers. CONCLUSIONS: LSM is easily misdiagnosed as polymyositis when it is characterized by limb weakness syndrome, and diagnosis depends on muscle pathology.