貌似多发性肌炎的脂质沉积性肌病病理改变

目的：研究貌似多发性肌炎的脂质沉积性肌病的肌肉病理改变，以解释临床现象和探讨发生机制。方法：择取脂质沉积性肌病伴血清肌酶明显升高的 人14例，将临床表现，血清肌酶，肌电图和肌肉病理及肌肉组织化学改变进行总结分析。结果：临床上一般呈急性或亚急性起病，或呈反复发作性病程，肌无力明显，血清肌酶明显升高，激素治疗反应好，肌肉病理可见肌纤维内脂滴明显增多，形成HE染色可见的空泡，ORO染色证实肌纤维内有大量脂滴，其中4例可见个别坏死肌纤维和再生纤维，5例有较严重的肌纤维坏死，对其中3例反复肌肉活检，发现在脂质贮积的基础上，病程中的某一阶段发生明显的肌纤维坏死，其余病例虽有血清肌酶的明显升高，而病理上并未见到明显的肌纤维坏死，部分肌纤维非特异性酯 酶活性增高。结论：有些脂质沉积性肌病与多发性肌炎十分相似，需要借助肌肉病理和肌肉组织化学检查，甚至反复肌肉活检才能确定，肌纤维内脂肪代谢障碍造成肌纤维破坏和肌酶的外漏，可能与细胞内环境改变和细胞膜通过透性加有关。

Pathlolgy of lipid storage myopathy presentiong as polymyositis

Objective Some cases of lipid storage myopathy (LSM) are similar to the polymyositis(PM) in clinic.To study the muscle pathology is helpful as to making clear the diagnosis and elucidating the mechanism of the LSM. Methods 14 cases of LSM with high level of CK in serum were analyzed clinically and compared with their muscle morphological and histochemical changes.Results Muscle weakness and fatigue were the main complaints of these patients.Most of them had acute or sub acute onset,some of them had relapse courses.All of the muscle specimens showed typical accumulation of lipid in muscle fibers.Necrotic fibers were found in 9 cases,3 of them showed changes with the courses. Conclusion Muscle biopsies and histochemical studies are necessary to differentiate LMS with PM.The fiber necrosis and CK leakage in LSM may be caused by changes of the inter circumstance and the membrane disturbance of cells.