摘要
目的探讨伴有破碎红纤维的包涵体肌炎的临床及病理学特点。方法回顾性分析1例伴有破碎红纤维的包涵体肌炎患者的临床资料。结果本例为中年男性,四肢近端进行性肌萎缩,血清肌酶轻度增高,肌电图示肌源性损害。肌活检示部分萎缩的肌纤维出现镶边空泡,空泡内含有嗜碱性颗粒,部分坏死肌纤维有吞噬细胞及炎症细胞浸润,改良Gomori染色见破碎红纤维,细胞色素酶染色见蓝纤维;泛素染色示肌纤维中有泛素阳性物质沉积。mtDNA突变分析未见线粒体DNA突变。结论伴有破碎红纤维的包涵体肌炎以近端肌肉受损为著,病理表现除包涵体和炎症性改变外,还存在代谢紊乱和异常折叠的蛋白沉积的特征。
Objective To investigate the clinical and pathological features of inclusion body myositis comlined with ragged red fiber （RRF）. Methods The clinical data of 1 inclusion body myositis patient comlined with RRF was analyzed retrospectively. Results The patient was a middle-aged man with progressive muscular atrophy in proximal limbs. His serum enzyme creatine kinase was mild elevated. Eleetromyogram showed myopathic injury. Mucular biopsy was showed some atrophic fibres with rimmed vacuoles, and rimmed vacuoles contained basophilic granules. A few necrotic fibres were infiltrated with macrophagocyte and imflammator cells. The RRF was found out by modified Gomori stain, and blue fibres was found out by cyeloxygenase stain. Ubiquitin stain showed Ubiquitin positive inclusions. MtDNA mutation analysis hadn＇t found mution. Conclusions Inclusion body myositis with RRF has significant injury of proximal limbs muscles. The pathologic features are inclusions and inflammatation changes, and it also has metabolic disorders and aggregation of misfolding protein.
出处
《临床神经病学杂志》
CAS
北大核心
2013年第1期19-21,共3页
Journal of Clinical Neurology
基金
江苏省自然科学基金项目(BK2007607)
关键词
包涵体肌炎
破碎红纤维
inclusion body myositis
ragged red fiber
