散发性包涵体肌炎七例临床及病理特点  

Clinical and myopathological features in sporadic inclusion body myositis:a 7-case report

作　　者：郑日亮[1] 焉传祝[2] 吕海东[3] 吕鹤[1] 毕鸿雁[1] 袁云[1] 

机构地区：[1]北京大学第一医院神经内科,100034 [2]山东大学齐鲁医院神经内科 [3]河南省焦作市人民医院神经内科

出　　处：《中华神经科杂志》2007年第12期796-799,共4页Chinese Journal of Neurology

摘　　要：目的探讨散发性包涵体肌炎(sIBM)患者的临床及病理诊断规律。方法收集7例于2001年至2005年就诊并明确诊断为 sIBM 的患者临床、病理资料,发病年龄为41～75岁,平均57.4岁,病程2～10年,平均5.4年。全部患者均有股四头肌无力和萎缩,4例出现肢体远端无力,1例出现延髓部和颈部无力。其中伴随高血压和腔隙性脑梗死者2例,伴随脑出血、周围神经病和糖尿病各1例。7例患者均进行了肌肉活体组织检查标本的组织学、酶组织化学染色,5例进行电镜检查,3例进行了 tau 蛋白免疫组织化学染色。结果 7例患者肌酶均升高,但未超过正常上限8倍。肌电图检查示5例呈肌源性损害,2例呈神经源性损害。所有患者的骨骼肌病理改变主要是肌内衣炎细胞浸润、肌纤维直径变异加大和镶边空泡(出现率2%～10%),5例出现不整红边纤维,3例有细胞色素 C 氧化酶染色阴性肌纤维。5例进行电镜检查者均存在管丝包涵体。3例免疫组织化学染色者均显示肌纤维内 tau 蛋白沉积。结论 sIBM 以股四头肌损害最明显,常合并其他老年性疾病。肌纤维内出现 tau 蛋白也可以作为该病诊断标准之一。Objective To investigate the clinical and myopathologic features of sporadic inclusion body myositis （sIBM）. Methods Seven patients with sIBM were collected from 2001 to 2005. The onset of the symptoms varied from 41 to 75 years old with an average age of 57.4 years old. All the 7 patients developed progressive weakness of extremities, predominantly in the quadriceps. Additionally, distal weakness of extremities appeared in 4 patients, and neck weakness with bulb palsy in 1 patient. Hypertension with lacunar infarct could be noted in 2 patients. Cerebral hemorrhage, peripheral neuropathy and diabetic mellitus were found in 1 patient respectively. Muscle biopsy was done in the 7 patients, and the sections were stained histologically and enzymhistochemicaUy. Uhrathin section was examed with electron microscopy in 5 patients. Anti-tau immunohistochemically staining was performed in 3 patients. Results Serum creatine kinase levels were mildly elevated （less than 8-fold） in all the 7 patients. Electromyography showed myogenic changes in 5 patients and neurogenic changes in 2. Rimmed vacuoles, endomysial inflammation, hypertrophy and atrophy of muscle fiber were found in all of the patients. Rimmed vacuoles appeared in 2%--10% of fibers which showed no necrosis or surrounded by inflammatory infiltration. Ragged red fibers appeared in 5 of them. Three patients showed COX negative fibers. Electron microscope showed tubulofilamentous inclusions in muscle fibers in all examined patients. Tau positive material appeared in the vacuoles of muscle fibers in the 3 patients. Conclusions The quadriceps is most involved in our patients. Bulb palsy could be occasionally noted in this disease. They are usually combined with other senile diseases. Mthough the histological examination plays an important role in the pathological diagnosis, that tau immunostaining shows positive in the muscle fibers could be one of the diagnostic criteria in inclusion body myositis.

关 键 词：肌炎 包涵体 肌纤维 活组织检查 TAU蛋白质类 

分 类 号：R746[医药卫生—神经病学与精神病学]