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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 pathological diagnosis of patients with sporadic inclusion body myositis (sIBM). Methods The clinical and pathological data of 7 patients who were diagnosed as sIBM from 2001 to 2005 were collected. The age of onset was 41-75 years (mean 57.4 years). The course of disease was 2-10 years (average 5.4 years). All patients had quadriceps weakness and atrophy, limb distal weakness in 4 cases, 1 case of medullary and neck weakness. One with hypertension and lacunar infarction in 2 cases, with cerebral hemorrhage, peripheral neuropathy and diabetes in 1 case. Tissue and enzyme histochemical staining of muscle biopsy specimens were performed in 7 patients. Electron microscopy was performed in 5 patients and tau protein was immunohistochemically stained in 3 patients. Results The muscle enzymes in all 7 patients were elevated, but not more than 8 times the upper limit of normal. Electromyography showed 5 cases of myogenic damage, 2 cases of neurogenic damage. All patients with skeletal muscle pathological changes mainly intramuscular inflammatory cell infiltration, muscle fiber diameter variation and edge vacuoles (incidence rate of 2% to 10%), 5 cases of irregular red edge fibers, 3 cases of cytochrome C oxidase Dyeing negative muscle fibers. 5 cases of electron microscopy were present in the tube inclusion body. Three cases of immunohistochemical staining showed tau protein deposition in muscle fibers. Conclusion sIBM is the most obvious damage to the quadriceps femoris, often associated with other senile diseases. Tau protein in muscle fibers can also be used as one of the diagnostic criteria of the disease.