散发性包涵体肌炎临床与肌肉病理特点  

Clinical and pathological features of sporadic inclusion body myositis

作　　者：李诗瑶 瞿千千[2] 崔文豪 王孟丽 吕正 周亚光 赵炯博 吕海东[2] LI Shiyao;QU Qianqian;CUI Wenhao(Graduate School,Xinxiang Medical College,Xinxiang 453003,China)

机构地区：[1]新乡医学院研究生院,453003 [2]河南省焦作市人民医院神经内科 [3]河南省焦作市人民医院病理科

出　　处：《临床神经病学杂志》2023年第4期262-266,共5页Journal of Clinical Neurology

基　　金：河南省医学科技攻关计划项目(LHGJ20191340)。

摘　　要：目的探讨散发性包涵体肌炎(sIBM)的临床和肌肉病理特点。方法回顾性分析5例sIBM患者的临床和肌肉病理资料。结果本组5例患者中,男3例,女2例;发病年龄36~68岁,平均49.8岁;出现症状至确诊时间为2~12年,平均6.2年。5例患者均以双下肢无力隐匿起病,症状缓慢进展,逐渐发展为四肢无力。脑神经正常,四肢肌张力减低,腱反射减低或消失,上肢近端肌力Ⅲ~Ⅴ级,远端肌力Ⅲ~Ⅳ级;下肢近端肌力Ⅱ~Ⅴ级,远端肌力Ⅲ~Ⅳ级。5例患者均有不同程度的肌肉萎缩。EMG检查示4例呈肌源性损害,1例呈肌源性和神经源性共存的混合性损害。肌肉病理表现为肌纤维大小不等,可见萎缩和肥大肌纤维,散在有变性坏死肌纤维,伴炎性细胞浸润。5例患者均可见镶边空泡,4例可见炎性细胞侵入非坏死肌纤维现象。淋巴细胞亚群免疫组化染色可见CD8和CD68阳性淋巴细胞浸润。结论sIBM好发于中老年人,除上肢远端指屈肌和下肢股四头肌无力以外,部分患者早期可有下肢远端肌无力。肌肉病理发现肌纤维中有镶边空泡和炎性细胞浸入非坏死肌纤维是确诊sIBM的重要依据。Objective To investigate the clinical and pathological features of sporadic inclusion body myositis(sIBM).Methods The clinical and muscle pathological data of 5 sIBM cases were analyzed retrospectively.Results Among the 5 patients,3 were male and 2 were female.The age of onset was 36-68 years old,with an average age of 49.8 years old.The time from symptom onset to diagnosis was 2-12 years,with an average of 6.2 years.All 5 patients presented with insidious onset of bilateral lower limbs weakness,the symptoms progressed slowly and gradually developed into extremities weakness.Normal cranial nerves,decreased muscle tension of limbs,decreased or disappeared tendon reflexes,the proximal muscle strength of upper limb was gradeⅢ-Ⅴ,and the distal muscle strength was gradeⅢ-Ⅳ;the proximal muscle strength of lower limbs was gradeⅡ-Ⅴ,and the distal muscle strength was gradeⅢ-Ⅳ.All 5 cases showed various degrees of muscle atrophy.Electromyography showed myogenic lesions in 4 cases and mixed myogenic and neurogenic lesions in 1 case.Muscle pathology showed that the size of muscle fibers was various,atrophic and hypertrophic fibers could be found,and degenerative and necrotic muscle fibers were scattered with inflammatory cell infiltration.Rimmed vacuoles were found in all patients,inflammatory cells invaded non⁃necrotic muscle fibers in 4 cases.Immunohistochemical staining of lymphocyte subsets showed infiltration of CD8 and CD68 positive lymphocytes.Conclusions sIBM mainly occurs in middle⁃aged and elderly patients.In addition to the weakness of the flexor digitorum at the distal upper limbs and the quadriceps at the lower limbs,some patients may have weakness of the distal lower limbs in the early stages.The pathology shows rimmed vacuoles in muscle fibers and inflammatory cells infiltrating non⁃necrotic muscle fibers,which is important evidence for the diagnosis of sIBM.

关 键 词：散发性包涵体肌炎 临床表现 病理特点 镶边空泡 

分 类 号：R746[医药卫生—神经病学与精神病学]