中央轴空病2例临床及病理分析  

Clinical and pathological analysis of central core disease in 2 cases

作　　者：殷飞[1] 江新梅[1] 胡静[2] 李娜[2] 

机构地区：[1]吉林大学第一医院神经内科,吉林长春130021 [2]河北医科大学第三医院神经肌病科,神经肌病实验室,河北石家庄050051

出　　处：《中风与神经疾病杂志》2006年第5期538-540,插页,共4页Journal of Apoplexy and Nervous Diseases

摘　　要：目的探讨中央轴空病的临床及病理特点。方法开放式骨骼肌活检,组织化学染色,病理分析。结果细胞色素C氧化酶、琥珀酸脱氢酶、还原型辅酶、单磷酸腺苷脱氨酶染色,大量肌纤维中均可见中央轴空现象,ATPase染色(酸性、碱性)肌纤维类型分布异常,几乎全部为型肌纤维。结论中央轴空现象是中央轴空病的特征性病理改变,可合并单一型肌纤维肌病;骨骼肌活检、病理检查是确诊本病的重要手段。Objective To investigate the clinical and pathological features of central core disease(CCD). Methods Skeletal muscle open biopsies,histochemical stains and the pathological analysis were performed. Results The Cytochrome C oxidase(CCO),Succinate dehydrogenase(SDH),Nicotinamide adenine dinucleotide -tetrazolium reductase(NADH-TR),Adenosine monophosphate(AMP deaminase) staining were perfomed,and the central core were observed in many muscle fibers. There are abnormal in the distribution of the fiber types,and almost all of the fibers were type I in the ATPase staining(acidity,alkali). Conclusion Central core is the characteristic pathological expression in the central core disease,and can complicate with the uniform type I fiber myopathy. Skeletal muscle biopsy,pathological analysis is an important method to make a definite diagnosis on this disease.

关 键 词：中央轴空病 骨骼肌活检 中央轴空现象 单一Ⅰ型肌纤维肌病 

分 类 号：R746[医药卫生—神经病学与精神病学]