杆状体肌病合并先天性单一Ⅰ型肌纤维肌病临床及病理报告  

The clinical and pathological analysis of the nemaline myopathy complicated with the uniform type Ⅰ fiber myopa thy

作　　者：袁军辉[1] 胡静[1] 陈俐[2] 梅丽[1] 康增军[1] 郭华[1] 刘彦[1] 

机构地区：[1]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 [2]河北医科大学第三医院儿科,河北石家庄050051

出　　处：《中风与神经疾病杂志》2005年第5期432-433,T0001,共3页Journal of Apoplexy and Nervous Diseases

摘　　要：目的探讨杆状体肌病及单一Ⅰ型肌纤维肌病的临床及病理特点。方法杆状体肌病合并单一Ⅰ型肌纤维肌病患者行组织化学染色病理分析。结果肌肉活检modifiedGomoritrichrome(MGT)染色肌浆内、肌膜下紫色杆状体聚集;ATPase染色(酸性、碱性)肌纤维类型几乎全部为Ⅰ型肌纤维,散在单个Ⅱ型肌纤维。结论肌活检发现特异性杆状体、单一型肌纤维分布是确诊此类疾病的唯一可靠方法。Objective To investigate the characteristic expression of the clinic menifestation and pathology in nemaline myopathy complicated with uniform type Ⅰ fiber myopathy. Methods Histochemical staining,pathological analysis and electron microscope diagnosis were adopted. Results The modified Gomori trichrome （MGT） staining of biopsied muscle showed that purple nemaline bodies accumulated in the sarcoplasm and under the sarcolemma. There were abnormality in the distribution of the fiber types, and nearly all of the fibers were type Ⅰ in the ATPase staining（acidity,alkali）. Conclusion Nemaline body accumulation is the characteristic pathological expression of nemaline myopathy, and can complicate with the uniform type Ⅰ fiber myopathy.

关 键 词：杆状体肌病 单一Ⅰ型肌纤维肌病 肌活检 杆状体 

分 类 号：R746.9[医药卫生—神经病学与精神病学]