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目的:总结强直性肌营养不良(DM)的肌肉病理特点。方法:分析8例强直性肌营养不良患者肌肉活检标本的光镜和电镜检查结果。结果:光镜下病理改变,8例标本示肌纤维萎缩,Ⅰ、Ⅱ型肌纤维均受累,以Ⅰ型肌纤维萎缩为主,且均有核内移,核链形成。电镜下病理改变:4例为肌节不清晰,4例肌丝排列紊乱,3例肌丝溶解、肌纤维坏死,3例肌膜下肌浆块。结论:DM的病理特征为核内移,核链形成,以Ⅰ型纤维为主的肌萎缩,伴有Z带破坏,肌纤维坏死,肌浆块和肌膜微小缺损等。
Objective: To summarize the muscle pathological features of myotonic dystrophy (DM). Methods: Light and electron microscopic examination of muscle biopsy specimens from 8 patients with myotonic dystrophy were analyzed. Results: Pathological changes were observed under light microscope. Myofibrillar atrophy was found in 8 cases. Type Ⅰ and Ⅱ myofibers were all involved. Type Ⅰ myofibers were atrophic, and all had nuclear translocation and nuclear chain formation. Electron microscopy pathological changes: 4 cases of unclear sarcomere, 4 cases of myofilaments arranged in disorder, myofilament in 3 cases, muscle fiber necrosis, 3 cases of subcondiia sarcoplasmic reticulum. Conclusion: The pathological features of DM are nuclear shift and nuclear chain formation. Muscle atrophy is characterized by type Ⅰ fibers accompanied by destruction of Z - band, muscle fiber necrosis, tiny sarcoplasmic reticulum and tiny defects of.