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目的探讨强直性肌营养不良症(DM)骨骼肌病变的病理学特点。方法选择19例经临床和肌电图确诊的强直性肌营养不良症患者为研究对象,骨骼肌标本采用恒冷冰冻切片和酶组织化学染色方法,在光镜下观察骨骼肌组织的病理学变化特点。结果在HE染色,19例患者的骨骼肌标本均可见不同程度的肌纤维萎缩,但变性坏死肌纤维较少见,有7例患者在肌纤维中可见到肌质块。在组织化学MGT染色中,6例患者肌纤维中可见数量不等的破碎红边纤维(RRF),肌质块在MGT染色上呈深绿色,在NADH染色中肌质块呈深蓝色,较HE染色更易识别。在ATP酶染色中,19例患者有11例存在Ⅰ、Ⅱ型肌纤维分布异常现象,其中9例以Ⅰ型纤维明显占优势,2例以Ⅱ型纤维稍占优势,8例患者肌纤维分布基本正常。结论强直性肌营养不良症的骨骼肌病理改变,除常见的肌核增多内移,肌核聚集成核袋及核链现象以外,肌质块的出现和Ⅰ、Ⅱ型肌纤维分布异常应视为强直性肌营养不良症重要的特征性病理改变,肌肉活检对本病的诊断与鉴别诊断有一定的临床意义。
Objective To investigate the pathological features of skeletal muscle in patients with myotonic dystrophy (DM). Methods Nineteen patients with myotonic dystrophy diagnosed by clinical and electromyography were selected as experimental subjects. Skeletal muscle specimens were examined by light microscopy and histopathology with frozen sections and enzyme-linked immunosorbent assay. Features. Results In HE staining, all the muscle samples from 19 patients showed varying degrees of myofibrillar atrophy, but the degenerative and necrotic muscle fibers were rare. Seven patients showed muscle mass in muscle fibers. In the histochemical MGT staining, a number of broken red edge fibers (RRFs) were seen in the muscle fibers of 6 patients, dark green in MGT staining and dark blue in NADH staining. Compared with HE staining Easier to identify. Among the 19 cases, there were 11 cases of abnormal distribution of type I and type II myofibers in ATPase staining. Among them, type I fibers were predominant in 9 cases, type II fibers were predominant in 2 cases, and muscle fiber distribution was normal in 8 cases . Conclusion The skeletal muscle pathological changes of myotonic dystrophy, in addition to the common myomere increase in the internal migration, muscle nuclei and nucleus aggregation phenomenon, the appearance of muscle mass and type I, Ⅱ abnormal muscle fiber distribution should be considered as Myotonic dystrophy important characteristic pathological changes, muscle biopsy of the diagnosis and differential diagnosis of the disease has some clinical significance.