尽管喉内肌常常在原发性肌营养不良和神经肌疾病中受累,但从活检材料上对其形态学改变的组织学及超微结构详细分析尚未见报道。作者们对取自Steinert氏紧张性肌营养不良、进行性眼咽肌营养不良、Duchenne氏肌营养不良及类肿瘤性肌病患者的5例声带肌活检标本及1例志愿者的对照标本进行了组织学及超微结构研究,所见如下: 〔紧张性肌营养不良〕位于肌纤维周边的密染核暗度增加,通常形成线状排列的短链。朋纤维横纹很好地被保存。无间质炎症反应或血管外周套的出现。肌纤维比对照者为薄。电镜下显示横小管系统明显扩张,很多部位和扩张的滑面内质网终池相交通而形成一个扩张三联体(triad)的连续网络。线粒体大而细长,并有在肌纤维周 Although the intrathoracic muscles are often involved in primary muscular dystrophy and neuromuscular disease, detailed analysis of their histological and ultrastructural changes on biopsy material has not been reported. The authors performed 5 cases of vocal cord muscle biopsy specimens from Steinert’s tense muscular dystrophy, progressive ophthalmopharyngeal muscular dystrophy, Duchenne’s muscular dystrophy and neoplastic myopathies and 1 volunteer control specimen Histology and ultrastructure studies, as follows: [Tension muscular dystrophy] Dense nuclei located around the muscle fibers increase in darkness, often forming short chains in a linear arrangement. Peer fiber stripes are well preserved. Stroke-free inflammatory response or the appearance of a perivascular sheath. Muscle fibers were thinner than controls. Electron microscopy revealed a significant expansion of the transverse tubule system, with many sites communicating with the expanded endplate of the sliding endoplasmic reticulum to form a continuous network of dilated triads. Mitochondria large and slender, and there in the muscle fiber week