Sokolina N A, Il'ina N A, Potomskaia L Z, Korolev V V
Zh Nevropatol Psikhiatr Im S S Korsakova. 1980;80(11):1607-11.
Data of a clinico-morphological examination of 4 patients with the ophthalmoplegic form of myopathy are presented. An electronmicroscopic examination has made it possible to disclose the primary morphological defect in the mitochondria of the patients' muscles. The mitochondrial pathology manifested itself in an increase of the number of these organelles, changes of their shape and size, structural abnormalities and presence of inclusions. The pathology of the mitochondria in muscular fibres with normal structure of the latter's contractile apparatus, gradual destruction of the mitochondria, and deepening of the destructive changes in the mitochondria in parallel to the lysis of the myofibrillary apparatus give one grounds to speak about the primary character of the mitochondrial pathology in those patients, and to regard this disease as a variant of mitochondrial myopathies.
本文呈现了4例眼肌麻痹型肌病患者的临床形态学检查数据。电子显微镜检查揭示了患者肌肉线粒体中的原发性形态学缺陷。线粒体病理学表现为这些细胞器数量增加、形状和大小改变、结构异常以及存在包涵体。在肌纤维收缩装置结构正常的情况下，线粒体出现病变，线粒体逐渐被破坏，且线粒体的破坏变化随着肌原纤维装置的溶解而加深，这使人们有理由认为这些患者线粒体病理学具有原发性特征，并将这种疾病视为线粒体肌病的一种变体。
