Defanti C A, Valenti G
Riv Patol Nerv Ment. 1978 May-Jun;99(3):164-77.
Two patients developed Progressive External Ophthalmoplegia at the age of 20 and 50 years respectively. There was no clinical invovlement of either skeletal muscles or the CNS. Electron microscopy of the lateral rectus muscle showed in one case regressive changes in the myofibrillary apparatus and in the mitochondria. Light microscopy of the quadriceps muscle in the other case showed only mild non-specific myopathic changes. EMG of the ocular muscles suggested a primary muscular damage in both cases.
两名患者分别在20岁和50岁时出现进行性眼外肌麻痹。骨骼肌和中枢神经系统均无临床受累表现。其中一例患者外直肌的电子显微镜检查显示肌原纤维装置和线粒体有退行性改变。另一例患者股四头肌的光学显微镜检查仅显示轻度非特异性肌病改变。两例患者眼肌的肌电图均提示原发性肌肉损伤。
