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池田修一等报告伴发肌原性和神经原性肌萎缩的慢性进行性眼外肌麻痹的1家系。发现两代人中有5名患者,存活2名,对其中1例进行了详细的检查。中年以后以两眼不能闭合发病,以后又出现眼外肌、咽、喉、四肢肌等的无力、麻痹和萎缩。肌电图呈神经原性改变,肌活体组织检查呈肌原性改变,未见线粒体的异常。作者根据有无线粒体异常,将慢性进行性眼外肌麻痹分为两大类,又将无线粒体异常病例根据其临床表现、肌电图、肌活体组织检查分为。(1)仅有肌原性改变者,(2)仅有神经原性改变者,(3)兼有肌原性和神经原性改
Ikeda Xiuyi and other reports of myogenic and neurogenic muscle atrophy chronic progressive extraocular muscle paralysis in a pedigree. Five of the two generations were found to have survived two and one of them was examined in detail. After middle age can not close the two eyes with onset, and later appeared extraocular muscles, pharynx, throat, limb muscle weakness, paralysis and atrophy. EMG showed neurogenic changes, muscle biopsy showed myogenic change, no abnormal mitochondria. Based on the presence of mitochondrial abnormalities, the authors divided the chronic progressive extraocular muscle paralysis into two categories, and classified the cases of abnormal mitochondria based on their clinical manifestations, EMG, and muscle biopsy. (1) those with only myogenic changes, (2) those with only altered neurogenicity, (3) both myogenic and neurogenic modifications