线粒体肌病和脑肌病的骨骼肌活检均有线粒体形态异常。用琥珀酸脱氢酶染色后,4％以上的肌纤维外周出现线粒体聚集,称线粒体肌病;累及中枢神经系统的谓之线粒体脑肌病。脑肌病综合征包括:(1)眼肌麻痹、视网膜病变和心脏传导阻滞的Kearns—Sayre综合征;(2)伴有蓬毛样红纤维(RRF)的肌阵挛癫痫;(3)线粒体脑病,乳酸酸中毒和卒中样发作(MELAS)。神经心理学检查的目的,在于确定 Mitochondrial morphology is abnormal in skeletal muscle biopsies of mitochondrial myopathy and encephalomyopathy. After staining with succinate dehydrogenase, more than 4% of myofibers appeared mitochondria around the periphery, called mitochondrial myopathy; involving the central nervous system called mitochondrial encephalomyopathy. Myopathic syndromes include: (1) Kearns-Sayre syndrome of ophthalmoplegia, retinopathy and heart block; (2) myoclonic epilepsy associated with promontory red fibers (RRF); (3) Mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS). The purpose of neuropsychological tests is to determine