eficiency of3respiratory plexes. There were no significant differences between biochemistry and genotype/clinical type.MT-TLl andMT-ND were mon mutation 2enes inrespiratory plex deficiency,while MT-TLl,MT-CYB andMT-ND3 were the lnon mutation genes inrespiratory plex III、and IV deficiency. Affected systems inthe patients with respiratory plex deficiency included skeletalmuscle,brain andendocrine system.Affected systems intheDatients with respiratory plex IV deficiency included brain,skeletal muscle and endocrine.Howerver,the involvement ofskeletalmuscle and brainwas equivalent in MELAS patients withrespiratory plex IIIandVdeficiency. Conclusion:MELAS isMitochondrial encephalomyopathy characterized with neurological manifestations and radiography as well as pathological features.It is 9 万方数据