MELAS是指一种以线粒体肌病、脑病、乳酸中毒和反复卒中样发作为表现的综合征,可以是家族性的,也可以散发.本征可能存在线粒体氧化磷酸化障碍,其生化原因繁多,CT无特异性改变.本文MRI发现该病有独特的病理改变,现报告如下.例1,女性,青春期患有偏头痛,运动时步态不稳.16岁开始双上肢肌阵挛,20岁听力缓慢进行性损害.18岁出现癫痫大发作,22岁发生癫痫持续状态,发作后有皮质盲,2周后视觉恢复,其母52岁病逝,有多发性中风,进行性痴呆,共济失调,耳聋病史,尸检发现骨骼肌锯齿状红纤维(RRF).22岁首次就诊,化验血清乳酸升高(2.3mmol/L),肌肉活检发现RRF,血管壁线粒体形态异常.CT和MRI仅见小脑轻度萎缩.癫痫持续状态后脑MRI复查显示左枕皮质广泛的高强度信号,其下方白质大部分 MELAS is a syndrome characterized by mitochondrial myopathy, encephalopathy, lactic acidosis and recurrent stroke, and may be familial or sporadic. There may be a mitochondrial oxidative phosphorylation disorder intrinsic to MELAS, with many biochemical causes, CT no specific changes in this article MRI found that the disease has a unique pathological changes, are reported as follows .Example 1, women, adolescent migraine headaches, gait instability during exercise .16 years old double upper limb myoclonus, 20 years old Hearing slow progressive loss of epilepsy onset at 18 years of age, 22-year-old status epilepticus, cortical blindness after the onset of visual recovery after 2 weeks, the mother died of 52-year-old, multiple strokes, progressive dementia, ataxia , History of deafness, autopsy found serrated red fiber (RRF) .22 years old first visit, serum lactate test (2.3mmol / L), muscle biopsy found RRF, vascular wall mitochondrial morphology abnormalities. CT and MRI showed only cerebellar light Degrees of atrophy .A review of epileptic brain MRI showed a wide range of high-intensity signals in the left occipital cortex, most of the white matter below it