线粒体脑肌病伴高乳酸血症和脑卒中样发作的临床、影像学及病理学特点(附1例报告).pdfVIP

·377· ·学术交流· 线粒体脑肌病伴高乳酸血症和脑卒中样发作的 临床、影像学及病理学特点【附1例报告) 黄光，赵建功，唐煜，王伟，高金力，刘兴洲 【摘要】 目的探讨线粒体脑肌病伴高乳酸血症和脑卒中样发作(MELAS)的临床、影像学及病理特点。 方法 回顾性分析l例MELAS患者的临床资料。结果本例患者临床表现为以卒中样发作为主，有时伴癫 痢发作、精神症状等，每次均为急性起病，经过治疗症状可有不同程度的缓解。头颅MRI提示以皮质受损为 主，病灶起自右侧颞叶，每次发病的病损部位按顺时针方向发展，新的病灶总是累及从未发病的部位。其肱二 头肌活检，病理结果示出现破碎红纤维。结论MELAS患者临床表现多样，影像学改变明显，且对诊断帮助 较大，但确诊仍需肌肉活检。 【关键词】线粒体脑肌病；高乳酸血症；卒中样发作；影像学；肌肉活检 【中图分类号】R742 【文献标识码】A 【文章编号】1004-1648(2009)054)377-02 and featuresofmitochondrial wimlacticacidosisand Clinical．imagingpathological encephalomyopathy stroke-like of1 aL episodes(reportcase)HUANGGuang，ZHAOJian-gong，TANGYu，et Departmentof Medical Neurology，FuzingHospital，CapitalUnwersay，Being100038，China To the and featuresofmitochondrial Abstract：Objectivestudy clinical，imagingpathological withlacticacidosisandstroke-like Theclinicaldataofone eneephalomyopathy episodes(MELAS)．Methods MELASwas The clinicalmanifestationswerestroke-like patientretrospectivelyanalyzed．Resultsmajor episodes， were onsetofeachtimeWaSacuteandthe relievedindifferent seizure，mentaldisorder，etc．The symptoms degree after MRIshowed incerebralcortex．The wasthat imagefinding treating．Craniocerebralabnormalitymostly special and totheleftcerebrallobeinaclockwisedirection． thediseasebrokeoutfromthe lobe righttemporal developed Newlesionswereaccumulatedandextendedtothose wherethediseasedidnotoccurbefore．Muscular positions ofthis re