MELAS型线粒体脑肌病的临床_影像学和肌肉病理分析.docVIP

——————————————————————————————————————————————— MELAS型线粒体脑肌病的临床_影像学和肌肉病理分析 ?1??论著? MELAS型线粒体脑肌病的临床、影像学和肌肉病理分析 吕海东1　李建章2　张三军1　瞿千千1　钱　琪1　秦东香1 ) ) ) ) ) ) 1)河南焦作市人民医院神经内科　焦作　454002　2)郑州大学第二临床医院神经内科　郑州　450014 【摘要】　目的　探讨MELAS型线粒体脑肌病的临床表现、影像学特点和肌肉组织病理学改变,提高人们对本病的认识。方法　回顾性分析5例MELAS型线粒体脑肌病的临床表现、脑影像学改变(MRI和CT),点。结果　MELAS 型线粒体脑肌病的主要临床表现为局灶性或全身性癫 、、认知功 能障碍、脑卒中样发作、血乳酸水平升高等。、,脑表现为长T1、长 T2信号,部分患者头颅CT可见基底节钙化。(RRF),2例行电镜检查 均可见异常线粒体聚集。结论　MEL碍综合征。,,结合骨骼肌活检病理检查发现RRF,。 【关键词】　MEL;MRI;CT;肌肉病理学 【中图分类号】　R74619　　【文献标识码】　A　　【文章编号】　167325110(2009)0320001203 Analysisonclinic,neuroimagingandmyopathologicalfeaturesofMELAS　LüHaidong,LiJianzhang,Zhang Sanjun,etal1DepartmentofNeurology,theFirstPeople’sHospitalofJiaozuo,Jiaozuo454002,China 【Abstract】Objective　Toinvestigatetheclinic,neuroimagingandmyopathologicalfeaturesinthepatientswithMELAS typeofmitochondrialencephalomyopathy1Methods　Theclinicalmanifestation,neuroimaging(MRIandCT)andmyopatholog2icalfeaturesof5patientswithMELASwereretrospectivelyanalyzed1Results　Themainclinicalfeatureswerecharacterizedbyfocalorgeneralizedseizures,visualdisturbance,sensorineuraldeafness,dementia,stroke-likeepisodes,lacticacidosisandsoon1Neuroimagingshowedmultiplelesionprimarilyingraymatterofoccipital,parietal,temporalcortex,whichwasexpressedbytheabnormallongerT1andT22weightedsignalsonMRI1CTshowedcalcificationinbasalganglia1Musclebiopsyshowedredraggedfiber(RRF)in5patients1Conclusion　MELAStypeofmitochondrialencephalomyopathyisacomplexdiseasewithdisorderofenergymetabolism1Stroke-likeepisodesandlacticacidosisaretheprominentsymptomsinMELAS1Accordingtoclinicalmanifestationsandneuroimagingfeatures,MELAScanbedefinedincombinationwithmusclebiopsy1 【Keywords】　MELASsyndrome;Magneticresonanceimaging;Computedtomography;Myopathology 　　MELAS型线粒体脑肌病(mitochondrialen2cephalopathylacticacidosisandstrokelikeepi2sodes)是一种以高乳酸血症和卒中样发作为特征的 均有不同程度的智力低下。临床曾诊断病毒性脑炎3例,癫 1例,脑梗死1例。 112　辅助