melas型线粒体脑肌病的mri诊断[1]

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1、-144-ChinJClinNeurosurg，Mar2009，Vol14，No3●论著●MELAS型线粒体脑肌病的MRI诊断李国雄阳昱恒＊王凤鸣＊＊【摘要】目的探讨合并乳酸血症和卒中样发作的线粒体脑肌病（MELAS）的磁共振成像（MRI）影像学特点。方法收集经临床病理证实的MELAS型线粒体脑肌病共6例，回顾性分析其MRI和磁共振波谱（MRS）资料。结果脑MRI检查，MELAS表现为大脑半球各叶大小不等片状病灶；病变位于脑皮质区，病灶的分布与脑血供分布不一致；自旋回波T1加权像呈低信号、T2加权像呈高信号；扩散加权成像（DWI）呈高信号。MRS分析显示病灶区见典

2、型的乳酸盐峰，N-乙酰天门冬氨酸盐/肌酸值正常或略降低。扩散张量成像（DTI）显示病灶区脑皮质下白质纤维束破坏、中断、稀少。结论MELAS型线粒体脑肌病的病变形态、分布具有特征性，常规MRI与DWI、DTI及MRS等磁共振技术，对MELAS的定性诊断具有很高的价值。【关键词】线粒体脑肌病；磁共振成像；诊断【文章编号】1009－153X（2009）03-0144－04【文献标识码】A【中图分类号】R742．8+9;R685.5DiagnosisofMitochondrialEncephalomyopathywithLacticAcidosisandStrokebyM

3、RILIGuo-xiong＊,YANGYu-heng,WANGFeng-ming.＊DepartmentofNuclearMedicine,WuhangeneralHospital,GuangzhouCommand,PLAWuhanHubei430070,China【Abstract】ObjectiveToinvestigatetheappearancesofmitochondrialencephalomyopathywithlacticacidosisandstroke(MELAS)onMRI.MethodMRIandmagneticresonancespect

4、roscopy(MRS)dataof6patientswithMELASconfirmedbypathologicalexaminationwereanalyzedretrospectively.ResultsThemultipleflakylesionswithvariedsizewereobservedindifferentlobeofbrainonbrainMRIofallthepatientswithMELAS.Thelesionsmainlylocatedinthecerebralcortexes,andnotdistributedinaccordanc

5、ewithcerebralvesselsrun.AllthelesionsshowedlowsignalintensityonT1-weightedimage,andhighsignalintensityonT2-weightedimageanddiffusionweightedimage(DWI).MRSshowedatypicalcrestoflactateinMELASlesions.TheratioofN-acetyl-aspartatetocreatinewasnormalordecreasedslightlyonMRS.Diffusiontensori

6、maging(DTI)showedthatthenervefiberbundlesofthewhitematterunderthecerebralcortexeswerebrokenoffanddegenerated.ConclusionsTheMELASlesionsappearancetheonMRIanddistributionofMELASlesionsinthebrainwerecharacteristic.RoutineMRIandfunctionalMRtechniquessuchasDWI,DTIandMRSareofanimportantvalu

7、etodiagnosisofMELAS.【KeyWords】Mitochondrialencephalomyopathy;Magneticresonanceimaging;Magneticresonancespectrum;Diagnosis线粒体脑肌病指由于线粒体基因或细胞核基因对本病的认识，提高诊断水平。缺失或发生点突变导致的线粒体结构和功能异常,1临床材料引起机体能量代谢障碍,主要累及脑和横纹肌的一类少见疾病[1~5]，以线粒体脑肌病合并乳酸血症和卒1.1一般资料本组收集了我院2006年7月至中样发作(mitochondrialencephalomyopat

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