melas型線粒體腦肌病的mri診斷[1]

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1、-144-ChinJClinNeurosurg，Mar2009，Vol14，No3●論著●MELAS型線粒體腦肌病的MRI診斷李國雄陽昱恒＊王鳳鳴＊＊【摘要】目的探討合并乳酸血癥和卒中樣發(fā)作的線粒體腦肌?。∕ELAS）的磁共振成像（MRI）影像學(xué)特點(diǎn)。方法收集經(jīng)臨床病理證實(shí)的MELAS型線粒體腦肌病共6例，回顧性分析其MRI和磁共振波譜（MRS）資料。結(jié)果腦MRI檢查，MELAS表現(xiàn)為大腦半球各葉大小不等片狀病灶；病變位于腦皮質(zhì)區(qū)，病灶的分布與腦血供分布不一致；自旋回波T1加權(quán)像呈低信號、T2加權(quán)像呈高信號；擴(kuò)散加權(quán)成像（DWI）呈高信號。MRS分析顯示病灶區(qū)見典

2、型的乳酸鹽峰，N-乙酰天門冬氨酸鹽/肌酸值正?；蚵越档?。擴(kuò)散張量成像（DTI）顯示病灶區(qū)腦皮質(zhì)下白質(zhì)纖維束破壞、中斷、稀少。結(jié)論MELAS型線粒體腦肌病的病變形態(tài)、分布具有特征性，常規(guī)MRI與DWI、DTI及MRS等磁共振技術(shù)，對MELAS的定性診斷具有很高的價(jià)值?！娟P(guān)鍵詞】線粒體腦肌病；磁共振成像；診斷【文章編號】1009－153X（2009）03-0144－04【文獻(xiàn)標(biāo)識碼】A【中圖分類號】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線粒體腦肌病指由于線粒體基因或細(xì)胞核基因?qū)Ρ静〉恼J(rèn)識，提高診斷水平。缺失或發(fā)生點(diǎn)突變導(dǎo)致的線粒體結(jié)構(gòu)和功能異常,1臨床材料引起機(jī)體能量代謝障礙,主要累及腦和橫紋肌的一類少見疾病[1~5]，以線粒體腦肌病合并乳酸血癥和卒1.1一般資料本組收集了我院2006年7月至中樣發(fā)作(mitochondrialencephalomyopat

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