NMOSD诊断和治疗

NMOSD诊断和治疗 主要内容 NMOSD定义和历史进展最新诊断标准MRI表现特点鉴别诊断病变主要治疗 TheDefinitionandProgressionofNMOSD Neuromyelitisoptica NMO isaninflammatoryCNSsyndromedistinctfrommultiplesclerosis MS thatisassociatedwithserumaquaporin 4immunoglobulinGantibodies AQP4 IgG WithdeeperunderstandingofNMO thediagnosticcriteriaofNMOalsoevolvedfromtheversionin1999 throughtherevisedonein2006 andfinallytothefirstinternationalconsensuscriteriain2015 ThenewcriteriahaveadoptedthebroadertermofNMOSDtoincludepatientswithlimitedmanifestations Moreover theyhavestratifiedNMOSDintotwotypes NMOSDwithAQP4 IgG NMOSD AQP4 andNMOSDwithoutAQP4 IgGorwithunknownAQP4 IgGstatus NMOSDdiagnosticcriteriaforadultpatients DiagnosticcriteriaforNMOSDwithAQP4 IgG1 Atleast1coreclinicalcharacteristic2 PositivetestforAQP4 IgGusingbestavailabledetectionmethod cell basedassaystronglyrecommended 3 ExclusionofalternativediagnosesDiagnosticcriteriaforNMOSDwithoutAQP4 IgGorNMOSDwithunknownAQP4 IgGstatus1 Atleast2coreclinicalcharacteristicsoccurringasaresultofoneormoreclinicalattacksandmeetingallofthefollowingrequirements a Atleast1coreclinicalcharacteristicmustbeopticneuritis acutemyelitiswithLETM orareapostremasyndromeb Disseminationinspace 2ormoredifferentcoreclinicalcharacteristics c FulfillmentofadditionalMRIrequirements asapplicable2 NegativetestsforAQP4 IgGusingbestavailabledetectionmethod ortestingunavailable3 Exclusionofalternativediagnoses Coreclinicalcharacteristics1 Opticneuritis2 Acutemyelitis3 Areapostremasyndrome episodeofotherwiseunexplainedhiccupsornauseaandvomiting4 Acutebrainstemsyndrome5 SymptomaticnarcolepsyoracutediencephalicclinicalsyndromewithNMOSD typicaldiencephalicMRIlesions6 SymptomaticcerebralsyndromewithNMOSD typicalbrainlesions AdditionalMRIrequirementsforNMOSDwithoutAQP4 IgGandNMOSDwithunknownAQP4 IgGstatus1 Acuteopticneuritis requiresbrainMRIshowing a normalfindingsoronlynonspecificwhitematterlesions OR b opticnerveMRIwithT2 hyperintenselesionorT1 weightedgadoliniumenhancinglesionextendingover 1 2opticnervelengthorinvolvingopticchiasm2 Acutemyelitis requiresassociatedintramedullaryMRIlesionextendingover3contiguoussegments LETM OR3contiguoussegmentsoffocalspinalcordatrophyinpatientswithhistorycompatiblewithacutemyelitis3 Areapostremasyndrome requiresassociateddorsalmedulla areapostremalesions4 Acutebrainstemsyndrome requiresassociatedperiependymalbrainstemlesions SpinalcordMRI acuteLETMlesionassociatedwithacuteTMIncreasedsignalonsagittalT2 weighted standardT2 weighted protondensity orSTIRsequences extendingover3ormorecompletevertebralsegmentsCentralcordpredominance morethan70 ofthelesionresidingwithinthecentralgraymatter GadoliniumenhancementofthelesiononT1 weightedsequences nospecificdistributionorpatternofenhancementisrequired OthercharacteristicfeaturesthatmaybedetectedRostralextensionofthelesionintothebrainstem figure1 DandE Cordexpansion swellingDecreasedsignalonT1 weightedsequencescorrespondingtoregionofincreasedT2 weightedsignal MRIPresentationsofNMOSD SpinalcordMRI chronicLongitudinallyextensivecordatrophy sharplydemarcatedatrophyextendingovermorethan3complete contiguousvertebralsegmentsandcaudaltoaparticularsegmentofthespinalcord withorwithoutfocalordiffuseT2signalchangeinvolvingtheatrophicsegmentOpticnerveMRIUnilateralorbilateralincreasedT2signalorT1gadoliniumenhancementwithinopticnerveoropticchiasm figure1 I K relativelylonglesions e g thoseextendingmorethanhalfthedistancefromorbittochiasm andthoseinvolvingtheposterioraspectsoftheopticnervesorthechiasmareassociatedwithNMO CerebralMRI NMOSD typicalbrainlesionpatterns increasedsignalonT2 weightedMRIsequencesunlessotherwisenoted Lesionsinvolvingthedorsalmedulla especiallytheareapostrema eithersmallandlocalized oftenbilateral orcontiguouswithanuppercervicalspinalcordlesionPeriependymalsurfacesofthefourthventricleinthebrainstem cerebellumLesionsinvolvingthehypothalamus thalamus orperiependymalsurfacesofthethirdventricleLarge confluent unilateral orbilateralsubcorticalordeepwhitematterlesionsLong 1 2ofthelengthofthecorpuscallosumorgreater diffuse heterogeneous oredematouscorpuscallosumlesionsLongcorticospinaltractlesions unilateralorbilateral contiguouslyinvolvinginternalcapsuleandcerebralpeduncleExtensiveperiependymalbrainlesions oftenwithgadoliniumenhancement DifferentialDiagnoses Diverseneurologicaldiseasesincludinginflammatory infectious malignant vascular andhereditaryetiologiescanresemblethephenotypesofNMOSD Nevertheless astheseNMOSDmimicsaredistinctfromNMOSDintreatmentaswellaspathophysiology earlydifferentialdiagnosisandappropriateindividualizedtreatmentwillimprovetheoutcomeofsuchpatients MultiplesclerosisAcutedisseminatedencephalomyelitisIdiopathicacutetransversemyelitisIdiopathicopticneuritisInflammatorydiseasesassociatedwithantibodytomyelinoligodendrocyteglycoproteinSarcoidosisCNSinvolvementinpatientswithsystemicautoimmunediseaseSjogren ssyndrome干燥综合征Systemiclupuserythematous CNSlymphomaNeuro BehetdiseaseSpinalduralarteriovenousfistulaInfectionsSyphilisMiscellaneousinfectionsLeberhereditaryopticneuropathy 治疗 NMOSD治疗应该遵循在循证医学证据的基础上 结合患者的经济条件和意愿 进行早期 合理 治疗目前NMOSD的治疗推荐主要是基于一些小样本临床试验 回顾性研究 以及专家共识并借助其他自身免疫性疾病治疗经验而得出NMOSD的治疗分为急性期治疗 序贯治疗 免疫抑制治疗 对症治疗和康复治疗 急性期治疗 与MS相同 大剂量MTP冲击血浆置换缓解期治疗 与MS不同 硫唑嘌呤利妥昔单抗麦考酚酸莫酯 NMOSD对症治疗 抑郁 depression 草酸艾司 西酞普兰 氟