Anti-NMDAR Encephalitis | Center for Autoimmune Neurology

Anti-NMDA receptor encephalitis is a neurologic disease first identified by Dr. Josep Dalmau and colleagues at the University of Pennsylvania in 2007. Home Personnel PatientCare Research DiseaseInformation  Classification AssociatedSyndromes Diagnosis Treatment Causes Anti-NMDAREncephalitis Resources&Links DiseaseInformationAnti-NMDAREncephalitis Anti-NMDAREncephalitis Whatisanti-NMDAReceptorEncephalitis? Anti-NMDAreceptorencephalitisisaneurologicdiseasefirstidentifiedbyDr.JosepDalmauandcolleaguesattheUniversityofPennsylvaniain2007. Itisanautoimmunedisease,wherethebodycreatesantibodiesagainsttheNMDAreceptorsinthebrain. Theseantibodiesdisruptnormalbrainsignalingandcausebrainswelling,orencephalitis. Itcanaffectbothmenandwomen,howeverismorecommonamongwomen. Itprimarilyaffectstheyoung,includingchildrenandyoungadults. Somepatientsalsohaveatumorassociatedwiththisdisease;themostcommontypeisanovarianteratomainwomen.ThenameofthisdiseasedescribesanimmuneattackontheNMDAreceptorsandcanbeexplainedasfollows: Anti-AutoimmuneResponse NMDAReceptor-againstNMDAreceptorsinthebrain Encephalitis-causingswellinganddisruptioninbrainsignaling Signs&Symptoms Anti-NMDAreceptorencephalitiscausesawiderangeofsymptomsvaryinginseverity.  Patientstypicallystartwithlessseveresymptoms,andthenrapidlyprogresstoaconditionrequiringhospitalization. Thelistbelowincludesthemostcommonsymptoms. Mostpatientswiththisdiseaseexhibitnearlyallofthesesymptoms;itisextremelyuncommonforpatientstohaveonlyoneortwo. Behavior(paranoia,hallucinations,aggression,etc.) Cognition MemoryDeficit SpeechDisorder LossofConsciousness MovementDisorder(rhythmicmotionswitharmsorlegs,abnormalmovementswiththefaceormouth) Seizures AutonomicDysfunction Titulaer,et.al.,2013 Diagnosis Anti-NMDAreceptorencephalitisisoftenfirstidentifiedthroughclinicalsymptoms. Diagnosisisconfirmedthroughlabtestingofcerebralspinalfluid(CSF)orbloodserum. Thistestingisavailableatavarietyofcommerciallabs,includingtheHospitaloftheUniversityofPennsylvania(1-800-PENNLAB). Treatment Treatmentofanti-NMDAreceptorencephalitiscanvaryaccordingtopatient,buttypicallyincludesacombinationofthefollowing: FirstLineTreatment: Tumorremoval(iftumorispresent) Steroids PlasmaExchange(plasmapheresis) Intravenousimmunoglulin(IVIG) SecondLineTreatment: CellCept Rituximab Cytoxan Recovery Recoveryisslowandtypicallyoccursinreverseofsymptomonset. Themostseveresymptomstypicallyresolvefirstwhilethecognitive,behavioral,andmemoryproblemstakelongertoresolve. Mostpatientswillmakeafullrecoverywithintwoyearsofdiseaseonset.  Predictorsofpositiveoutcomesincludepresenceofatumor,quickdiagnosis,andaggressivetreatmentincludingsecondlinetherapies.  Causes Likemostautoimmunediseasesitisnotknownwhatexactlywhatcausesanti-NMDARencephalitis. Therearelikelymultipleenvironmentalandgenetictriggersthatmakepeoplemoresusceptibletothisdisease,somethingwehopetopinpointwithfutureresearch. Top ©TheTrusteesoftheUniversityofPennsylvania|Sitebestviewedina supportedbrowser.| ReportAccessibilityIssuesandGetHelp| PrivacyPolicy| SiteDesign:DARTWebTeam.