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TeachingVideoNeuroImages:Anti-IgLON5
Disease
ALong-CoursePresentationWithaResponsetoImmunotherapy
JavierVillacieros-Alvarez,´MD,CarlosManuelOrd´as,MD,GustavoTorres-Gaona,MD,AnaD´ıez-Barrio,MD,Correspondence
CristinaPrieto-Jurczynska,MD,andCarlesGaig,-Alvarez´
******@
Neurology®2021;96:e2901-:
A74-year-oldwomanpresentedwitha5-yearhistoryofprogressivegaitinstabilitywithre-MOREONLINE
currentfalls,dysphonia,dysphagia,andsleepproblems,whichincludedinsomnia,breathing
Video
difficulties,andcomplexvocalizationsduringsleep(talking,singing,laughing).Examination
showedbilateralptosis,upwardgazepalsy,hoarseness,dysprosody,andgaitinstability
Teachingslides
(video1).Noparkinsonism,chorea,ordementiawasdetected.
-polysomnography
-IgLON5antibodieswere
positiveinCSFandserumbycell-basedassayofHEKcellstransfectedwithIgLON5and
-
DQB1*05:01.
ImmunotherapywithIVmethylprednisolone1g/dfor5daysandimmunoglobulinsresultedin
improvementofgaitandspeechoveramonth(video1).Asecondcourseofimmunoglobulins
providedclinicalstability.
Anti-IgLON5diseaserepresentsaparadigmofautoimmuneneurodegenerationwithcore
featuresofaspecificsleepdisorder,bulbarsymptoms,
StudyFunding
Notargetedfundingreported.
Disclosure
disclosures.
FromtheDepartmentofNeurology(.-A.,´.,.-G.,.-B.,.-J.),HospitalUniversitarioReyJuanCarlos,Mostoles;´DepartmentofNeurology(.-A.,´.,.-G.,.-B.,
.-J.),HospitalUniversitarioInfantaElena,Valdemoro;DepartmentofNeurology(.-A.,´.,.-G.,.-B.,.-J.),HospitalGeneraldeVillalba,ColladoVillalba,Madrid;and
DepartmentofNeurology(.),HospitalClinic,Barcelona,Spain.
.
Copyright©2020AmericanAcademyofNeurologye2901
Copyright©.
AppendixAuthorsAppendix(continued)
NameLocationContributionNameLocationContribution
JavierHospitalUniversitarioReyDesignedandconceptualizedCristinaHospitalUniversitarioReyMajorroleinacquisitionof
Villacieros-JuanCarlos;Hospitalthestudy,majorroleinPrieto-JuanCarlos;Hospitaldata,criticalreviewofthe
Alvarez,´MDInfantaElena;Hospitalacquisitionofdata,draftedtheJurczynska,InfantaElena;Hospitalmanuscriptforintellectual
GeneraldeVillalba,Madrid,manuscriptfortheintellectualMDGeneraldeVillalba,Madrid,content
Spaincontent,literaturereviewSpain
CarlosHospitalUniversitarioReyMajorroleinacquisitionofCarlesGaig,HospitalCl´ınic,Barcelona,Majorroleinacquisitionof
ManuelJuanCarlos;HospitalInfantadata,criticalreviewofthePhDSpaindata,criticalreviewofthe
Ord´as,MDElena;HospitalGeneraldemanuscriptforintellectualmanuscriptforintellectual
Villalba,Madrid,Spaincontentcontent
GustavoHospitalUniversitarioReyMajorroleinacquisitionof
Torres-JuanCarlos;HospitalInfantadata,criticalreviewofthe
Gaona,MDElena;HospitalGeneraldemanuscriptforintellectual
Villalba,Madrid,Spaincontent
References
AnaD´ıez-,GrausF,ComptaY,-IgLON5disease.
Barrio,MDJuanCarlos;HospitalInfantadata,criticalreviewoftheNeurology2017;88:1736–1743.
Elena;-Garc´ıaP,Mena-V´azquezN,EstivillTorr´usG,Serrano-
Villalba,Madrid,Spaincontenttoimmunotherapyinanti-IgLON5disease:
2020;141:263–270.
e2902Neurology|Volume96,Number23|June8,
Copyright©.
TeachingVideoNeuroImages:Anti-IgLON5Disease:ALong-CoursePresentation
WithaResponsetoImmunotherapy
JavierVillacieros-Álvarez,CarlosManuelOrdás,GustavoTorres-Gaona,etal.
Neurology2021;96;e2901-e2902PublishedOnlinebeforeprintDecember22,2020
ThisinformationiscurrentasofDecember22,2020
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