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1、PediatricNeurology47(2012)209e212ContentslistsavailableatScienceDirectPediatricNeurologyjournalhomepage:www.elsevier.com/locate/pnuCaseReportRituximabinSevereSeronegativeJuvenileMyastheniaGravis:ReviewoftheLiteratureRoshanKoulMD,AmnaAlFutaisiMD,ReemAbdwan
2、iMD*DepartmentofChildHealth,SultanQaboosUniversityHospital,Muscat,OmanarticleinformationabstractArticlehistory:Myastheniagravisisanautoimmuneneuromusculardisordercausedbycirculatinganti-Received14February2012bodiesthatblockacetylcholinereceptorsattheposts
3、ynapticneuromuscularjunction.Accepted23May2012Awiderangeofsymptomatictherapywithacetylcholinesteraseinhibitorsandimmuno-therapysuchascorticosteroids,azathioprine,cyclosporine,mycophenolatemofetil,plas-mapheresis,andintravenousimmunoglobulinhasbeenusedinth
4、etreatmentofmyastheniagravis,withvariableresponses.However,mostmodalitiesoftreatmentinvolvedelayedonsetofaction.Wedescribeachildwithsevere,life-threateningseronegativemyastheniagraviswhorepeatedlyfailedextubationandrespondeddramaticallytorituximab.Sheachi
5、evedcompleteandsustainedremissionformorethan9months,withgradualreductioninsteroiddosewithoutanysideeffects.Advancesinthetreatmentofmyastheniagravishavereducedmortalityandmorbidityandimprovedthequalityoflifeinthesepatients.ó2012ElsevierInc.Allrightsreserve
6、d.IntroductionRituximabisachimericmonoclonalantibodythattargetstheCD20antigenonBlymphocytes,andmodu-MyastheniagravisisthemostcommonacquiredprimarylatesB-cellactivation.CD20isa33-kDaproteinexpressedneuromusculardisorder.Ininfantsandchildren,threebyallmatur
7、eBcells,butnotonpre-Bmatureordiffer-formsofmyastheniagravisexist:juvenilemyastheniaentiatedplasmacells.Rituximabhasbeenusedaspartofgravis,congenitalmyastheniagravis,andtransientneonatalthestandardtherapyfornon-Hodgkinlymphomaandformyastheniagravis.Juvenil
8、emyastheniagravisaccountsforanumberofautoimmunediseasessuchassystemiclupusabout10-15%ofallcasesofmyastheniagravis[1].However,erythematosus,autoimmunehemolyticanemia,rheuma-inChinaandJapan,ahigherfrequencyofjuvenilem
