MSfollowingpoliomyelitis53lIIIIllllliI11 ID: 946293
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52Chroni,Howard,Panayiotopoul0s,SpencerMultiplesclerosispresentingaslatefunctionaldeteriorationafterpoliomyelitisEChroni,RSHoward,CPPanayiotopoulos,GTSpencerLane-FoxUnitandDepartmentsofNeurophysiologyandNeurology,Guy'sandSt.Thomas'HospitalTrust,StThomas'Campus,London,UKEChroniRSHowardCPPanayiotopoulosGTSpencerCorrespondencetoDrRSHoward,DepartmentofNeurology,Guy'sandStThomas'Trust,StThomas'Campus,LondonSEl7EH,UKAccepted27July1994SummaryWedescribefivepatientswithpreviouspoliomyelitiswhodevelopedmultiplesclerosis(twolaboratorysupporteddefiniteandthreeclinicallydefinite).Theinitialsymptomsoffunctionaldeteriora-tiondevelopedameanof30yearsfollow-ingpoliomyelitis.Initialfunctionaldeteriorationwasduetoprogressivelimbweaknessorimpairedmobilityandinthreecasesthisledtoaninitialdiagnosisofpost-poliomuscularatrophy.Theclinicaldiagnosisbecameapparentwiththesubsequentdevelopmentofcharac-teristicclinicalfeatures,includingopticnerve,brainstem,cerebellum,andspinalcordinvolvement.Theoccurrenceofmultiplesclerosisinthesepatientsemphasisesthatlatefunctionaldeterior-ationmaybeapparentlyunrelatedtopreviouspoliomyelitis.Furthermore,characteristicclinicalfeaturesmaybemaskedbytheseverepre-existingneuromuscularandorthopaedicimpair-mentleadingtodiagnosticdelayandconfusionwiththeill-definedclinicalsyndromeofprogressivepost-poliomus-cularatrophy.Keywords:multiplesclerosis,poliomyelitisIntroductionManypatientswithpreviouspoliomyelitisdeveloppost-poliosyndromes(PPS)inwhichlatefunctionaldeteriorationfollowsaperiodofrelativestability.'Newweaknessoratrophymayoccurintheabsenceofanyunderlyingcauseinprogressivepost-poliomuscularatrophy(PPMA)whichisaformofPPS.However,becauseoftheirexistingdisability,post-poliopatientsarealsoparticularlysuscep-tibletotheeffectsofintercurrentorthopaedic,neurological,respiratoryorgeneralmedicalfactors.2Diagnosticdifficultycanarisebecausethecharacteristicfeaturesofsecondarycausesofdeteriorationmaybemaskedbythepre-existingimpairmentsleadingtoanerroneousdiagnosisofPPMA.Wereportfivecasesfromapersonalseriesof589patients(GTS)inwhompost-poliodeteriorationwasduetomultiplesclerosis(MS).CasereportsPatient1A25-year-oldwomandevelopedpoliomyelitisduringpregnancy.Shewasextensivelyparalysedduringtheacuteillnessbutdidnotreceiveventilatorysupport.Followingrehabilitationshewasleftwheelchair-bound,withsevereupperlimbweaknessandflaccidparaplegia.Attheageof40shedevelopedprogressiveincreasingweaknessoftherighthandandarmmanifestasalossofabilitytofeedherself,combherhairorbrushherteeth.Overthenexteightyearsshehadepisodesofdip-lopia,upperlimbspasticity,weaknessandhyperreflexia.Shewasreferredbecauseofpost-poliodeteriorationatwhichtimeshehadbilateralinternuclearophthalmoplegia,verticalnystagmus,opticdiscpallor,leftrelativeafferentpupillarydefect,bilateralupperlimbspasticityandweaknessinadditiontothepre-existingwasting,spasticityandproprio-ceptivelossintherightleg.Shesubsequentlydevelopedprogressivebulbarfailurebeforeherdeathfromaspirationpneumoniain1986.ThediagnosisofMSwasclinicalandbasedontheoccurrenceofclearepisodesofrelapsingandremittingneurologicalsymptomsandsigns.Patient2A42-year-oldwomanhaddevelopedacuteparalyticpoliomyelitiswhenagedthreemonths.Shewasextensivelyparalysedduringtheacuteillnessandreceivedartificialventila-tionwithanIronLungforsixmonths.Shewasleftwithsevereupperlimbweakness,flaccidparaplegiaandwaswheelchair-bound.Shehadatransientepisodeofpainfulvisuallossfromthelefteyewhenaged25years.Nineyearslatershedevelopedprogressiveweaknessofbotharms,frequencyandurgeincontinence.Inadditiontotheprecedingsevereflaccidweaknessthereweremyoclonicjerksoftherightarmandreducedspinothalamicsensoryappreciationbelowtheclavicleontheleft.Visual-andbrainstem-evokedpotentialswerenormalbuttherewasdelayedcentralmotorconductionbilaterally.Shedevelopedepisodicincreasingweaknessandparaesthesiaofbotharmswithclearfunctionaldeterioration,urinaryincontinenceandchronicconstipation.Inadditiontothesevereupperlimbweaknessandflaccidparaplegiatherewasalowthoracicleveltovibrationsense
andjointpositionsenseappreciation.AdiagnosisofclinicallydefiniteMSwasmade.Magneticresonanceimaging MSfollowingpoliomyelitis53...~~~~~~~~~.....................-.l~~~~~~~~~~~~~~~~~~.........I.I|-~~.........II|_-__l_~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.|ll__-l*li--_I_11~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.......-~~~~~~~~~~~~~~~~~~~~~~..............._III_______________*--11_~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.======_~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..__________|*--l_~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..........lll=======-====_=_l-___I_~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~....I~~~~~~~~~~~~~~~~~~~..._____.........-1l~~~~~~~~~~~~~~~~~~~~~~.====lll__________I-___|_~~~~~~~~~~~~~~~~~~~~~~~~~~~;RFigureMultiplesclerosispresentingaslatefunmctionaldeteriorationafterpoliomyelitis(MRI)showedmultiplehigh-signalperiventri-cularlesionsonT2-weightedimages.Patient3A48-year-oldwomanwasreferredforfurtherinvestigationofpost-poliofunctionaldeterioration.Shehadpoliomyelitisattheageof8yearsandwasleftwithsevereflaccidweaknessoftherightlegwhichwassubse-quentlyamputated.Sheremainedwheelchair-bound.Betweentheagesof39and40shehadthreeepisodesofweaknessoftherightarmandleg.Examinationshowedarightfacialweak-ness,asymmetricalbilateralwastingwithoutfasciculations,variableflaccidweaknesspre-dominantlyinapyramidaldistributionontherightwithpathologicallybriskreflexesandaglobalflaccidweaknessoftheleftleg.Visual-andauditory-evokedpotentialswerenormal,somatosensory-evokedpotentialsshoweddelayfollowingleftarmstimulationandanabsentresponseontheright.Cerebrospinalfluid(CSF)showedpositiveoligoclonalbandsandMRIshowedmultiplesmallfocalareasofhighsignalonT2-weightedimagesinapredomi-nantlyperiventriculardistribution.Patient4A44-year-oldmandevelopedpoliowhenaged18months,leavinghimwithflaccidlegweakness.Hewasreferredattheageof42yearshavingdevelopedintermittentdiplopia,leftfootdropandincreasingincoordinationoftheleftleg.Onexaminationtherewashorizontalnystagmus,milddistalweaknessofthelefthand,left-sidedataxiaanddysdiadochokinesis,absentabdominalreflexes,severeflaccidrightlegweaknessandmoderatepyramidalweak-nessoftheleftlegwithleft-sidedhyperreflexiaandanextensorplantarresponse.Visual-evokedpotentialsshoweddelayedlatencybilaterallyandcentralmotorstimulationshoweddelayinthecentralmotorpathwaysontheleft.MRIofthecervicalcordshowedsignificantnarrowingattheC6-7interspacebutT2-weightedimagesshowedmultiplehighsignallesionsinapredominantlyperiventri-culardistribution.Patient5A43-year-oldwomanhaddevelopedpoliowhenagedfiveyearsandwasleftwithaflaccidparaparesisalthoughshewasabletowalkshortdistanceswithlegcalipers.Attheageof40yearsshedevelopedtransientepisodesofweaknessandnumbnessaffectingtheleftleg.Thefollowingyearshehadanepisodeoftransversemyelitiswithcompletesensorylossbelowthewaistandurinaryincontinenceandfrequency.TherewasaT7sensoryleveltoallmodalities.Therewasdeteriorationintheparaparesisandbothplantarresponseswereextensor.Investigationshowedpositiveoligo-clonalbandsintheCSF,MRIbrainscanshowedmultipleareasofhighsignalinaperiventriculardistributionontheT2-weightedimages.Shehassubsequentlydevelopedanepisodeofleftopticneuritis.DiscussionTheLaneFoxunitisanationalreferralcentreforpatientswithpreviouspoliomyelitisandthispopulationof589patientsformsaselectedgroupbecauseoftheseverenatureoftheirunderlyingdisabilityandlatefunctionaldeterioration.2Inthepresentseriesoffive 54Chroni,Howard,Panayiotopoulos,SpencerPost-poliosyndromes*post-poliomuscularatrophy*syringomyelia*radiculopathy/myelopathyduetocervicalspondylosispatientswithmultiplesclerosis(twolaboratory-supporteddefiniteandthreeclini-callydefinite3)initialfunctionaldeteriorationwasduetoprogressivelimbweaknessorim-pairedmobilityandinthreecasesthisledtoaninitialdiagnosisofPPMA.Theclinicaldiag-nosisbecameapparentwiththesubsequentdevelopmentofcharacteristicclinicalfeaturesincludingopticnerve,br
ainstem,cerebellumandspinalcordinvolvement.OnepatienthadrelapsingandremittingmultiplesclerosisandtheremainingfoursecondaryprogressiveMS.4InonepatientadiagnosisofclinicallydefiniteMSwasmadeandnofurtherinvestigationswereundertakenbecauseofherseveredisability.Intheremainingfourpatients,oligoclonalbandswerepresentintwoofthethreepatientsinwhomlumbarpuncturewasperformed,evokedpotentialswereabnormalinfourandMRIscanshowedmultipleperiven-tricularwhitematterlesionsinfourpatients.Anumberofneurologicaldisordersmaycauselatedeteriorationinpatientswithpreviouspoliomyelitis(seebox).6'7Otherneurologicaldisordersdescribedinpatientswithpreviouspoliomyelitisincludecerebro-vasculardisease,epilepsy,cerebralandspinaltumours;however,thereisnosuggestionofanincreasedprevalenceofthesedisorders.2Salazar-Grueso8describedneurologicaldis-ordersin6/21patients,includingonepatientwithclinicallydefiniteMS.Thepresenceofuppermotorneuronesignshasalsobeennotedin13/150patientsseeninapost-polioclinic.9Learningpoints/summarybox*latefunctionaldeteriorationmaybeunrelatedtopreviouspoliomyelitis*characteristicclinicalfeaturesmaybemaskedbypre-existingneurologicalsignsTheseincludedcorticobulbarswallowingdifficulties,spasticparaparesis,hyperreflexiaandextensorplantarresponses.Thecauseofthesesignswasattributedtoapolioencephalitisaccompanyingtheoriginalpoliomyelitisoraspartoftheprogressivepost-poliosyndrome.Martynetall'"'arguethatsubclinicalinfectionwithpoliomyelitismightbesevereenoughtodepletemotorneuronesandthatmotorneur-onediseasemightthendevelopasaconse-quenceoffurthermotorneuronelossduetoageingorasecondinsult.However,analysisofmorbidityandmortalityratesformotorneuronediseaseinScotlandshowsnoevidencethatmotorneuronediseaseiscausedbyearlyasymptomaticpolioinfectionorthatpoliovaccinationoffersprotectionagainstthiscondi-tion.2FurthermoreinaliteraturereviewArmonetall3suggestedthepaucityofreportsofclassicalamyotrophiclateralsclerosisdevelopinginsurvivorsofparalyticpolio-myelitismightindicatethatantecedentpoliomyelitishasaprotectiveroleagainstthedevelopmentofamyotrophiclateralsclerosis.TheoccurrenceofMSinthesepatientsemphasisesthatlatefunctionaldeteriorationapparentlymaybeunrelatedtopreviouspoliomyelitis.Furthermorecharacteristicclinicalfeaturesmaybemaskedbytheseverepre-existingneuromuscularandorthopaedicimpairmentleadingtodiagnosticdelayandconfusionwiththeill-definedclinicalsyn-dromeofprogressivepost-poliomuscularatrophy.1DalakasMC,ElderG,HallettM,etal.Along-termfollow-upstudyofpatientswithpost-poliomyelitisneuromuscularsyndromes.NEnglJMed1986;314:959-63.2HowardRS,WilesCM,SpencerGT.Thelatesequelaeofpoliomyelitis.QJMed1988;251:219-32.3McDonaldWI,SilberbergDH.Thediagnosisofmultiplesclerosis.In:WIMcDonald,DASilberberg,eds.Multiplesclerosis.London:Butterworth,1986;pp1-11.4ThompsonAJ,KermodeAG,MacManusDG,etal.Pat-ternsofdiseaseactivityinmultiplesclerosis:clinicalandmagneticresonanceimagingstudy.BMJ1990;300:631-4.5PezeshkpourGH,DalakasMC.Longtermchangesinthespinalcordsofpatientswitholdpoliomyelitis:signsofcontinuousdiseaseactivity.ArchNeurol1988;45:505-8.6FeveA,WallaysC,NicolleMH,GuillardA.Syringomyeliaasalatecomplicationofparalyticpoliomyelitis.Neurology1992;42:1421-2.7CarrollSD.Syringomyeliaasapossiblecomplicationofpoliomyelitis.Neurology1967;17:213-5.8Salazar-GruesoEF,GrimaldiLME,RoosRP,VariakojisR,JubeltB,CashmanNR.Isoelectricfocusingstudiesofserumandcerebrospinalfluidinpatientswithantecedentpoliomyelitis.AnnNeurol1989;26:709-13.9Salazar-GruesoEF,CashmanNR,MaselliR,RoosRP.Uppermotorneurone(UMN)findingsinpatientswithantecedentpoliomyelitis.Neurology1987;37:215.10MartynCN,BarkerDJP,OsmondC.MotoneuronediseaseandpostpoliomyelitisinEnglandandWales.Lancet1988;i:1319-22.11MartynCN.Poliovirusandmotorneuronedisease.JNeurol1990;237:336-8.12SwinglerRJ,FraserH,WarlowCP.MotorneuronediseaseandpolioinScotland.JNeurolNeurosurgPsychiatry1992;55:1116-20.13ArmonC,DaubeJR,WindebankAJ,KurlandLT.Howfrequentlydoesclassicamyotrophiclateralsclerosisdevelopinsurvivorsofpoliomyelitis?Neurology1990;40:172-