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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_186_библиотеки_им_акад_М_И_Перельмана

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knows for certain whythis is.Hypotheses include lowerlevels ofUV radiationexposure or lowerserum levels ofvitamin D amongpopulationsathigherlatitudes. Important:Neitherahistoryoftraumanoranyvaccine(none!)hasbeendefinitivelyassociatedwithanincreasedriskofMS.
DefiningMS
ThereisnoonespecifictestthatestablishesthediagnosisofMS.Classically,thediagnosisofMS requires:
AtleasttwoepisodesofneurologicdysfunctiondisseminatedinspaceandtimewithintheCNS.In otherwords,atleasttwoneurologicdeficitsmustappearovertwodistinctperiodsoftimeandmust belocalizabletotwodifferentanatomicregions(i.e.,‘space’)withintheCNS.
Thisdefinitionstillapplies,buthasbeen,andcontinuestobe,expandedasourunderstandingofthe diseasehasgrownandourimagingtechniqueshaveimproved.Themostrecent2017iterationofthe McDonaldcriteria(thegold-standardcriteriausedforMSdiagnosis)requiresfivethingsforthe diagnosisofMS:
A “typical” clinical syndrome (see page 236 for details; it is important to remember that the McDonaldcriteriaareonlyvalidatedinpatientswhopresentwithsymptomsconsistentwithMS),as opposedtopatientswithnonspecificsymptomssuchasheadacheorfatigue Objectiveclinicalevidenceonneurologicexamination(forexample,Emma’sINO) Dissemination in space (this criterion can be met by either clinical findings OR the presence of lesionsonmagneticresonanceimaging[MRI]) Disseminationintime(thiscriterion,too,canbemetbyeitherclinicalorMRIfindingsor,entirely unrelatedtotimebutanappropriatesurrogateaccordingtothemostrecentcriteria,bythepresence ofcerebrospinalfluid(CSF)-uniqueoligoclonalbandsintheCSF;moreontheselater) Lack of a better explanationfor the patient’s presentation (i.e., the overall clinical picture is not betterexplainedbya different inflammatoryor infectiousetiology).Thisis animportantcaveatin thatwestillneedtoensurethatotheretiologiesarenotmissed.
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AsagittalMRIofthebraininapatientwithMS.Notetheperiventriculardemyelinatingplaquesthatradiate awayfromthelateralventricleatapproximately90-degreeangles.Thesearecolloquiallyreferredtoas DawsonfingersandarecharacteristicofMS.(ReprintedfromLeeE.PediatricRadiology:Practical ImagingEvaluationofInfantsandChildren.WoltersKluwer;2017.)
MScomesintwobasicclinicalphenotypes:
Relapsing remitting MS—85% of patients have this form of MS, which is characterized by intermittentattacksofneurologicdysfunctioninvolvingdifferentsitesintheCNS.Theseflaresare variablyreferredtoasrelapses,attacks,orexacerbations;don’tbeconfused,thesetermsallreferto the same thing. Patients may recover completely from each attack or experience some degree of residualneurologiccompromiseanddisability.
Patientspresentingwith afirstclinicalattackaresaidtohaveclinically isolatedsyndrome (CIS).Althoughthese patientsdo notfulfilltheclassic definitionofMS(remember,you needdisseminationinspace andtime),manydomeetcurrentMScriteria (basedonradiographicorCSFevidence;seetheabovediscussion).Thosewhodonot meetthecriteriaforMSareathighrisk forconversiontoclinicallydefiniteMS. There is also an entity termed radiologically isolated syndrome (RIS) , in which 2 lesions consistent with MS are seen incidentallyonanMRIina patientwithoutanyclinicalsymptomsofMSwhatsoever. Asmany as40%oftheseindividualswill
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experiencetheirfirstclinicalattackwithin5years.
PrimaryprogressiveMS—thistypeofMSislesscommon;itevolvesgradually,withoutdiscrete episodesofacutedysfunctionandrecovery.Somepatientswiththerelapsing,remittingtypeofMS will evolve into this type of clinical picture, and when disability accumulates insidiously, these patientsarethensaidtohavesecondaryprogressiveMS.
Box9.1
ThedistinctionbetweenrelapsingremittingMSandprogressiveMSisanimportantone, sincethetreatmentandprognosisareverydifferent.
ClinicalSignsandSymptoms
Nowthatwe’vedefinedMS,let’slookatitsclinicalmanifestations.Whatarethe“typical”symptoms? Theyaremanyandvaried,asyouwouldexpectfromadiseasethatcancausedamageanywhereinthe CNS,solet’sfocusonthemostcommonones.
OpticNeuritisThistermreferstoinflammationoftheopticnerve.Symptomsincludeunilateralvision lossthattypicallyprogressesoverseveraldaystoweeks.Thelossofvisionmaybetotalorjustsome mildblurring.Colorvisionisoftenlostpreferentiallyoveracuity.Ocularpainiscommonandisoften exacerbatedbyeyemovements.Themostcommonfindingonphysicalexaminationisanafferentpupillary defect,orAPD(seeBox9.2).
Funduscopicexaminationmayrevealpapillitis(aswollenopticnervehead),butinthemajorityof casestheinflammationoftheopticnerveinvolvesonlytheretrobulbar(meaningbehindtheeyeball)part ofthenerveandthereforecannotbevisualized.Aspartofyourbedsideexaminationyouwilllikelybe abletodemonstratedecreasedvisualacuityandcompromisedvisualfields(theclassicfindingisa centralscotoma,ordarkspot,inthecenterofvision).Inanypatientwithopticneuritis,obtainanMRI withgadoliniumoftheorbitsandthebrain,whichmayrevealenhancementandswellingoftheaffected opticnerveaswellasotherlesionsconsistentwithprior,clinicallysilentdemyelinatingattacks. Approximately20%ofpatientswithafirstattackofopticneuritisandanotherwisenormalMRIwillgo ontodevelopMS;if,however,theMRIshowsevidenceofpriordemyelinationconsistentwithMS,that numberjumpsto80%.
Mostpatientswillrecoveradequatevisualfunctionwithinseveralweekstomonthsafteranacute attack.
Box9.2AfferentPupillaryDefect
Oneofthecharacteristicfindingsofopticneuritisisanafferentpupillarydefect(APD). Swingaflashlightbackandforthbetweenthegoodeyeandthebadone.Whenthelight swingsbacktothebadeye,thepupil,whichyouwouldordinarilyexpecttoconstrict,will insteaddilate.Thisoccursbecausethepupillaryreflexisconsensual:inotherwords,lightin oneeyecausesbothpupilstoconstrict.Thus,whenlightilluminatesthegoodeye,botheyes constrictconsensuallyinthenormalway,butwhenthelightilluminatesthebadeye,overall lightperceptioniscompromisedandthepupilsappeartodilate.
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(A)Demonstrationofanafferentpupillarydefect.(B)Anatomyofthepupillaryreflexpathway.(1)The afferentlimbofthereflex:lighthitstheretina,activatingtheipsilateralopticnerve,whichprojectstothe twobilateralEdinger-Westphal(EW)nucleioftheoculomotornerves(thustheconsensualnatureof thereflex).(2)Theefferentlimbofthereflex:parasympatheticfibersrunningwithintheoculomotor nerve(CN3)projectfromtheEWnucleitotheciliaryganglion,wheretheyactivatetheshortciliary nervesthatinnervatethesphincterpupillae,causingbilateralpupillaryconstriction.
Box9.3TheDifferentialDiagnosisofOpticNeuritis
AlthoughitismostcommonlyassociatedwithMS,opticneuritishasmanyotherpossible causes.Ingeneral,bilateralopticneuritisoropticneuritisassociatedwithnewneurologicor systemicsymptomsshouldpromptamorethoroughinvestigationintootheretiologies.Afew importantonestobeawareofinclude:
Neuromyelitisopticaspectrumdisorder(NMOSD)(seepage243) Chronicrelapsinginflammatoryopticneuritis(CRION) Connectivetissuediseases(e.g.,systemiclupuserythematosusandsarcoidosis) Paraneoplasticopticneuropathy(mostoftenassociatedwiththeCRMP5autoantibody) Infectioussyndromes(e.g.,Lymedisease,syphilis,cytomegalovirus)
SpinalCordInvolvement,(i.e.,myelitis)Withanacuteattackaffectingthespinalcord,patientsmay experiencefocalmotororsensorysymptomsbelowtheaffectedspinallevel.Althoughsymptomsare oftennotperfectlysymmetric,bothlegsareusuallyinvolvedtosomeextent.Patientsmayinitiallyfeela tightnessattheaffecteddermatomelevel;thishasbeencalledthe“MShug.”Whenmotorpathwaysare involved,theaffectedmusclesmayinitiallybeweakandflaccid,butovertimespasticityand hyperreflexiawilldevelop.Spinalcordinvolvementcanalsoleadtourinaryandbowelsymptoms.An MRIofthespinetypicallyshows“short-segment”lesions,thatis,lesionsinvolvingfewerthan3 vertebrallevels.Foramoredetailedreviewofthespinalcord,seeChapter10.
Box9.4TheLhermitteSign
TheLhermittesignisacharacteristicfeatureofMSthatisoftenhighlightedinlecturesand
onrounds.Thepatientdescribesanelectricalsensationthatshootsdownthespinewhenthe neckisflexed.AlthoughsuggestiveofMS,theLhermittesignisnotpathognomonicandcan beseeninotherdiseasesthataffectthedorsalcolumnfibersinthecervicalspinalcord.
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TheLhermittesign.
BrainstemandCerebellarSyndromes.Oculomotorabnormalitiesarefarlesscommonthanoptic neuritis.However,doublevisioncanoccur,oftenfrominternuclearophthalmoplegia(INO),asinthecase ofEmma,whichbeganthischapter,orfrompalsyofasinglenerve(usuallythesixthcranialnerve).MS canalsocausetrigeminalneuralgia(seepage108).Cerebellarinvolvementcancausevertigoorataxia.
Cerebral,CognitiveDeficits.Thesedeficitsusuallydevelopwithadvanceddiseaseaffectingmultiple areasinthecerebrum.Short-termmemory,executivefunction,visuospatialfunction,andthespeedat whichonethinksandcommunicatescanbecompromised;thelastiscolloquiallyknownas“MSbrain fog.”
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Overtime,inadditiontocognitivedeficitsandmooddysfunction,patientsmaydevelopdisabling symptomsfromirreversibleaxonalinjury.Amongtheseare:
Neurogenicbladder(incontinence,frequency,urgency) Neurogenicbowel(incontinence,constipation) Sexualdysfunction Neuropathicpain Chronicfatigue Spasticity(increasedmuscletone,oftenwithsuperimposedspasms) Alteredgait
HowtoMaketheDiagnosis
Let’srestatethemajordiagnosticcriterionforMS:evidenceofneurologicdeficitsdisseminatedin spaceandtime.Thefirstattack,aswe’vealreadymentioned,isreferredtoasclinicallyisolated syndrome,althoughevaluationatthattimemayrevealotherlesionsthatqualifyforthefulldiagnosisof
MS.Athoroughhistoryandphysicalexaminationarethereforeessential.
PerformaCompleteHistoryandNeurologicExamination.Inparticular,askaboutthemostcommon manifestationsofMS.Promptthepatienttotrytorecallanyothereventthatmayhavebeenignoredor forgottenbutthatmayhavebeenasentinelMSeventthatcompletelyresolved.Next,carryoutacareful neurologicexamination.Youmayuncoverasubtlefindingthateventhepatientisunawareof(e.g.,eye movementabnormalities,subtlesensorylossorabnormalreflexes).
GetanMRI.Ifyoususpectthediagnosis,getanMRIofthebrain.Contrastisnecessaryifthepatientis presentingwithnew,activesymptoms;otherwise,there’snoneedforgadolinium.VirtuallyallMRI centersuseastandardizedMSprotocol.IfabrainMRIisinconclusive,oriftherearesignsorsymptoms ofspinalcordinvolvement,thenthecordshouldbeimagedaswell.
Box9.5SpinalCordImaging
Keypoint:ifyouareinterestedinthespinalcorditself—which,inthecaseofMSyouare (remember,itisadiseaseof,andonlyof,thecentralnervoussystem)—ordercervicaland thoracicspineMRIs.Thereisnoneedforalumbarscan.Remember:thecorditselfendsat approximatelyL1;thus,alumbarspineMRIdoesnotvisualizethespinalcord,onlythe bundleofspinalnervesandnerverootswerefertoasthecaudaequina.
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Thelumbarspine.NotehowthecordendsatthelevelofL1.
WhitematterlesionsonMRIcanbeseeninmanydiseases,notjustMS.Long-standingcerebrovascular diseaseand,perhapssurprisingly,migraine,arethetwomostcommonpotentialmimics(seepage98,Box
3.3).However,therearespecificMRIcriteriathat,ifmet,makeMSthemostlikelydiagnosis.The classicMSlesionsareovoidasopposedtoroundandtendtooccurinfourspecificlocations:
Periventricular Juxtacortical(andcortical,arecentadditiontothe2017McDonaldcriteria)
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Infratentorial(brainstemandcerebellum) Spinalcord
(A)Juxtacortical(bluearrow)andperiventricular(purplearrow)MSlesions.Themoreconfluentwhite matterdisease(pinkarrows)ischaracteristicoflong-standingmicrovasculardisease(i.e.,dueto uncontrolledhypertension,hyperlipidemia,etc.);theseareasdonotrepresentMSplaques.(B)High cervicalspineMSlesion(redarrows).(A,modifiedinpartfromSanelliPC,SchaeferPW,LoevnerLA.
Neuroimaging:TheEssentials.WoltersKluwer;2016;andB,reprintedfromBarkovichAJ,RaybaudC. PediatricNeuroimaging.6thed.WoltersKluwer;2018.)
AllMSlesions(oldornew)arehyperintenseonT2imaging.Activelesionsaregadoliniumenhancing (andcontinuetoenhanceforapproximately1month);oldlesionsarenotenhancingandcan,overtime, formso-calledblackholesonT1imagingindicativeofaxonalloss.Therefore,ifyouseebothenhancing andnonenhancinglesions,youhaveevidencefordisseminationintimeaswellasspace.TheMRIcan alsoassesstheseverityofthediseaseandtosomedegreehelppredictthepatient’sprognosis.
LookattheCSF.Ifthediagnosisisstilluncertain,CSFanalysisisthenextstep.Whatyouarelookingfor are(1)CSF-specificoligoclonalbands,whicharepresentinthemajorityofpatientswithMS,plus(2) anincreasedIgGsynthesisrate.Theformerreferstobandsonelectrophoresisthatarenotpresentinthe serum.ThelatterreferstotherateatwhichIgGismanufacturedwithintheCSF.Thesefindingsarenot specifictoMS,sotheymustbeassessedwithintheoverallclinicalcontext.However,theabsenceof CSF-specificoligoclonalbandssuggeststhatthepatientmaynothaveMS(insuspectedMS,only2%to 3%ofpatientswillprovetoactuallyhavethediseaseintheabsenceofoligoclonalbands).Thenumber ofwhitebloodcellsintheCSFisusuallynormal(lessthan5)butcanbeelevated,althoughitrarely exceeds50.
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