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Box2.5
Thedominantcerebralhemisphereisdefinedasthehemispherethatcontrolslanguage.In
right-handedpeople,thisisinvariablythelefthemisphere.Inleft-handedpeople,it’sabout
80/20,rightversusleft-dominant.
Aphasiaisanacquireddisorderoflanguagecomprehensionand/orproduction.Don’tgetthisconfused
withdysarthria,whichisamotordeficitcharacterizedbytheimpairedabilitytocontrolthemusclesused
forspeech,resultinginuncleararticulationofspeech(oftendescribedas“slurred”)thatisotherwise
normal.Therearetwomainlanguagecentersinthebrainthatcausetwodistinctlanguagedeficitswhen
damaged:
1. Broca’s are aislocatedintheinferiorfrontalgyrusofthefrontallobeandisresponsibleforlanguageproduction.Brocaaphasia(also
known as expressive, or nonfluent, aphasia) is characterized by halting and effortful speech, often with long pauses and difficulty
naming objects. The abilityto repeat words spoken to themis also lostbutcomprehension remains intact. Damage to the cortex
surroundingBroca’sareaproducesasimilarexpressiveaphasiabutwithpreservedrepetition(calledtrans corticalmotoraphasia).
2. Wernicke’s area is located in the superior temporal gyrus of the temporal lobe and is responsible for language comprehension.
PatientswithWernickeaphasia(alsoknownasreceptive,orfluent,aphasia)demonstratefluentspeechwithintactsyntaxandprosody
butdevoidofcontent ormeaning.ListeningtoapatientwithWernickeaphasiaspeakislikelisteningtosomeonespeakinginaforeign
languagethatyoudon’tunderstand;itcanbeprettybutitismeaningless.Patientsareunabletofollowspokencommandsand,likethose
withBrocaaphasia,losetheabilitytorepeat. DamagetothecortexsurroundingWernicke’sareaproducesasimilarreceptiveaphasia
butwithpreservedrepetition(calledtrans corticalsensoryaphasia).
FewpatientsactuallypresentwithapureBrocaorWernickeaphasia.Mostaphasiasaremixed,
althoughtheytendtofavoroneortheother,meaningtheypresentwitheitherpredominantlyexpressiveor
receptivedeficits.
ThearcuatefasciculusisthebundleofnervesthatconnectsBrocaandWernicke’sareasandis
responsibleforrepetition;thus,damagehereresultsintheinabilitytorepeat,withpreservedfluencyand
comprehension.
Globalaphasiaismostoftenseeninthesettingofbigleft-sidedMCAinfarcts(somewherebetween
70%and95%ofthepopulationisright-handed),whichknockoutcomponentsofthearcuatefasciculus,
Wernicke’sareaandBroca’sarea,resultinginimpairedabilitytorepeat,understandandproduce
language.
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Majordomainsofthebrain,illustratingtheregionsthat,whendamaged,produceBrocaandWernicke
aphasia.
TypeofAphasia LocationoftheLesion Presentation
Brocaaphasia Inferiorfrontalgyrus Expressive(nonfluent)aphasia,withretainedcomprehension
Transcorticalmotoraphasia CortexsurroundingBroca’sarea Asabove,withpreservedrepetition
Wernickeaphasia Superiortemporalgyrus Receptive(fluent)aphasia,withretainedfluency
Transcorticalsensoryaphasia CortexsurroundingWernicke’sarea Asabove,withpreservedrepetition
Conductionaphasia Arcuatefasciculus Isolatedinabilitytorepeat
Globalaphasia Somecomponentofalloftheabove Inabilitytospeakfluently,comprehend,orrepeat
Neglect. Neglect is the second classic sign of a cortical stroke. It usually localizes to the
nondominantparietalcortexbutcanoccurwithdominantaswellasnondominantcortical lesions.
Neglectcanbedramatic,suchaswhenpatientsfailtorecognizetheirownarmoronlyappropriately
dresshalfoftheirbody,or—amuchmoresubtlefindingonexamination—whenpatients(likeLaura,
whomwemetatthebeginningofthischapter)extinguishtodoublesimultaneousstimuli(seepage31
forareviewonneglectandextinction).
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Adrawingofaclockbyapatientwithneglectduetoastrokeinthenondominantparietalcortex.
Visualfieldcuts.Visualfieldcutsinwhichtheentirecontralateralvisualfieldislost(homonymous
hemianopia), or in which the upper or lower quadrant of the contralateral visual field is lost
(homonymousquadrantanopia)areduetolesionsthatinvolvethevisualcortexintheoccipitallobe
orlesionsimpactingtheopticradiations,whicharealsobyandlargecorticalpathways.
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Parisasseenthroughtheeyesofapatientwitharighthomonymoushemianopia.
Gazepreference.Gazepreference(seepage32)ismostoftenduetoinvolvementofthefrontaleye
fields,whichare tractslocatedinthefrontal cortex.Forinstance,when stimulated(asinseizure),
theleftfrontaleyefieldpushestheeyestotheright,andviceversa.Whenknockedout(asinstroke),
theleftfrontaleyefieldlosesitsinfluenceandtherightfrontaleyefield“wins,”pushingtheeyesto
the left. Theresultis that patientswho are seizing“lookawayfromthe lesion,”and patients with
stroke“looktowardthelesion.”
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Thefrontaleyefields.(A)Whentheleftfrontaleyefieldisstimulated,therightsixthnervenucleusand(via
themediallongitudinalfasciculus[MLF];seepage232)theleftthirdnervenucleusarestimulated,
resultinginrightgaze.(B)Whentheleftfrontaleyefieldisknockedout,therightfrontaleyefieldtakes
over,andtheeyesdeviatetotheleft.
Again(becausethisisimportant!):aphasia,neglect,visualfieldcuts,andgazepreferencearethe
fourmajorcorticalsignsyouneedtoknow.Thereareothercorticalsignstobeawareofthatarenot
partoftheofficialNIHstrokescale(NIHSS)usedtoassessstrokeseverity(seethenextpage)butwhich
canbeequallyashelpful.Listedbelow,thesetendtolocalizetotheparietallobe.
Apraxia—the inability to execute a previouslyknownmotor task,not explainedbyother deficits
(suchasweaknessorblindness).Totestforvariousapraxias,youcanaskyourpatientstoshowyou
howtheybrushtheirteeth,combtheirhair,orbuttontheirshirt.
Astereognosis—theinability torecognizeobjects through touch alone. Askyour patientstoclose
theireyesandthenplaceanobject—apennyorapaperclip—intheirhand.Iftheycannotfigureout
whattheobjectis,theyhaveastereognosia.
Agraphesthesia—theinabilitytorecognizewritingontheskin.Askyourpatientstoclosetheireyes,
butthistimedrawaletterornumberonthepalmofthehand.Iftheycannotfigureoutwhatyou’ve
written,theyhaveagraphesthesia.
Anosognosia—aconditioninwhichpatientsdonotrecognize—orhavesignificantlyreducedinsight
into—theirowndeficit.
Box2.6TheNIHSS
TheNIHSSbecamethegold-standardscaleforratingstrokeseverityfollowingthe
publicationoftheNationalInstituteofNeurologicalDisordersandStroke(NINDS)trialin
1995.aItrangesfrom0(nodeficits)to42.Itcanbeusefulinanacutesettingtogetaquick
senseofjusthowbadapatient’ssymptomsarebutshouldnotbesubstitutedforareal
neurologicexamination.Itsignificantlyunderrepresentsbothright-sidedandposterior
circulationsymptoms,and—althoughhighscoresaremeanttoconveymoresevere
symptoms—lowscorescanhidedevastatingdeficits:pureaphasia,forinstance,mayresult
inonlya1,2,or3onthisscale.
1a.LevelofConsciousness 0-Alert
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1b.Whatismonth/age 0-Answersbothcorrectly
1c.Open/closeeyesandhand 0-Performsbothcorrectly
2.Bestgaze 0-Normal
3.Visualfields 0-Novisualloss
4.Facialpalsy 1-Minor
5a.Motor—leftarm 0-Nodrift
5b.Motor—rightarm 0-Nodrift
6a.Motor—leftleg 0-Nodrift
6b.Motor—rightleg 0-Nodrift
7.Limbataxia 0-Absent
8.Sensory 1-Mildtomoderateloss
9.Bestlanguage 0-Noaphasia
10.Dysarthria 0-Normal
11.Extinction/inattention 1-Extinctiontoonemodality
OurpatientLaura’sNIHSSresult.Althoughyoupickeduponsubtleleftupperextremityweaknessonyourexamination,Lauraisableto
holdherleftarmupagainstgravityforafull10secondswithoutanydownwarddrift;thus,shescores0forleftarmweakness.Asisoften
thecase,theNIHSSdoesnotcapturethefullextentofherdeficits.
a
seeNationalInstituteofNeurologicalDisordersandStrokert-PAStrokeStudyGroup.TPAforacuteischemicstroke.NEnglJ
Med.1995;333(24):1581-1587.
ACA,MCA,andPCAStrokes.MCAstrokesarebyfarthemostcommon,butACAandPCAstrokesare
notrare.Thetablebelowisfarfromcomprehensivebutgivesagoodoverviewofthemostimportantand
mostfrequentsignsandsymptomstorecognize.You’llnotethatallofthesesyndromesincludethe
corticalsignswejustdiscussedabove.
Symptom Localization
MCA Contralateralhemiparesis(face/arm>leg) Motorcortex
Contralateralhemisensoryloss(face/arm>leg) Sensorycortex
Contralateralhomonymoushemianopia Opticradiations
Aphasia(dominantMCA) BrocaorWernicke’sareas
Neglect(eitherdominantornondominantMCA) Parietalcortex
ACA Contralateralhemiparesis(leg>face/arm) Motorcortex
Contralateralhemisensoryloss(leg>face/arm) Sensorycortex
Abulia—i.e.,apathy(inabilitytoactwillfully),oftenassociatedwithdecreasedspontaneous
speechandmovement
Uncertain;thoughttoinvolvethe
cingulategyrus
Aphasia(dominantACA) Transcorticalmotorarea
Gaitapraxia—i.e.,difficultyinitiatinggait Frontalcortex
PCA Contralateralhomonymoushemianopia Visualcortex
Contralateralhemisensoryloss Thalamus
Memoryimpairment(dominantorbilateralPCA) Hippocampus
Alexiawithoutagraphia(dominantPCA)—i.e.,theinabilitytoreadwiththeretainedabilityto
write
Temporal/Occipitalcortex
Prosopagnosia(nondominantPCA)—i.e.,theinabilitytorecognizefaces Fusiformgyrus
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(A)Areminderoftheanteriorcerebralartery(ACA),middlecerebralartery(MCA),andposteriorcerebral
artery(PCA)territories.(B)Thehomunculus(i.e.,“smallhuman”)isatopographicrepresentationofthe
corticalmotorareasdedicatedtodifferentpartsofthebody(asimilarversionexistsforthecortical
sensoryareas).Asyoucansee,ACAstrokeswillpredominantlyaffectthelowerextremities,whereas
MCAstrokeswillaffecttheupperextremitiesandface.
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Box2.7AFewStrokeSyndromestoKnow
Gerstmannsyndromeischaracterizedbytheclinicaltetradofleft/rightconfusion,finger
agnosia(theimpairedabilitytodiscriminateamongone’sownfingers),acalculia(theinability
toperformmathematicalcalculations),andagraphia(theinabilitytowrite).Itiscausedby
lesionsinthedominantparietalcortex,typicallyMCA(butoccasionallyPCA)territory.
Balintsyndromeiscausedbylesionsinthebilateralparieto-occipitalcortex,corresponding
totheMCA/PCAborderzone.Itpresentswithoculomotorapraxia(theabsenceof
controlled,purposefuleyemovements,oftencausingsignificanttroublewithreading),optic
ataxia(poorvisual-motorcoordination),andsimultagnosia(theinabilitytoperceivemorethan
oneobjectatatime).
AntonSyndromeisaformofcorticalblindnessassociatedwithanosognosia,inwhichthe
patientisunawarethatheorsheisblind.Patientswillcontinuetoinsist—oftenquite
adamantly,andinthefaceofclearevidencetothecontrary—thattheycansee,andwill
oftenconfabulate(meaningtheywillfabricateimaginaryinformation)whenaskedabout
objectsorimagesplacedinfrontofthem.Thisiscausedbybilateraldamagetotheoccipital
cortex,aresultofbilateralPCAortop-of-the-basilararteryocclusion.
BasilarArteryStroke.Basilararteryocclusionscanbedevastatingduetothalamic,brainstem,and
cerebellarinvolvement.UnlikeACA,MCA,andPCAstrokes,theycanbedifficulttorecognizegiven
theirhighlyvariableandoftenstutteringpresentation.Symptomsrangefromisolatedoculomotorpalsies
tolocked-insyndromeorcoma.Importantly,unlikemostACA,MCAandPCAstrokes,basilarocclusions
canpresentwithadecreasedlevelofconsciousness,aresultofinvolvementofthereticularactivating
system(RAS).
The“topofthebasilar”syndromeisastrokecausedbyaclotlodgedattheverytopofthebasilar
artery,justbeforeitsplitsintothetwoPCAs.Thepontineperforatorsarespared,butbothPCAterritories
areatrisk,resultinginischemiaofthebilateralthalami,midbrain,posteriortemporalandoccipitallobes.
Classicsymptomsincludeadecreasedlevelofconsciousness,verticalgazepalsy,corticalblindnessand
—ifthesuperiorcerebellararteriesareinvolved—vertigo,nausea,vomiting,andataxia.
Locked-insyndromeisacatastrophicconditioncausedbybilateralpontineischemiaduetobasilar
arteryembolismorthrombosis(pontinehemorrhage,usuallyrelatedtohypertension,isanothercause).It
ischaracterizedbyquadriplegiaandtheinabilitytospeakorswallow,butconsciousness,cognitive
functionandverticaleyemovementsarespared.Patientsareeffectively“lockedin”—wideawakebut
onlyabletocommunicatebyblinking.
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Anteroposterior(AP)angiographicviewshowingatopofthebasilarocclusionpre-(A)andpost-(B)
thrombectomy.InfigureA,bloodflowisblockedandyoucan’tseetheposteriorcerebralarteries(PCAs);
butinfigureB,followingthrombectomy,thePCAsfillwithbloodandcannowbeclearlyvisualized.
(ReprintedfromBarkovichAJ,RaybaudC.PediatricNeuroimaging.6thed.WoltersKluwer;2018.)
BrainstemStrokeSyndromes.Thebrainstemstructures—themidbrain,pons,andmedulla—aresmall
butimportant:theycontainthemajorityofthecranialnervenucleiaswellasthesensoryandmotortracts
thatruntoandfromthecortexandthespinalcord.Theresultisthataverysmallbrainstemstrokecan
haveverybigconsequences.Therearedozensofspecificbrainstemstrokesyndromes,butifyoukeepthe
followingprinciplesinmindyouwon’tneedtomemorizemuchatall.
Crossed symptoms, a term referring to ipsilateral cranial nerve deficits (affecting the face) and
contralateralsensorimotor deficits (affectingthebody), are a classic feature ofbrainstem strokes.
Remember,thecranialnerves(CNs)donotdecussate(withtheexceptionofCN4andthebranchof
CN3that innervates the contralateral superior rectus; don’t worry aboutthis!), but the motor and
sensorypathwaysdo(i.e.,thecorticospinaltractsanddorsalcolumnmediallemniscaltractsinthe
medullaandthespinothalamictractsinthecord;seepages18and23).
Themajormotorpathway(thecorticospinaltract)runsmediallyinthebrainstem.
Thepain/temperaturepathway(thespinothalamictract)runslaterallyinthebrainstem,oftenside-bysidewiththesympathetictract.
Cranialnerves3through12exitfromthebrainstem(CN3and4exitfromthemidbrain;5,6,7,and8
exitfromthepons;and9,10,11,and12exitfromthemedulla;seethepicturebelow).Thus,among
other symptoms,midbrainstrokesoften presentwith CN3and4 involvement,pontinestrokeswith
somecombinationofCN5,6,7,and8involvement,andmedullarystrokeswithCN9,10,11,and12
involvement.Keepinmindthatthetrigeminalnerve(CN5)nucleusisthelargestofthecranialnerve
nucleiandactuallyextends from the midbrainthrough the ponsandmedulla into the highcervical
spinalcord.Ipsilateralfacialnumbnessisthereforenotaparticularlyhelpfullocalizer,asitcanbe
seeninstrokesaffectinganyoftheabovementionedstructures.
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