landau kleffner syndrome (lks)
TRANSCRIPT
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Landau Kleffner Syndrome (LKS)
Information for families
Great Ormond Street Hospital for Children NHS Trust
Page
Medical overview 1
Whathappenstothechild? 2
Whydoesithappen? 4
Howdoesithappen? 4
How is it diagnosed? 6
Whattestsmaybedone? 7
What treatment is available? 9
Medicaltreatment 9
Surgicaltreatment 12
Theclinicalcarepathway 14
The Effects of LKS
and Therapeutic Strategies 16
Languageand
communicationskills 16
Languagetherapy
andeducationalsetting 17
Speech&languagetherapy18
Visualcuesandalternative
communication 19
Auditorytraining 21
Socialinteractionand
communication 22
Othercognitiveabilities 23
Non-verbalskills 23
Memoryandattention 24
Behaviour 25
Attentiondeficits,
hyperactivity&aggression 26
Sleepdisorders 27
Otherbehaviours 30
Motordifficulties 30
Page
General support principles 31
School 33
Educationalchallenges 33
Statementof
EducationalNeeds 34
Placement 35
Usefulteachingapproaches36
Specificpatterns
ofimpairment 37
Keyelementsfora
successfulplacement 38
Prognosis (What does
the future hold?) 40
Family adjustment
and support 42
Research 43
Useful contacts 44
Further reading 48
Commonly encountered
medical concepts 49
Childdevelopment 49
Catch-up 50
Epilepsy 50
Seizures 51
Todd’sparesis 51
Convulsivestatusepilepticus51
Non-convulsivestatus 51
Epilepsywithelectricalstatus
epilepticusduringsleep
(ESES) 52
Contents
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Page
Medical overview 1
Whathappenstothechild? 2
Whydoesithappen? 4
Howdoesithappen? 4
How is it diagnosed? 6
Whattestsmaybedone? 7
What treatment is available? 9
Medicaltreatment 9
Surgicaltreatment 12
Theclinicalcarepathway 14
The Effects of LKS
and Therapeutic Strategies 16
Languageand
communicationskills 16
Languagetherapy
andeducationalsetting 17
Speech&languagetherapy18
Visualcuesandalternative
communication 19
Auditorytraining 21
Socialinteractionand
communication 22
Othercognitiveabilities 23
Non-verbalskills 23
Memoryandattention 24
Behaviour 25
Attentiondeficits,
hyperactivity&aggression 26
Sleepdisorders 27
Otherbehaviours 30
Motordifficulties 30
Page
General support principles 31
School 33
Educationalchallenges 33
Statementof
EducationalNeeds 34
Placement 35
Usefulteachingapproaches36
Specificpatterns
ofimpairment 37
Keyelementsfora
successfulplacement 38
Prognosis (What does
the future hold?) 40
Family adjustment
and support 42
Research 43
Useful contacts 44
Further reading 48
Commonly encountered
medical concepts 49
Childdevelopment 49
Catch-up 50
Epilepsy 50
Seizures 51
Todd’sparesis 51
Convulsivestatusepilepticus51
Non-convulsivestatus 51
Epilepsywithelectricalstatus
epilepticusduringsleep
(ESES) 52
Landau Kleffner Syndrome (LKS)
LandauKleffnerSyndrome(LKS)isa
rareformofepilepsythatonlyaffects
children,andcausesthemtolosetheir
understandingoflanguage.Themain
epilepticactivityhappensduringsleep
andisusuallynotobvioustoothers.It
canbeseenonbrainwaverecordings
(EEG,electroencephalography.).There
may,however,alsobevisibleseizures
atnightand/orduringtheday.LKS
mayalsobereferredtobyavarietyof
relatedtermsthatdescribeitseffects
(seepage49).
Astheconditionisnotwellknownand
hascomplexeffectsonlanguageand
oftenalsoonbehaviour,itcantake
sometimebeforethewholepicture
isrecognisedbothbyparentsand
professionalsandsoitcantakesome
timebeforeLKSisdiagnosed.
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What happens to the child?
Inmostcases,thechildhasnormal
earlydevelopment,including
normaldevelopmentofspeechand
language.Onsetofthediseaseis
usuallybetweenthreeandnine
yearsandthechildexperiences
deteriorationinspeechand
languageability(seepage16).
Thislossmaybeabruptorgradual
overaperiodofweeksormonths
andisofteninitiallymistaken
fordeafness.Manychildren
compensatenaturallyfortheloss
oflanguagebyusingvisualcues
andgesture,andmayinitiallyhide
theextentoftheirdifficulty.The
deteriorationinskillsisoftencalled
aregression,asthechildappears
tohavereturnedtoanearlier
stageintheirdevelopment.
Thereareoftenassociated
behaviouralchanges(seepage25)
includingover-activity,reduced
concentrationspan,irritability,
tantrumsanddifficultieswith
socialinteraction(seepage17).
Thechildmayalsohaveproblems
withfinemotorco-ordinationand
movement(forexample,dribbling,
messyeating,lossofspeechclarity,
clumsinessandtremor).These
difficultiesarethoughttobea
directresultofthediseaseprocess,
andnotsimplyanemotional
reactionbythechildtotheirlossoflanguage.
Mostofthechildrenhaveclinically
obviousseizures,andtheseoften
startbeforetheinitialregression.
Thecourseoftheillnessisvery
variable.Itisn’tusuallylife
threatening,butcangreatlyaffect
achild’sfunctioning.Somechildren
mayrecoverspontaneously,while
othersmayrecoverwiththeuse
ofanti-epilepsydrugs(AEDs)
includingcorticosteroids,oreven
brainsurgery.Recoverymaybe
completebutmoreoften,children
havesomedegreeofpersisting
difficultieswithlanguage,
behaviourorcognitiveskills.The
activephaseofthediseaseoften
lastssomeyearsuntiladolescence.
Duringtheactivephasetheremay
berepeatedepisodesofregression
andrecovery,andachild’s
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understandingandperformance
maybehighlyvariableeven
withinthesameday.Thevariation
canberelatedtothedoseof
corticosteroidsandattemptsto
weanthem.Thereistheimpression
thatformanychildren,thefirst
regressionisthemostsevere,
howeveritisn’tunusualfor
childrentorecovertheirskills,only
tolosethemagaininafurther
regression.
More information regarding
treatment is given on pages 9 to
13 and prognosis (or outcome) is
discussed further on page 40.
SomechildrenhavesimilarEEG
abnormalitiesasinLKS,butlose
skillsinallareas(includinggeneral
intelligence),notspecificallyin
language.Thisbroadgroupis
usuallyreferredtoasElectrical
StatusEpilepticusduringsleep
(ESES)orcontinuousspike-and-
wavedischargesinsleep(CSWS).
LKS(inwhichlanguageismainly
affected)iseffectivelyaspecific
typeofESES.
Werecogniseatleasttwovariants
ofLKS:
• thosewhohadamilddegreeof
early(developmental)language
delaybutwhoshowedtypical
LKSregressionlater
• thosewithanabnormalityon
scanbutotherwiseatypical
history.
ThediagnosisofLKSdoesnot
includechildrenundertheageof
twoyearswhoregressaspartofan
autisticspectrumdisorder,evenif
theyhaveseizuresordischargeson
anEEG.Thisisbecauseexperience
hasshownthatthesechildrenfit
bestwithintheautisticspectrumof
disorders,anddonotconformto
thepatternofdisorderseeninLKS.
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Why does it happen?
Verylittleisknownaboutthe
causesofLKS.Theconditionis
twiceascommoninboysandonly
veryoccasionallyrunsinfamilies.It
maybethatthereisagenetically
determinedvulnerability,which
becomesapparentinresponse
toanenvironmentaltrigger,for
example,infection,butthereisas
yetnoscientificevidenceforthis.
How does it happen?
AllchildrenwithLKScanbeshown
tohaveseizureactivityduringthe
activephase,thatusuallyaffects
bothsidesofthebrain(although
onesidemayseemmoreaffected),
andisoftenconcentratedin
areasknowntobeimportant
forlanguage(centro-temporal
region).Someofthisactivityresults
inactualseizuresbutmuchofit
doesnot,thatis,itis‘sub-clinical’.
EEGrecordingsshowthatthere
isaparticularlyhighrateofsub-
clinicalepileptiformactivityin
sleep,whichoftenamountsto
nearlycontinuousspike-and-wave
(CSWS)discharges(ElectricalStatus
EpilepticusduringSleeporESES)
duringtheactivephaseofthe
disease.
Itisthoughtthatregressionand
impairmentsarerelatedtothese
epileptiformdischargesduring
sleep,andthattheseelectrical
seizures‘short-circuit’thenormal
wiringsocertainfunctionsofthe
brainareprevented.Thisseizure
activity,whichisoften-widespread,
preventsthechildfromusing
hisorherbrainnormallysothey
regressinabilities.Initially,the
brainisnot‘damaged’inthe
conventionalsense,butrather
caughtupinan‘electricalstorm’
thatblockscertainbrainfunctions
(especiallylanguage,attention,
socialfunctioning).Stopping
seizureactivitymayrestorethesefunctions.
LKSmainlyaffectsachild’s
languageabilities,andthisis
probablyrelatedtothecommon
locationofrecordeddischarges
overthekeylanguageareas
(centro-temporalregion).Itwas
initiallythoughttobespecificto
language,butcertainlycurrent
experienceisthatotherhigher
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functionsarealsocommonly
affected,includingattention,social
interaction,behaviourandmotor
control.Non-verbalcognitive
skillsareusuallyrelativelyspared,
althoughnotalways,anditisnot
unusualtohavespecificormore
generallearningdifficulties.
Unlikephysicalinjurywherebrain
‘plasticity’allowsotherareasof
thebraintotakeupimportant
functions,inLKS,thebrain’s
capacityandreservesappeartobe
limitedbytheelectricalactivity.
Consequently,relocationofskills
(suchaslanguage)tootherbrain
areasisnotgenerallypossible.
Theactivephaseofthedisease
relatestotheperiodofsub-clinical
seizureactivityandappearstobe
time-limited,startingaftertheage
ofthree,and‘burningout’byearly
adolescence.Thevisible,clinical
seizuresaregenerallyshortanddo
notshowacloserelationshipwith
theeffectsonlanguageandother
areasofdevelopment.
This‘seizuremechanism’that
producesthedeficits,makes
LKS(andrelatedepilepsies)
quiteunlikemorecommon
developmentaldisabilities,which
areusuallypresentfrombirth
withstaticdeficitsaffectingall
aspectsoflearningevenly.LKSis
alsoquitedifferentfromtraumatic
braininjurywherethereisactual
damagetobrainsubstance,usually
visibleonabrainscan,with
predictablelossofabilitiesrelated
tothedamagedareas.Thebrain-
injuredchildusuallymakessteady
progressoncetheyhaverecovered
fromtheimmediateinjury,andin
somecases,theuninjuredbrain
areasmaytakeoverthelostskills.
LKSandrelatedsevereseizure
disordersareuniqueincausing
extremefluctuationbecauseofthe
variablenatureoftheelectrical
activity.Achild’sunderstanding
andabilitiesmaychange
dramatically(forbetterorworse)
overshortperiodsoftime,and
forsomechildren,theremaybe
obviousvariationevenwithina
day.Thisposesamajorchallenge
forthosesupportingthechild
particularlyintheclassroom(see
page33).
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How is it diagnosed?
LKSisaclinicaldiagnosis,which
meansitismadeonthebasisof
thechild’shistoryandassessment.
Thecorefeaturesareahistory
ofnormalearlydevelopment
followedbylossoflanguageskills,
ofteninassociationwithmild
observedseizuresandbehavioural
changes.Thereisnospecifictest,
althoughEEGrecordingscanbe
veryhelpful,especiallyintheactive
phaseofthedisease.MRIscansare
usuallynormal.
Theconditionisrareandmay
notbethoughtofinitially.It
iscommonforchildrentobe
investigatedfordeafness,autism,
selectivemutism,verbaldyspraxia
orbehaviouralproblemsbefore
thediagnosisismade.
Yourchildwillhaveaninitial
medicalassessment,including
examination.Thephysical
examinationisusuallynormalapart
fromoccasionalmildco-ordination
orothermovementproblems.The
doctormayrequestteststocheck
forvariousalternativediagnoses.
Thetestsaretypicallynormal,
apartfromtheEEG.
Therewillalsobeassessments
ofyourchild’sdevelopment
acrossdifferentareasoflearning,
particularlylanguage.Itisimportant
torecordyourchild’scurrentskills
asabaseline,whichcanbeusedto
gaugetheeffectofthediseaseand
anymedicaltreatmentortherapy,
inthefuture.Thisassessmentwill
alsoallowthetherapisttoidentify
appropriateintervention(s)for
yourchild(forexample,speechand
languagetherapy).Yourchildshould
thenhaveregularassessmentsto
monitorchangesinskillprofile.This
informationwillbeimportantfor
makingdecisionsaboutmedical,
educational,behaviouraland
therapeuticmanagement.
Itisimportantthatyourchildis
assessedatanearlystagebya
multidisciplinaryteamincluding
medical,speechandlanguageand
clinicalpsychologyservices.This
enablesyourchild’sfullprofile
tobeassessedandconsideredin
themanagementprogramme,
andaco-ordinatedapproachto
beadoptedbyallpeopleworking
withyouandyourchild.
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What tests may be done?
MRI (Magnetic Resonance Imaging)
brain scan
Thisproducesaverydetailed
imageofthebrain.Yourchildhas
tolieinsideamachine,whichis
likeasmalltunnel,andcanbe
noisy.Themachineusesabig
magnetandradiowavestotake
apictureofthebrain,abitata
time.Thenacomputercreatesthe
picture.ItdoesnotinvolveX-rays.
Thescantakesquitealongtime
(upto40minutes)andsomany
childrenwillneedeithersedation
orageneralanaesthetictohelp
themtoliestill.Formostchildren
withLKS,thescanappearsnormal.
CT (Computer Tomography)
brain scan
Thisalsoproducesapictureofthe
brain,butitislessdetailedthan
MRI.ItusesX-rays,andismuch
quickertoperformbutisnot
thepreferredimagingmodefor
epilepsy.
EEG (Electroencephalogram)
brain wave record
TheEEGisaspecialtestthat
recordstheelectricalactivityfrom
thebrain.Itisusedparticularly
tolookforcluesaboutfits.Your
childhaswiresstuckontohis/her
headwithspecialglue,which
recordelectricitycomingfromthe
brain(itissimplyrecordingthe
brain’snormalactivity).Duringthe
recording,yourchildwillbeasked
toopenandshuthisorhereyes,
andatonepointtobreathdeeply
(orblowawindmill).Heorshewill
alsobeaskedtolookataflashing
light.Ifpossibletherecordingwill
includeaperiodofsleep,whichis
particularlyimportanttomonitor
withLKS.Insomechildrenthese
activitiesmayincreaseorreveal
abnormalities,whichcanthenhelp
toguidethemedicaltreatment.
DuringtheactivephaseofLKS,
EEGrecordingswillusually
showabnormaldischargeson
bothsidesofthebrainoverthe
centro-temporalregions,and
thesedischargesoftenbecome
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continuousinsleep.Thereforea
sleeprecordisusuallyrequired
whenassessingachildwithLKS,
andoftenthiswillbeachieved
byaperiodofvideo-telemetry
(typicallyovernight).
Video-telemetrymeansusinga
closedcircuitvideocamera,which
islinkedtoanEEGmachine.The
camerarecordswhatishappening
tothepatientatthesametimeas
theEEGrecordsthebrainwaves,
andthescreendisplaysthepatient
andtheEEGtracesimultaneously.
Ifsurgeryisbeingconsidered,the
followingspecialisedtestsmaybe
used:
Methohexital suppression test
Inthistest,yourchildismonitored
usingEEG.Alightanaesthetic
isgivenandashort-acting
barbituratedrug(methohexitol)
isgiventoputyourchilddeeply
asleeptothepointwheretheEEG
recordingofbrainactivitybecomes
aflatline.Thedrugisthenallowed
towearoff,andtheEEGbeginsto
showelectricaldischargesagain.
Thefirstplacewherethisactivity
returnsisthoughttoberelatedto
thesourceoftheseizureactivity.
Thisinformationishelpfulin
planningsurgery.
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Magnetoencephalography (MEG)
Thisdetectstinymagneticfields
thatarepartofseizureactivity,
andisthoughttolocalisethe
seizuresourceveryaccurately.Itis
particularlyhelpfuliftheseizure
sourceappearstobelocatedinone
ofthefoldsofthebrain’ssurface
(commonlythesylvianfissurein
LKS)asitgivesathreedimensional
localisationwhichissuperiorto
EEGinformation.However,the
equipmentisexpensive,bulkyand
notcurrentlyavailableforchildren
intheUKalthoughthereareplans
toaddressthis.Ifthistestwere
needed,yourchildwouldcurrently
needtotraveltoHelsinki.
Single Photon Emission Computed
Tomography (SPECT):
Aradio-labelledtracerisinjected
intoavein(oftenusinga‘plastic
drip’thathasbeeninsertedinto
thebackofthehand),andthe
brain’suptakeofthetraceris
measured,withtheseizuresource
showingreduceduptaketotherest
ofthebrain.
What treatment is available?
Managementcanbedividedinto
twocategories:
• treatingtheseizuresandseizure
activity,therebytryingto
changethediseaseprocessand
reduceitseffectonyourchild
• providingfunctionalsupportto
optimiserecovery.
Thefirstcategoryisdescribed
below.Strategiesfromthesecond
categoryaredescribedwithin
therelevantsectionsonpages
17to39.
Medical treatment
Asdescribedearlier,therearetwo
aspectstotheseizuresinLKS
• theobservable‘clinical’seizures
whichdoNOTappearto
correlatewithseverityofthe
developmentalimpairment
• theelectricalseizureactivity
thatoccursinsleepandis
thoughttocausetheregression
Antiepilepticdrugs(AEDs)or
anticonvulsantsaredrugsthat
areusedtostopseizures.They
areusuallyveryeffectiveforthe
�0
visibleseizuresbuttheireffecton
thesub-clinicalseizureactivity,
whichischaracteristicofLKSand
typicallyoccursinsleep,isoften
disappointing.Somechildrenmay
respondtoconventionalAEDs,
anditiswellrecognisedthat
highdosebenzodiazepines(for
example,clobazamtakenusuallyat
night)canbeparticularlyeffective.
Sodiumvalproateisalsocommonly
usedandoccasionallyotherAEDs
appeartobeeffective.
Corticosteroiddrugscanbe
dramaticallyeffectiveinstopping
seizuresandreversingachild’s
losses.Theyareeitherusedin
shorthigh-dosecoursesorin
prolongedweekly(pulsed)courses
withcarefulmonitoringofside
effects.Somechildrenrecover
wellwithasingleshortcourse
(steroid-responsive),othersmake
goodrecoverybutloseskillswhen
steroidsarestopped(thesechildren
aresteroid-dependentandmay
respondtolonger-termweekly
steroids).Othershaveonlypartial
ornoresponse.Themostcomplete
steroidresponsesappeartobe
seeninchildrenwhoseregression
islargelylimitedtoinabilityto
understandspeech(thatis,pure
auditoryagnosia)andwhodonot
haveadditionalimpairmentsin
behaviour,socialcommunication,
cognitionetc.
Aswithalltreatments,itis
importanttoconsiderthebenefits
andrisksinvolved,andtobeclear
abouttheaims.LKSisnotoriously
difficulttotreat,soitisimportant
tohaveevidenceofatreatment’s
effectiveness,beforesubjecting
achildtoprolongedmedication.
Specialistassessmentbyaspeech
andlanguagetherapist,before
andafterstartingtreatment
isveryhelpfulindocumenting
changesinskillsandjudging
effectiveness.Baselineassessment
ofcognitiveskillsisalsoveryuseful
indeterminingtheoveralllearning
profileandidentifyingstrengths
andweaknesses.
Alldrugshavesideeffectsandit
isnecessarytomonitorforthese
(forexample,steroids–sugarin
urine,bloodpressure).Steroids,in
particular,arepowerfuldrugsthat
whengiveninhighdoseonadaily
basiscanaffectachild’sgrowth,
bonestrength,abilitytofight
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infectionandleadtodiabetes,
highbloodpressureandeven
stomachulcers.Thisiswhydaily
steroidsareusuallyrestrictedtoa
shortperiod(suchassixtotwelve
weeks).Weeklypulsedsteroids
appeartoallowthemedical
benefit,withoutthesameside
effects.However,chickenpoxisa
seriousillnessifachildcatchesit
whilstonanyformofsteroids.Itis
importanttodiscussthiswithyour
doctorifyourchildhasnothad
thisinfectionandwerecommend
immunisationbeforetreatment.
Manyparentsworryaboutthe
effectsthatdrugshaveonlearning
butthisisrarelyaproblem.Itis
thesubclinicalseizureactivitythat
hasthemajorimpactonlearning,
andgenerally,drugsthatcontrol
thisactivityenablelearningtotake
placewithoutongoinginterference
fromseizureactivity.
Someparentsalsoexpressconcerns
aboutthepossiblebehavioural
effectsofdrugs(forexample,
drowsiness,overactivity,changed
appetite,insomnia,bedwetting).
Thiscanbeaproblem,and
childrenwithLKSappear
particularlyvulnerabletosome
sideeffectssuchasirritabilitywith
sodiumvalproate,orsleeping
problemswithlamotrigine.Itis
oftenhardtodisentanglethese
fromthebehaviouraldifficulties
commonlyseeninchildrenwith
LKS.Forexample,itisnotunusual
forparentstodescribeincreased
aggressionandhyperactivity
associatedwiththeearlyphase
ofsteroids–althoughequally,
manyparentsreportdramatic
improvementintheirchild’s
behaviouronsteroidsasthe
diseasecomesundercontrol.
Specificconcernsshouldbe
discussedwiththelocalteam
managingthechild.
Itisimportanttorealisethatall
drugshavetwonames,asthiscan
beconfusing.Thereisthegeneric
name(mainchemicalthemedicine
ismadefrom)andthetradename
(usedbythecompanywhich
producesthedrug).Forexample,
sodiumvalproateisthegeneric
nameandEpilim®isthetrade
name.Itcanbedifficulttopersuade
childrentotakemedicationand
differentformulationssuchassyrup
orsprinklesmaybehelpful.
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Therearealsooccasionalanecdotal
reportsofbenefitsfromother
treatmentssuchasimmunoglobulins
oraketogenicdiet.
Surgical treatment
Brainsurgeryisoccasionallyused
inLKStolimittheeffectofthe
seizures.
Thesurfaceofthebrain(cortex)is
organisedintospecificareasthat
dealwithspecialfunctionssuch
asmovementorlanguage.Brain
cells(neurones)inthissurface
layer,haveimportantfibresthat
passthroughthebrainsubstance
tocarrymessagestocontrolthe
restofthebody.Thesebraincells
alsohavesmallfibresthatbranch
outandconnectthemtothe
otherbraincellsinthesurface
layer.InLKS,oneareaofthe
brain’ssurfacedevelopselectrical
dischargesorseizures.Thisarea
thenspreadstheseizurestoother
areasofthesurface,through
itsnetworkofsmallfibres,and
therebybecomes‘dominant’and
‘drives’therestofthesurfaceor
cortexintodischargesthat‘tie-up’
thebraincellsandpreventsthem
fromcarryingouttheirspecialised
function,suchaslanguage.
BrainsurgeryforLKSaimsto
preventspreadofseizuresthrough
thissurfacenetworkbymaking
tinycutsoverthesurfacewhere
theseizuresoriginate,preventing
thedischargestravellingsideways
toothersurfaceareas,whilst
preservingthelongfibresthat
carrythespecialistmessagestothe
restofthebody.Thissurgeryis
called‘multiplesubpialtransection’
andrequiresspecialisedassessment
toidentifythe‘dominantdriving’
areaofthebrainsurfacetobetargeted.
Brainsurgerymaybeusedfor
childrenwhohaveactivedisease
withpoorrecoveryofskillsand
EEGevidenceofcontinuous
seizuresinsleep,orforthosewho
requireunacceptablyhighdosesof
steroidstomaintaintheirrecovery.
Itdoesnotaimtocurethechild,
buttolimitanyfurtherlossofskills
andallowsomerecovery.
Lessthanhalfofthechildrenwho
areassessedforsurgery,arefound
tobesuitableoninvestigation.
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Brainsurgeryinevitablyhassome
risks.However,theexperiencein
reputablecentresisthatmorethan
halfofthechildrenexperience
significantimprovement,not
simplyinlanguage,butoftenmost
markedlyinbehaviour,particularly
autisticfeatures.Brainsurgery
however,isnotcurativeandthe
childrenwillhavesomeremaining
impairments,althoughexperience
todateisthatnochildren
havebeenmadeworsebythe
procedure.
Surgery(MST)aimstocutthesurfacefibres(solidarrows)andhenceprevent
thespreadofseizurestoothersurfaceareas,whilststillpreservingthelong
fibres(dashedarrows)whichtakemessagestotherestofthebody.
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The Clinical Care Pathway
TheNICE(NationalInstitute
forClinicalExcellence)Epilepsy
guidelines(Oct2004)recommend:
• earlyreferraltoapaediatrician
withspecialresponsibilityfor
epilepsy(within2weeksoffirst
seizure)
• developmentofacomprehensive
careplan
• regularreview
• referraltotertiaryservicesif
thereisdiagnosticuncertaintyor
treatmentfailure
Theservicesshouldbechild-
centred,andthereviewshould
provideaccesstowrittenandvisual
informationabouttheircondition,
counsellingservices,voluntary
organisations,epilepsynurse
specialistsandintegrationwith
othercommunityandmulti-agency
servicesinvolvedinchildren’s
education,welfareandwellbeing.
Thisintegrationmaycommonly
bemediatedbytheepilepsynurse
specialists.
Manyoftheserecommendations
areveryappropriateinLKS,once
thediagnosishasbeenmade.
However,therearedifficultiesas
theseguidelinesrefertothecase
ofclinicallyapparentseizures,
whichdonotalwaysoccurin
LKS(andinanycase,arenotthe
mainproblem).Furthermore,
thereisoftenasignificanttime
delaybeforethediagnosisof
LKSismade,socarepathway
recommendationsmustbeslightly
modified,asbelow.
�. Early referral to a paediatrician
shouldbetriggeredeitherby
aseizureORlossoflanguage
abilitieswithoutovertseizures.
InLKS,childrendemonstrate
lossofpreviouslyacquired
languageabilitiesinassociation
withsubclinicalseizureactivity,
althoughthismayinitiallybe
mistakenforotherconditions
(e.g.mutism,deafness,
behaviouralproblems).
2. Apaediatricianshouldconduct
aninitialassessmentand
investigation.Ideally,this
shouldbeamultidisciplinary
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assessment bythelocal
team,includingspeechand
languageassessment,and
assessmentofcognitiveabilities
/developmentallevel.A
paediatricneurologistwould
usuallybeinvolvedinfurther
assessmentofsucharegression,
andwouldarrangespecialist
investigations(e.g.sleepEEG
orvideotelemetry)anda
multidisciplinaryassessmentas
necessary.
3. AfterdiagnosisofLKS,a
paediatric neurologist would
generally oversee the child’s
medical managementandliaise
withthelocalpaediatrician,
whowouldberesponsible
forcoordinatingtherapyand
supportforthechildandtheir
family.
4. Regular reviewduringthe
activephaseofthedisease
wouldinvolvecloseliaison
betweenpaediatricneurologist
andpaediatrician,and
thefacilityforlanguage
andcognitiveassessments
(particularlytomonitor
responsetochangesin
medication).Thereshouldbe
accesstoadviceonappropriate
educationalplacement,and
behaviourmanagementif
necessary(childpsychiatry/
psychology).
5. The child may be referred
on to a specialist paediatric
epilepsy centre(suchasthe
DevelopmentalEpilepsyClinic
atGOSH)ifthereis:
a. poorresponsetotreatment
b. furtherlossofskillsor
‘plateauing’indevelopment
c. complexorseverebehaviour
problems
d. thepossibilityofepilepsy
surgery
��
Language and communication skills
LKSencompassesabroadspectrum
ofchildrenwithvaryingdegrees
oflanguagedifficulties.During
thecourseofthedisorder,itisnot
uncommonforlanguageskillsto
fluctuateespeciallywhentheEEG
abnormalityisnotcontrolled(see
page4).
Languageproblemsareusually
firstcharacterisedbydifficultiesin
understandingspokenlanguage.
Asmentionedearlier,hearing
lossmayinitiallybesuspected
butformaltesting(puretone
audiometry)invariablyconfirms
anormalhearingsystem.The
difficultyliesintheinterpretation
ofthesounds.Thedifficultieswith
comprehensionvaryfromproblems
understandingcomplexandlonger
instructionstocompleteinability
tounderstandspokenlanguage,
includinglossofunderstanding
ofpreviouslyknown,simple
vocabulary.Insomechildren,the
problemsmaybecomesosevere
thatevenenvironmentalsounds
(suchasadogbarking,atelephone
ringing,trafficnoise)losemeaning
forthechild.
Difficultieswithspokenor
expressivelanguagetypically
followandshowthemselvesin
manydifferentways.Forthose
whoarestillabletospeak,
sentencesmaybesimplifiedand
reducedinlength.Somechildren
experienceproblemsretrieving
knownwordsfromtheirmemory
(a“tipofthetongue”experience).
Theirspokenlanguagemay
consequentlycontainmanypauses
astheytrytofindthewordor
theymaysubstitutealternative
words(forexample,writingstick
forpencil).Somechildrenslotthe
incorrectsoundsintowordssothat
thewordproducedresemblesthe
targetbutisnotarealword(for
example,gilatforgiraffe).Speech
mayalsobeaffectedwithchanges
tointonationorvoicequality.Some
childrensoundslurredorspeakina
jerky,hesitantmanner.Thespoken
The Effects of LKS and Therapeutic Stratgies
��
languagedifficultycanbecomeso
severethatthechilddoesnothave
anyspeechatall.Insuchcases,the
childmayresorttousinggesture
ormimetocommunicate.Reading
mayormaynotbepreserved
inchildrenwhohadpreviously
acquiredthisskill.Morethanhalf
ofchildrenwithLKSalsohave
difficultyusinggesture.
Insomechildren,socialfunctioning
mayalsobeaffected,with
problemsresemblingthoseof
childrenwithautisticspectrum
disorders(ASD).Thismayormay
notamounttoanadditional
diagnosisofautismorASD,which
ismadeonthebasisofapattern
ofdifficultiesobservedinthe
areasofsocialinteraction,social
communicationandimagination.
Individualsvaryinhowitaffects
thembutfeaturescaninclude
lossofdesiretointeract,self-
directednessandproblemswith
eyecontactandfacialexpression.
Inaddition,childrenmayhave
difficultyusingnaturalgestures
orsignstocommunicateorthey
mayusetheircommunicationskills
onlywhenhighlymotivatedtoget
something(thatis,needsdriven,
suchaswantingadrinkwhen
theyarethirsty)ratherthanjust
forsocialreasons(forexample,
todrawattentiontoanobject
ofinterestorsharepleasure).At
themilderendofthespectrum,
problemsmaybenotedwith
conversationalskillsandmore
subtleaspectsofinteraction
(forexample,understanding
andproducingsophisticated
facialexpressionssuchasguilt,
embarrassment).Thisgroupof
symptomsisconsideredinmore
detailonpages22to23.
Language therapy and educational setting
Sincelanguageforthepurposes
oflearningandcommunicating
iscloselyrelatedtocontext,the
environmentisacriticalfactor
inthesuccessfulmanagementof
languageproblemsassociatedwith
LKS.Someexpertshavepointed
outthatcreatingasituation
wherethechildfeelsateaseisa
keyaspecttotherehabilitation
programmeasitsetsthescene
forenthusiasticlearning.Others
��
haveadvisedthatlanguage
therapyshouldbeintegrated
intoclassroommanagement.
Languagetherapyandeducational
managementshouldtherefore
alwaysbeconsideredinrelationto
theother.
Educationalplacementswillbe
coveredinmoredetailinthe
‘school’section(pages33–39).
Speech and language therapy
Speechandlanguagetherapyisan
importantpartofthemanagement
ofchildrenwithLKS.Itshould
bedeliveredaspartofaglobal
approach,whichalsoincludes
medicalintervention,educational
management,behaviour
management(ifneeded)and
pastoralcare.Knowledgeofthe
child’sgeneralcognitiveskillsis
essentialtoensureanappropriately
tailoredprogramme,whichgives
considerationtoallofthechild’s
abilities.Itisalsoimportantfor
thespeechandlanguagetherapist
toworkinconjunctionwiththe
medicalteamandtobeawareof
changesinmedicaltreatmentas
languageassessmentscanhelp
todeterminetheeffectivenessof
theseinterventions.
Ithasbeensuggestedthatspeech
andlanguagetherapyshould
beprovidedassoonaspossible
aftertheonsetofthedisorder.All
interventionmustbetailoredto
theindividualneedsofthechild
andmustincludeahighdegree
offlexibilityandresponsiveness.
Manychildrenareveryvariable
intheirperformance,andthe
disorderisalsoverychangeable,so
itisinevitablethatanyprogramme
willneedfrequentreviewand
adaptation.Inputduringthe
earlystagesorfollowingmedical
interventionshouldbeintensive
andhavehighprioritytomaximise
thechild’spotentialforprogress.
Speechandlanguagetherapy
islikelytobenecessary(forthe
majorityofchildren)onalong-
termbasis.LKSisararesyndrome
andspecialistadviceshould
besoughtasrequiredtohelp
determinethemostappropriate
formofintervention.
��
Itisappropriateforrestorationand
developmentofspokenlanguage
comprehensionandexpressionto
beafirstgoalfortheintervention
programme.Thismay,however,
needtobeadapteddependingon
theresponsivenessandprogressof
thechildovertime.Consequently,
abroadbasedfunctionalapproach
thatbuildsonresidualskillswhile
maximisingthechild’sstrengthsis
recommended.Suchapragmatic
methodenablesthedevelopment
ofarangeofcommunicationskills
andtherapygoalscanbeadapted
dependingonthechild’sprogress
overtime.Forchildrenwithsevere
languageproblems,thefocusmay
beonprovidinganalternative
meansofcommunication(for
example,symbolsorsigning)to
ensurethatthechildcanstill
communicatetheirneedsand
interests.Forthosewithmildto
moderatelanguageproblems,the
focuswouldbeondeveloping
areasofweaknesstofacilitate
thechild’sabilitytocommunicate
moresuccessfullythroughspoken
language.
Visual cues and alternative communication
Visualcuesareanimportant
supportasthebrainstillprocesses
visualinformationrelatively
normallyandthiscantherefore
beusedtocompensatefor
problemswithprocessingauditory
information.Thisenablesthechild
tocommunicatedespitetheir
difficultieswithspokenlanguage
andthiscanreducethefrustration
andbehaviouralproblemsthat
sofrequentlyarise.Visualcues
takeanumberofdifferentforms
includingsigns,pictures,symbols
andwrittenlanguageetc.Some
childrenwithLKShaveparticular
difficulties(forexample,with
gesture,interpretingvisualcues
includingfacialexpressionorlip-
reading)thatcanmakeitvery
difficultforthemtousesomeof
thealternativecommunicationmethods.
Childrenintheearlystagesof
thediseaseorthosewhohave
notregainedsufficientspoken
languagetoenablethemtouse
thisfunctionally,maybenefitfrom
signingwhichhastheadvantages
�0
ofbeingquick,portableandnot
dependentonhavingspecific
picturesorsymbolstohand.
Experiencewithchildrenwhosign
showsthatthiswillnotprevent
themfromdevelopingspoken
languageiftheyarecapableof
thisandindeed,therehasbeen
somesuggestionthatitmayeven
helptopromoteit.Somechildren
benefitfromsystemssuchas
Makaton,whichprovidebasiclevels
ofsigning.Othersprogressbeyond
thistoamoresophisticatedsystem
suchasBritishSignLanguage
whichenablesthemtoexpress
themselvesusingcomplexlanguage.
Signingisnotsuccessfulinall
childrenandshouldthereforebe
monitoredcarefullytodetermineits
usefulness.Nevertheless,gauging
thesuccessofsigningisdependent
onprovidingthechildwith
adequateopportunitytolearnthe
signsanditisimportantthatthese
areusedconsistentlyacrossthe
wholedayandinallcontexts(for
example,homeandschool).
Forchildrenwithmoresignificant
learningdifficultiesorautistic
spectrumtypeproblems,theuseof
concretevisualcuessuchasobjects,
picturesorsymbolscanbevery
helpful.Visualcuesprovidethe
childwithmoretimetoprocessthe
informationcomparedtospoken
languageorsignlanguageand
tendtolookliketheobjectthey
arerepresentingtherebyproviding
thechildwithmoreconcreteclues.
Visualcuescanalsobeusedina
waythathelpstomakethetwo-
waynatureofthecommunication
processmoreexplicit(forexample,
handingapictureofthedesired
objecttoanotherperson)whichis
importantforchildrenwhodonot
readilyunderstandthisprocess.The
PictureExchangeCommunication
System(PECS)isanexampleofa
programme,whichaimstodevelop
theunderlyingunderstanding
ofthecommunicationprocess.
Childrenaretaughtexplicitly
aboutthe‘giveandtake’nature
ofcommunicationthroughexplicit
demonstrationofthisprocessby
actuallyhandingoverapictureor
symbolastheymaketheirrequest.
PECSalsoencourageschildrento
initiatecommunicationratherthan
waitforotherstoapproachthem.
Itisimportanttochoosehighly
motivatingmaterial.
��
Cuedarticulationinvolvesthe
useofsimplehandsignstoshow
thepositionofthetonguefor
consonantsoundsinchildrenwho
havearticulationproblems.
Writteninstructionscanbeuseful
asameansofsupportingor
supplementingspokeninstructions
inchildrenwhocanread.Those
whoarebeingtaughttoread
maybenefitfromtheadditional
useofcolourtoreinforcethe
differentcategories(nouns,verbs
etc)asdescribedbyLea.Vancealso
describedtheprocessof‘graphic
conversation’todevelopreading
skillsthroughtheuseofspeech
balloonstorecordachild’sstory.
SeetheReadinglistonpage48for
furtherdetails.
Ithasbeensuggestedthata
visualratherthanaphonological
approachtoteachingreadingmay
bebest.Thiswouldmeanteaching
thechildthewholewordatonce
(usuallywrittenunderthevisual
symbolorpicture,orevenstuckto
therealobject)andallowingthem
torecognisetheoverallpattern
ofthewrittenword,ratherthan
soundingouttheindividualletters
andthentryingtoblendthemand
pronouncetheword.Onceachild
hasreadingskills,thisinturncanbe
usedtoimproveauditoryanalysis.
Auditory training
ManychildrenwithLKShaveshort-
termauditorymemoryproblems
aswellasproblemsprocessing
individualsoundswithinwords
(animportantskillforacquiring
literacy).Strategiessuchas
repetitionofspokeninstructions,
reducingspeechrateand
backgroundnoiseordistraction
areparticularlyimportantfor
classroommanagement.Specific
auditorytraininghasbeenused
alongsidemoretraditionaltherapy
todeveloptheskills,which
underpinlanguagedevelopment.
Somehaverecommendedtheuse
ofFMamplificationsystemswitha
lowgainoutputintheclassroom
asameansofhelpingthechild
tofocusontheclassteacher’s
voice.Thisdoeshowevermakeit
difficultforthechildtoengagein
classroomdiscussionwithpeers,
andmaybedifficultforthechild
totolerate.
��
Social interaction and communication
Asmentionedearlier,children
mayexperiencedifficultywith
aspectsofsocialinteractionand
communication.Thiswillimpair
theirabilitytorelatetopeersand
formormaintainfriendships.They
maycontinuetoshowpleasurein
certainactivitiesbutfailtoshare
thispleasurewithothersthrough
languageorothercommunication
modes(forexample,eyecontact,
facialexpression).Theymaymake
inappropriateremarksorbehave
insociallyunacceptableways
withlittleawarenessofthesocial
implicationsofthesebehaviours.
Forchildrenwhohaveverylittle
language,theremaybefailureto
compensateforthisproblemby
gesturingormiminginorderto
gettheirmessageacross.Some
childrenwhoareabletoproduce
languagemayhavedifficultyusing
theirlanguagesociallyorengaging
inatwo-wayconversation.They
mayecholanguagearoundthem,
orreproducesetlearntphrases
inaninappropriateway.In
addition,thesechildrenmayhave
problemswithabstractthought
orgeneralisingfromexperience.
Theymayhavedifficultywith
imaginativeplayandsomechildren
showobsessionalandrepetitive
behaviour.Theymayalsofind
unstructuredsituations,suchas
theplayground,andperiodsof
changeortransitionverydifficult,
preferringtosticktofamiliar
routines.
Althoughtheseproblemsare
acquiredusuallyaroundthetimeof
thechild’sillnessratherthanbeing
developmental(thatis,present
sinceinfancy),theysharemany
similaritiestochildrenwithautistic
spectrumdisorders.Forsome
childrenanadditionaldiagnosisof
autism/autisticspectrumdisorder
maybeappropriatewhilstinother
cases,theirbehaviouralfeatures
willnotamounttoafulldiagnosis,
butremediationstrategiesrelevant
tothispopulationmaynevertheless
berecommended.
Ageneralemphasisontheuse
ofstructureincludingdaily
schedulesasdescribedinthe
TEACCHapproach(Treatmentand
EducationofAutisticandRelated
��
Communicationhandicapped
Children)canbeusefulinterms
ofitsabilitytoconveymeaning,
predictabilityandordertothe
child.Theuseofvisualcues(see
page19)canbeveryuseful.
Socialskillstrainingmaybeuseful
forchildrenwhoareexperiencing
problemswithsocialinteraction
andcommunication.Theevidence
(basedonchildrenwithautism)
suggeststhatthereisoftena
perceivedbenefitbythechildand
parentsalthoughtheseskillscanbe
verydifficulttoteachandtransfer
toeverydaysituations.Inaddition
toformaltraining,manychildren
benefitfromsupportaimedat
providingthechildwithskillsto
useinsocialsettings(forexample,
teachinggameswhichcanthenbe
re-enactedintheplayground)as
wellaspracticalhelpforspecific
situationsastheyarise.
Therearealsosomechildrenwho
donothaveautisticspectrum
disorder,butwhorespond
negativelyandavoidsocial
situationsasanunderstandable
reactiontotheirlossoflanguage.
Itisimportanttorecognisethese
difficultiesastheyhavesignificant
implicationsforclassroomlearning,
behaviourandthedevelopmentof
socialrelationships.
Other cognitive abilities
Non-verbal skills
Asdescribedabove,LKScausesa
significantimpairmentoflanguage
skills,usuallyintermsofboth
understandingandspeaking.
Althoughverbalabilitiesare
probablyourmostobvioussetof
skills,eachindividualalsopossesses
arangeofother‘cognitive’abilities
contributingtotheirintelligence,
oftenreferredtoasnon-verbalor
‘performance’skills.Asthename
suggests,theseunderlieournon-
verbalunderstandingoftheworld
andinchildrentheyincludeskills
suchasvisualmatching,drawing,
designandconstruction,geometry,
andmathematicalproblemsolving.
Thesenon-verbalabilitiesmaybe
assessedusingavarietyofdifferent
psychometrictestsorintelligence
tests.Dependingonthechild’s
agethesemayincludetaskssuch
asinsetpuzzlesorjigsawpuzzles,
drawingandcopying,and‘block
design’(constructingageometric
��
patternfromcolouredblocks).
Accurateassessmentoftheseskills
canbeverydifficult,however,
ifthechild’smotorskillsand/or
attentionandconcentrationhave
beenaffected.
Asageneralrule,non-verbal
skillsarerelativelysparedby
LKS,thatis,thereisoftensome
impairment,butusuallythisis
lessseverethanthelanguage
deficits(andsometimesthereis
nomeasurableimpairmentatall).
Thishasimportantimplications
forthechild’seducation(see
schoolsectiononpages33to39),
asitisimportanttocontinueto
usethesepreservedvisuo-spatial
skillsinordertooptimisetheir
developmentlong-term,andalso
toboostself-esteematatime
whenmanyofthechild’sabilities
havebeentakenawayfromthem.
Amoresevereimpairmentof
non-verbalabilitiesissometimes
seen,however;thatis,equivalent
severitytothelanguage
impairment,suchthatthereis
anevenor‘global’patternof
delayinthechild’sdevelopment.
Theclinicalimpressionisthatthis
picturepredominantlyaffects
childreninwhomtherehasbeen
anearlyonsetofLKS.Where
therehasbeensomesignificant
impairmentofnon-verbalabilities
associatedwithLKSregression,
theyareoftenthefirsttorecover
oncethechildstartstomakegainsagain.
Somespecificstrategiesfor
supportingchildrenwiththis
patternofdifficultiesaresetout
intheUsefulTeachingApproaches
sectiononpages36-38.
Memory and attention
LKSmayresultinthechildhaving
specificdifficultieswithmemory
andattention,particularlyrelated
toverbalmaterial.Ifthereisa
moderatelyseveredegreeof
generalcognitiveorlanguage
impairmentthenanysuchspecific
deficitsmaynotbemeasurable.
However,forchildrenwhose
cognitiveimpairmentisnotsevere
(orhasrecoveredsignificantly)
specificmemoryproblems
��
maybecomeapparent.These
specificdifficultiesareadirect
consequenceoftheabnormal
brainfunctioningthatoccursin
LKS,particularlyaffectingthe
fronto-temporalregionsofthe
brain,whicharecloselyinvolvedin
memoryprocesses.
Ifthesedifficultiesaresuspected,
afullneuropsychological
assessmentshouldbecarried
outbyaclinicalpsychologistto
determinethepatternandseverity
oftheproblem.Intermsofverbal
memory(whereproblemsare
mostoftenexpected),careshould
betakentotryanddifferentiate
betweenproblemsthatstemfrom
thechild’sdifficultyattending
toand/orprocessingincoming
information(thatis,relatedto
aprimaryauditoryprocessing
problem)andanyadditional
difficultiesrelatedtostoringthis
information.
Dependingonthepatternof
difficultiesfound,avarietyof
strategiescanbeemployedat
homeandatschooltominimise
theconsequences.Theseinclude
usingsimplevisualmnemonics
(memoryprompts),timetables,
checklistsofwhattotake
toschool,etc.Somefurther
suggestionsaregivenintheUseful
TeachingApproachessectionon
pages37-38.
BehaviourItisestimatedthatatleasthalf
ofchildrenwithLKSexperience
neuropsychologicaland
behaviouraldifficultiesasaresult
ofthecondition.Awiderange
ofspecificdifficultieshasbeen
observed,withthemostcommon
categoriesdescribedbelow.
��
Attention deficits, hyperactivity and aggression
Somefeaturesofpoorattention
orconcentration,orover-activity
affectmanychildrenwithLKSat
somepoint,andthesemaybe
associatedwithirritabilityand
aggression(thatisoftentowards
particularfamilymembers)insome
cases.
Inthemostseverelyaffected,these
featuresmaybeconsistentwith
AttentionDeficitHyperactivity
Disorder(ADHD)andthechild’s
abilitytoengagemeaningfully
withtheirenvironmentismarkedly
compromised.However,inmany
casesthecharacteristicsare
muchmilderandmayonlybe
noticeabletoclosefamilymembers
orteachers(thechildisabit
more‘bouncy’thanusual,has
becomeslightlyimpulsive,orhas
difficultysustainingconcentration
throughoutawholelesson).In
others,thefeaturesaremarked
butepisodic,forexample,a
coupleofhoursofoveractive
behaviourintheevening,ormay
bemorepronouncedinparticular
environments,forexample,large
gatheringswherethereisahigh
levelofnoiseandstimulation.The
mostcommonfeaturesreported
are:inattention,hyperactivity,
impulsiveness(thatis,notthinking
beforedoingorsayingsomething),
nosenseofdanger,verbaland/or
physicalaggression,moodchanges,
anddisinhibition(failuretoinhibit
inappropriatebehaviour,for
example,makingrudecomments
tounfamiliaradultsorpullingtheir
trousersdowninpublic).
Itisoftenassumedthatthese
behavioursarepurelyaresponse
tothefrustrationfeltbythe
childtothelossoflanguage.
AlthoughmostchildrenwithLKS
doexperienceepisodesofextreme
frustrationandconfusionasa
resultofthecondition,thereis
littleevidencetosuggestthatthis
istheprimarycauseofADHDtype
behaviours.Forexample,attention
difficultiescanpresentbeforethere
isanyapparentlanguagedeficit.In
addition,recoveryofmostareasof
dysfunction,includingbehaviour,
canoccurevenwhensignificant
languagedifficultypersists.Itis
thereforethoughttobeadirect
resultofthecondition(seebelow).
��
However,thesocialandemotional
impactofasuddenlossofabilities
shouldnotbeunder-estimated
andthisfactorwillalmostcertainly
contributetobehaviourpatterns.
Mostoften,ADHD-typeproblems
willshowsomeimprovement
associatedwithimprovement
incontroloftheunderlying
seizureactivityduringsleep,and
withrecoveryfromregression
(andconversely,deteriorationin
behaviourisfoundtoberelated
tothediseaseworsening).Insome
cases,thebehaviourswillresolve
completelyanddramatically
whenthediseaseiseffectively
treated.Inotherinstances
wherehyperactivityisverysevere
orpersistent,itmayrespond
totreatmentwithmedication
thatspecificallytargetsthis
groupofdisorders(forexample,
methylphenidateoratomoxetine).
Itisimportanttotreatthese
ADHD-likedifficultiesintheirown
right,astheymaypreventthechild
fromusingotherskillstolearnand
interact.Itisoftenmosteffective
touseacombinedapproach
throughabehaviourprogramme
andmedication.
Itisthoughtthatthesebehaviours
primarilyresultfrominterference
withthebrain’snormalfunctions,
causedbytheabnormalelectrical
activitythatisassociatedwithLKS
(whetherornottherearefrequent
overtseizures).Thismeansthat
thechildprobablyhasverylittle
controlovertheseaspectsoftheirbehaviour.
However,thereisafurther
acquiredelementthatcanalso
influencetheoccurrenceof
challengingbehaviours.First,
inchildrenwithaverylong-
standingdisorder,poorlyregulated
behaviourmayinpartreflect
thefactthatoneofthemost
importantchannelsforteaching/
learningsuchbehaviouralcontrol
(thatis,oralcommunication)isnot
available.Second,throughsimple
associationchildrenmay‘learn’
thatsomeofthesebehaviours
produceadesirableoutcome,for
example,iftheyhaveatantrum
andthrowthingsaroundwhen
theTVisturnedoff,thensomeone
turnsitonagain.Thismeansthat
thebehaviourwillthenoccurmore
frequentlyasitis‘rewarded’by
theconsequence.Itisimportant
��
thatparentsshouldbeawareof
thispossibilityandstickfirmly
totheirpre-determinedrules
wherepossibleandcontinueto
provideascalmandstructuredan
environmentaspossible.Although
allowancesmustbemadebecause
oftheinvoluntarynatureof
someofthesebehaviours,itis
stillimportanttomakeclear
whatisandisnotacceptable,
andtodevelopstrategiestodeal
withcommonsituations.Studies
haveshownthatbehaviour
managementtechniquesremain
successfulinhelpingthisgroup
ofchildren,despitethefactthat
thebehaviourshaveasignificant
organiccomponent(thatis,are
duetothediseaseprocess,not
simplyasecondarypsychological
reactiontoit).
Usefulapproachesinclude:
• immediateandconsistent
responsestobehaviour
• timeout
• distractiontechniques
• rewardsforpositivebehaviour
andachievements.
Judgingwhetherachildhascontrol
overtheirbehaviourornot,canbe
verydifficult,andtheadviceand
inputofalocalclinicalpsychologist
(oftenfromtheChildand
AdolescentMentalHealthService
orCAMHS)maybenecessaryto
helpresolvesituationswhere
behaviourshavebecomevery
challenging.Itisusuallyhelpfulto
discussthesemattersopenlywith
theschool,sothatappropriate
boundariesandresponsestothe
behaviourcanbeagreedtoensure
aconsistentresponse.
Inchildrenwithmilderdifficulties
involvingmore‘cognitive’
inattentionandimpulsivitythese
strategiesmayhelp:
• playinggamesthatrequire
attentionandmemoryto
encouragetheseskills(thereare
manyexamplesavailable,for
examplefromEarlyLearningCentre®)–butparticular
attentionshouldbepaidtothe
appropriatelevelofdifficultyso
thechildhastheexperienceof
achievement,notfailure.
��
• theparentcountingtoten
beforerespondingtoasituation
thatisupsetting
• discussingbasicrulestohelpwith
impulsivity–“Stop&Think”
• creatingsimplevisual
mnemonics(memoryprompts)
tohelprememberimportant
verbalinformation.
Sleep disorders
ManychildrenwithLKSare
particularlyactiveintheevenings
andparentsreportthatthey
cannotsettletosleepuntillate.
Inothercasestheygooffto
sleepreadilyintheeveningbut
thenhaveprolongedepisodes
ofwakefulnessduringthenight,
orwakeintheearlyhoursand
cannotgobacktosleep.LKSis
particularlyassociatedwithseizure
activityduringsleepsoitisperhaps
notsurprisingthatsomany
childrenhaveproblemsatnight.
Indeed,manyparentsreportthat
theirchildiswokenbytheseizures
themselvesduringthenight.
Alsosomedrugs(forexample,
lamotrigine)maydisturbsleep.
Childrenwhohavedifficulty
gettingofftosleepmaybe
helpedbymelatonin(itisalso
usedtotreatjet-laginthe
adultpopulation).Itisharder
totreatnighttimewaking.It
maynotbepossibleto‘cure’the
underlyingmedicalreasonfor
thesesleepdifficulties,butthe
situationcanusuallybeimproved
byconsistentapplicationof
standardbehaviouralmanagement
strategies.Thesemayinclude:
• aregular,quietbed-time
routine(bath,warmdrink,
beingreadastoryorshowna
picturebook)
• removingTVsandvideosfrom
thechild’sbedroom
• sleepingintheirownbedin
theirownroom(withababy-
monitorifyouareconcerned
thatyouwillnothearthem
whenasleep)
• comfortandreassurewhenyour
childwakesatnightbutdon’t
overdoit(thatis,resistswitching
onallthelights/givingfood/
turningonavideo/stayingwith
themuntiltheyfallasleep).
�0
Other behaviours
Someparentsreportthattheir
childseemsverytearfuland
depressed,andthisshould
becarefullymonitored.
Understandably,manychildren
withLKSwillrequiremore
reassurancethanusualandmay
seekphysicalcomfortingor
becomeanxiousinsocialsituations.
Othersmaybecomemore
controllingoftheirenvironment.
Asmallproportionofchildren
becomepassiveandapatheticin
theirmanner.Inourexperience,
thisismostcommonlyassociated
withamarkedglobalregression
andearlyonset(beforetwo-and-a-
halfyearsofage).
Somechildrenareextremely
irritableandaggressivewith
violentmanicoutbursts.Others
maydevelopobsessionalbehaviour,
anxietyorsevereimpulsivity.They
needpsychiatricreview,andafew
willrequiremedication.
Motor difficulties
Motorproblemsareverycommon,
occurringinaroundtwothirds
ofchildrenwithLKS.Theyoften
relatetothediseaseactivity
(thatis,correspondtoperiodsof
regressionorfluctuation).They
mayincludedyspraxiaorinco-
ordination,tremor,unsteadiness,
jerkymovements,unusuallimb
postures,weaknessorevenneglect
ofoneside.Theymayaffect
activitiessuchaswriting,dressing,
walkingandmaymakeitdifficult
tousegestureandsigning.The
musclesaroundthemouthand
throatarecommonlyinvolvedand
willcausedifficultieswithfeeding,
controllingsalivaandspeech.
Incertaincases,thechildmay
experienceweaknessfollowinga
clinicalseizure(Todd’sparesisor
postictalparalysis)orsometimes
lossofspeech(postictalaphasia).
Theseimmediatepost-seizure
difficultiesusuallygetbetterover
somehoursoroccasionallydays.
Howeversomechildrenchange
handpreferencefollowingthis
typeofepisode.
��
General support principles
Languageistheeasiestand
quickestwayformostofusto
communicate,findoutinformation
andrecordideas.Wedothis
throughspeech,readingand
writing.Ofcourseitisnotthe
onlyway,peoplealsousefacial
expressions,gestures,symbolsand
soon.Butformostofusandfor
theworldaroundus,language
isfundamentaltohowwelive.
ForthechildwithLKS,theeffect
onlanguagemaybesuchthat
theworldremainsfamiliarbutis
subtlytransformedsopeopleuse
alanguageyoucan’tunderstand
orspeakyourself.Youmighttryto
guesswhatishappeningfromclues
aroundyou,butitwillbevery
tiringandunrewarding.
Becauselanguageisfundamental
tosomuchofwhatwedo,
thechildwithLKSneedsa
comprehensiveprogrammeto
supportthemthroughouttheday,
athomeandatschool.Thisismost
effectivelyachievedifeveryone
iscommittedtostrategiesthat
helpcommunicationforthechild.
Thesestrategieswillvarywith
thechildanddiseaseseverity,
butwillincludecommonthemes
suchassimplifyinglanguage
andthelisteningenvironment,
offeringalternativecommunication
strategiesandprovidingvisual
reinforcement.
Childrenwholosetheabilityto
understandenvironmentalnoise,
willneedspecialsupportand
supervision.Certainsituations
willbemoredangerousforthem
forexample,astheycannot
detecttrafficnoiseorwarning
shouts.Theymayfindcrowded
environmentsandgroupsituations
distressing,astheynolongerhave
anauditoryforewarningofwhat
isabouttohappen,orwhatis
expectedofthem(thiscanalsobe
trueforchildrenwhoretainsome
language,butwhofinditdifficult
topickoutspeechinanoisy
environment).Evenplayingteam
games,suchasfootball,where
teammemberssignaltoeachother
verbally,canbedifficult.
Somechildrenbecomevery
sensitivetoandintolerantof
certainnoisesorevenmusic.
Thisisprobablyduetothebrain
��
processingthesoundinanunusual
way,suchthatitisperceivedas
anunpleasantstimulus.Thismay
restrictfamilyoutings,ascertain
noises(forexample,tannoy
announcements)canbevery
distressingforthechild.
Thefamilyprovidesthemain
careforthechild.Parentsare
usuallywiththeirchildmost
frequently,andarethebestsource
ofinformationaboutthechild
throughouttheillness.Theywill
oftendetectchangeinthechild’s
condition,beforeitisformally
apparent.Theyacceptandnurture
thechild,providestructureand
sensetotheirworld,andwillbe
themaincommunicationpartners.
Theyshouldbeactivelyinvolvedin
decisions,andgivenappropriate
informationandsupport,including
opportunitiestolearnspecialskills
(forexample,signing,PECS)that
canbeusedathome.
Inadditiontolanguage,the
childwithLKSoftenexperiences
difficultiesinotherareas(for
example,behaviour,motorskills
andnon-verbalunderstanding).
Thesemustbetackledwithan
integratedapproachthatsupports
thechildinallenvironments.Thus
thelocalteammustbeableto
drawonawiderangeofservices
andskills(language,psychology,
psychiatry,physiotherapy,
occupationaltherapy,social
work)inordertoprovidean
appropriatelytailoredprogramme.
Therapists(speechtherapists,
psychologists,autismadvisory
serviceetc)areskilledat
establishingachild’sstrengthsand
weaknesses,andatidentifyingthe
bestapproachestosupportthe
child.Theywillworkcloselywith
classteachersandassistantsand
manyoftheirrecommendations
willbeimplementedthrough
classwork.Regularreviewsare
importanttojudgethesuccess
ofanyschemesandtomonitor
thechildforrapidchangesin
ability.Rapidgainsmaymerit
intensivetherapytooptimise
therecoveryphase.Rapidlosses
willmeanthatthechildneeds
moresupport,perhapsevennew
waysofcommunicating,andany
deteriorationshouldbebroughtto
medicalattention.
��
School
Schoolprovidesavitalframework
forachild’srecoveryand
management.Itisthekeymedium
throughwhichteachersand
therapistscansupportthechild’s
learningandhelpmakesenseof
theirworld,aswellasproviding
astablesocialstructure.Given
thecomplexandunusualnature
oflearningdifficultiesassociated
withLKS,andthebehavioural
problemsthatmayalsobepresent,
identifyingasuitableeducational
placementcanbedifficultandwill
dependontheindividualpattern
ofabilitiesanddifficultiesineach
childandtheabilityoftheschool
tomeettheseneeds.
Educational challenges
Whateverformofschool
placementischosen,achildwith
LKScontinuestoposemany
challenges,whichtheschoolmust
adaptto,mostnotably:
1. Theirconditioncanchange
rapidlyovertime,thatis,
‘fluctuate’,makingprogress
atschoolerratic,andsupport
needstoberesponsiveto
this.Regularmonitoringand
updatingoftherapeuticand
educationalplansisnecessary
2. Whenthechild’sdiseaseis
active,performancecanvary
evenwithinaday,making
themsusceptibletofatigueand
difficultieswithconcentration.
Teachers/LSAsmustbemade
awareofthisandcareful
timetablingoflessonsmayhelp
tominimisetheimpact
3. Despitehavingsignificant
languagedifficulties,many
childrenwithLKSretainaverage
oraboveaverageabilitiesinthe
non-verbaldomain.However,
becausestandardclassroom
presentation(instructionand
soon)isalmostinvariably
verbal,thismeansthataspecial
teachingapproachmustbe
devised(seebelow).Itisvital
thatthesegoodskillsare
recognised,andthatitisnot
assumedthatthechildhas
generallearningdifficulties,
simplybecauseofthelanguagedifficulties
��
4. Othercognitiveeffectsof
LKSsuchasslowprocessing
andimpairedverbalmemory
makeitevenharderforLKS
childrentounderstandwhat
isrequiredofthem.For
example,childrenwithLKSmay
understandlanguageinaquiet
onetoonesituation,butina
noisyclassroomthelistening
environmentisverycomplexand
thechildmaywellbeunable
todecipherthesameauditory
information.Inotherinstances,
thechildmayunderstand
spokeninformationatasimple
level,buthaveauditorymemory
problemsthatmeanthatthey
arequiteunabletoremembera
sequenceofverbalinstructions
orastory–whichwouldcause
enormousdifficultyinclassand
alsowithplaymates.However,
theseverityofthisdifficultymay
bemaskedbytheabilitiesthat
arepreservedandbycleveruse
ofwell-learnedsocialbehaviours
(childrenusuallywanttocover
upwhattheycan’tdo)and
thismaybemisconstruedas
‘naughtiness’.Usefulstrategies
fortacklingmemory/processing
problemsaredescribedbelow
5. LKSisassociatedwithanumber
ofbehaviouraldifficulties
thatmaybeverydisruptive
tolearningandschoollife,
forexample,poorattention
andconcentration,social
communicationproblems,
aggressiveoutbursts.(Amore
detaileddescription,including
suggestedcopingstrategies,is
setoutinthe‘Behaviour’section
onpage25-30).
Statement of Educational Needs
Childrenwitheducationalneeds
areoftenfirstidentifiedand
placedontheSchoolActionor
SchoolActionPluslevelofthe
CodeofPractice.Iftheselevelsof
supportareinsufficienttomeet
thechild’sneeds,aStatement
ofSpecialEducationalNeeds
mayneedtobeproduced.The
statementingprocessiscarriedout
byyourlocaleducationauthority
andmaytakeseveralmonths,
involvingassessmentsbylocal
educationalpsychologistsand
speechandlanguagetherapists.It
��
shouldautomaticallybereviewed
annuallyalthoughaparentor
schoolcanaskforareviewto
bebroughtforwardifthereisa
markedalterationincircumstances
(forexample,aregression).
Thestatementwillsetoutyour
child’scurrentlevelofabilityand
highlightthekeyareasofdifficulty
(bothintermsofabilitiesand
behaviour),recommendingwhat
levelofsupport/inputisrequired
tooptimisetheirprogress.Each
schoolhasanominatedspecial
educationalneedsco-ordinator
(SENCO)whoshouldthentake
responsibilityforimplementing
therecommendations.This
shouldincludecarefulplanning
anddrawingupofanindividual
educationplan(IEP),specifyingthe
waysinwhichyourchild’slearning
willbesupportedandteaching
methodsadaptedtofacilitatethem.
NOTE:Thewayinwhichprovision
isdeliveredwillberatherdifferent
ifyourchildisbeingeducated
withintheprivatesystem.
Placement
Inchildrenwhoshowgood
recovery,mainstreameducation
maybethemostappropriate
placement.Forsomechildren
whoshowamoderatedegreeof
recovery,mainstreamschoolingcan
becontinuedwithadultsupport
(forexample,one-to-onehelp
providedbyalearningsupport
assistantor‘LSA’)toprovidea
semi-adaptedcurriculumthatis
appropriatetothechild’slevels
ofability.Forotherchildrenwho
havemorespecificneeds,itmaybe
necessarytoconsideralternative
settingstoensureawholeschool
approachtothechild’sparticular
needs.
Childrenwithaprofoundlanguage
losswillusuallybenefitfrom
learningsignlanguage(alongwith
theirfamilies).Theymaybewell
accommodatedinlanguageunits
wherethereisspecificexpertise
indealingwithchildrenwith
languagedisorders(althoughitis
importanttocheckforanygiven
unit,theparticularfocusand
provision).Othersmaybemore
appropriatelyeducatedinschools
��
orunitsforchildrenwithhearing
impairment.However,although
inmanyways,thechildwithan
inabilitytounderstandspoken
languagebecauseofLKSresembles
thechildwithhearingloss,there
aredifferencesandtheseshould
beaddressedintheireducational
plan.
Wheremoregenerallearning
difficultiesexist,schoolsthatcater
foranoverallslowerpaceof
learningmaybethebestoption.
Finally,thosewithpervasive
developmentaldisordersorautistic
spectrumdisordersmaybebest
placedinschoolsorunits,which
caterforchildrenwithautism.
Useful teaching approaches
Itisessentialtousestrategies
thatallowthechild’sgoodskills
tocontinuetodevelop,asthese
mayultimatelybethewaythe
childcompensatesforanyresidual
deficitsandisabletofunction
inlaterlife.Visualprocessingis
usuallyrelativelysparedandcan
thereforebeusedtocompensate
forproblemsinprocessingauditory
informationandasanalternative
modeofcommunication(seepage
19-21).
Eachchild’seducational
programmeneedstobecarefully
tailoredtomeettheirparticular
needs.Itmaybeimportantto
allocateresourcetoactivitiesthat
arenotobviouslyeducational,
butwhichareimpairinga
child’sfunctionsignificantly.For
example,thechildwhofindssocial
interactiondifficultmayneed
additionalhelpinunstructured
situationssuchastheplayground.
Otherchildrenwouldbenefit
fromhelptotacklebehavioural
problemsthatmightotherwise
takethemoutofthelearningenvironment.
��
Specific patterns of impairment
- Good non-verbal skills in
conjunction with language
impairments
TheIndividualeducation
plan(IEP)whichisproduced
bytheschoolanddetailsthe
objectivesforthechildwill
needtospecifywaysinwhich
pictorialandsymboliccues
canbeusedtobackupverbal
explanations.Wherethereis
amoderateorseveredegree
oflanguagedeficititmay
alsobenecessarytoadaptthe
contentofschoolworksothat
heavilylanguage-basedtasks
orclasses(forexample,English)
aresignificantlymodified.It
isworthnotingthatalthough
numberconceptsaregenerally
consideredtobenon-verbal,
mentalarithmetic(whichforms
asubstantialandfundamental
partofearlyyearsmaths
teaching)isaverbalskilland
reliesonmemoryandmay
thereforebeverydifficultfor
childrenwithLKS.Anadditional
unusualfeatureaffectingsome
childrenwithLKSisthatspelling
andwritingskillsthathave
alreadybeenacquiredmaybe
retainedduringanepisodeof
regression,sothatthechildmay
stillbeabletowriteandspell
wordsthattheyarenotable
tounderstandorproducein
speech.
- Impairment of verbal memory & auditory processing
Wherethechildhasretained
areasonableleveloflanguage
comprehensionthenthe
followingwillusuallybehelpful:
• repetitionofverbal
instructionsseveraltimes
• preferentialseating(thatis,
closetotheclassteacher)
• reducingspeechrate
• reducebackgroundnoiseand
distractions
• shortandsimplewritten
(orsymbolic)formsof
communicationwhere
possible
• breakworkdownintosmall
chunks
��
• allowlongerforthechildto
respondtoquestions
• lowerexpectationsforwork
subjectsthatareveryreliant
onverbalmemory(for
example,history,geography)
• useofcomputers(supported)
astheauditoryrequirement
isminimalandthereisgood
scopeforvisualcuesusing
attractivegraphicsetc.
Note:Itwillalmostcertainly
benecessaryforachildto
haveone-to-oneclassroom
supportinorderforthese
recommendationstobe
implemented.
- Poor attention and
concentration
Manyoftherecommendations
fromabovewillapply.
Inaddition,thesemayalsobe
useful:
• aquietanddistraction-free
classroomenvironment(asfar
aspossible)
• smallclass-sizes
• structurethedaysothattasks
requiringmostattention
arescheduledforthetime
ofthedaywhenthechildis
mostattentive(usuallythe
morning)
• giveplentyofopportunityfor
positivefeedback
• ensureyouhavethechild’s
attentionbeforepresenting
themwithatask
• organisationalprompts,for
example,topickupwork-
sheets,ortakecertainthings
tothenextlesson
• startwithveryshortperiods
ofsustainedfocusand
graduallyincrease
• rewardperiodsspent
concentratingonworkwith
shortperiodsof‘relaxing’
withsomethingthechild
findseasierandenjoyable
(oftenanon-verbaltask).
��
Key elements for a successful placement
Ingeneral,thefollowingaresome
keyelementsinanysuccessful
schoolplacementforachildwith
LKS:
• comprehensiveandflexible
approachgivingappropriately
targetedsupportthroughout
theday
• goodcommunicationbetween
parentsandschoolinorderto
capitaliseonnewdevelopments
inthechild,andachieve
consistencyinmanagementof
anydifficulties
• regularmonitoringofthechild’s
abilities(byspeech&language
therapists,educational/clinical
psychologists,occupational
therapists,physiotherapists
andsoon)andeffective
disseminationofthis
informationandrelated
recommendationsorstrategies,
fromtherapiststotheschool
andtoparents
• teachersandsupportassistants
whoaremotivatedtolearn
aboutLKS,aresensitiveto
changesinthechildandflexible
intheirresponsestothis,and
canconsistentlyimplement
suggestionsfromparentsand
therapiststomaximisethe
academicandsocialpotentialof
thechild
• appropriatepeergroupthatis,
agroupofchildrenwithsimilar
skills,difficultiesorinterests,
thatcanprovideasocial
networkandfriends
• educationofthechild’s
peerssothattheyhavesome
understandingofspecific
difficultiesandappropriate
behaviourandresponses.It
maybehelpfultousea‘Buddy’
schemetosupportthechild.
�0
What does the future hold? (prognosis)
Somechildrenexperiencegood
recovery,butmanyareleftwith
significantresidualimpairments,
anditmaybethatthereisacritical
periodforrecovery,outsidewhich
childrenareleftwithirreparable
damage.Outcomeappearsto
berelatedtothelengthoftime
oftheactivephaseofLKS.Itis
generallybetterinchildrenwith
late-onsetdisease(languageloss
aftertheageofabout5years),
andinthosewithshorterperiods
ofdocumentedelectricalstatus
epilepticusinsleep–ESES(thereis
researchsuggestingthatchildren
withESESlastinglessthanthree
yearshavebetteroutcome).
Relatedtothis,childrenwho
respondtomedicaltreatmentof
theregressionsandoftheESES
tendtohavebetterprognosis,
althoughresponsetotreatmentof
theclinicallyvisibleseizures,does
notgenerallyaffectoutcome.Ina
smallnumberofchildren,clinical
seizuresareasignificantand
continuingproblemintheirownright.
Thedevelopmentalprofilealso
hasaneffectonprognosis.
Childrenwhoareknowntohave
haddifficultiesintheirearly
languagedevelopment,priorto
LKSonset,appeartohaveaworse
outcome.LKSitselfoftencauses
difficultiesinmanydevelopmental
areas.Thosechildrenwherethe
acquireddifficultiesarelimitedto
languageappeartodobetterand
oftenrespondbettertomedical
treatment.Forthosechildrenwith
additionalacquiredimpairments,
itisoftenthedifficultiesinsocial
communicationandinteraction
orgenerallearningproblems
thatposethegreatestbarriersto
recovery.
LKSmaybebestthoughtofas
aspectrum,inwhichlanguage
tendstobefirstandmostseverely
affected,butinwhichmanyother
skillsmaybeinvolved.Giventhis,it
isverydifficulttopredictoutcome,
asitdependsontheparticular
child’sskillprofile,thedisease
process(ageofonset,number
andseverityofregressions,length
ofactivedisease,responseto
treatment),andtheirprogressin
differentskillareasovertime.
��
Theactivephaseoftheepileptic
diseasetypicallyendsaround
adolescenceandthechild’sgood
skills,andremainingareasof
difficultyshouldbecomeclearer.
Howeverthereissomeevidence
thatsomerecoverycancontinue
intoadultlife.
Itisthoughtthatingeneralterms,
abouthalfofthechildrenmakea
reasonablerecovery,aquarterhave
apartialrecoveryandafurther
quarterhaveverysignificant
persistingdifficulties.
Languageoutcomevaries
significantly.Childrenwithagood
outcomeareintheminoritybut
theyusuallyregaincompetencein
spokenlanguageandtendtoscore
withinthenormalrangeonformal
assessments.Eventhosewithgood
outcomehowever,mayexperience
difficultiesofamoresubtlenature,
suchasproblemswithshort-term
memoryanddifficultieslistening
inthepresenceofnoise.Those
withamoderateoutcomewill
demonstratesomedegreeof
languageimpairmentbutspoken
languagewillusuallybetheirself-
chosenmeansofcommunication.
Thosewithapooroutcomemay
neverregainspokenlanguage
butmaybeabletodevelopskills
usingothercommunicationmodes
suchassignlanguage,pictures
orsymbols.However,becauseof
additionaldifficultieswithgesture
andfinemanipulation,signingmay
notbesuccessful,andthereare
reportsthatlip-readingskillsmay
alsobedifficultforthechildrentoacquire.
��
Family adjustment and support
TheexperienceofLKSislikelyto
bebewilderinganddistressing
bothforthechildandtheirfamily.
Somechildrenmaybeveryaware
oftheirlossofabilitiesorsudden
difficultiesrelatingtotheirfriends,
andthosewithsevereimpairments
oflanguageandcomprehension
mayfindthisveryfrighteningand/
orfrustrating.Itisnotunusualfor
themtodeveloppoorself-esteem
andlowmoodastheyadjust
totheirlosses.Itisimportant
tosupportthechildasmuchas
possibleduringthisdifficulttime,
byfacilitatingopportunitiesfor
themtospendtimewiththeir
existingfriendsandalsocreating
opportunitiesforthemtofinda
newandappropriatepeergroup,
perhapsdrawnfromotherchildren
withlanguagedifficulties,learning
difficulties,orevenfromthedeafcommunity.
Forparents,thereisthevery
painfulexperienceofhavinghad
anormalchildwhoisapparently
lost.Inadditiontotheanxietyand
distresscausedbyvisibleseizures
andtheneedformedicationor
othertreatments,parentsmust
findwaystocopewithachildwho
suddenlycannotunderstandthe
worldastheydidbefore,whomay
bedistressedandfrightened,and
whomayhaveextremelydifficult
behavioursandanapparent
‘personalitychange’.Manyparents
reportthatthebehavioural
changesintheirchild,particularly
aggressionandsleepdisturbance,
arethehardestthingtodealwith.
Aswellasthedemandsofcaring
fortheirchildwithLKS,there
arealsotheneedsofanyother
siblingstoconsider,whomaybe
bewilderedandresentfulofthe
attentionpaidtotheirbrotheror
sister.Changesinthebehaviourof
achildwithLKScanalsodirectly
leadtodeteriorationinsibling
relationshipsandincreasesin
fighting,anothercauseoffamilystress.
Siblingsmayneedinformation
aboutwhathashappenedtotheir
brotherorsister,andguidanceon
theirrole,particularlyastheytoo
mayhavelostacloseplaymateand
nowbethetargetofaggression.
��
ThecourseofLKSis
characteristicallyvariableand
fluctuant,andthetreatments
arenotcertain,soitmaybe
impossibletodetectanysteady
progressinachildortopredict
theirfutureoutcome,andthiscan
beparticularlydiscouragingfor
parents.LKSisararediagnosisand
theremaybelittlelocalknowledge
orexperienceofthecondition
sothatparentsfindthemselves
spendinghoursonthetelephone
tryingtodealwithlocaleducation
andhealthservicestoensurethat
theirchild’sdevelopingneedsare
met,orfacedwithalargenumber
ofdifferingviewsandapproaches
bysuccessiveprofessionals.This
canbedaunting,frightening
andexhausting.Itiscommon
forparentstofeelcompletely
overwhelmedattimes,anditis
possiblyalltheharderthatthere
isnoidentifiable‘event’suchasa
headinjuryorinfection,toexplain
suchadevastatingeffectontheir
child.Itisimportantforparentsto
identifylocalsourcesofsupport.
Research
LKSishardtoresearchasthe
conditionisrareandanycentre
seesrelativelyfewchildren.
Inaddition,thefluctuating
natureofthediseaseprocess
andseizureactivitymeansitis
hardtointerpretobservations.
Despitethis,thereisatremendous
interestinthisgroupofchildren,
asunderstandingtheircondition
wouldshednewlightonmany
areasofepilepsy,languageand
behaviour.
ThereisaspecialinterestinLKSat
GreatOrmondStreetHospital,and
thereareactiveplansforresearch
intothecondition.
��
Useful contacts
Therearevarioussourcesofbothpracticalandemotionalsupportfor
parentsofchildrenwithLKS,andalistofrelevantorganisationsisgiven
below.
FOLKS (Friends of Landau Kleffner Syndrome)
3StoneBuildings(GroundFloor),Lincoln’sInn,LondonWC2A3XL
Tel:08708470707
Website:www.friendsoflks.com
Email:[email protected]
KIDS (Range of services provided for children with disabilities including
home based learning, respite care, holiday play schemes and independent
educational advisory service)
80WaynFleteSquare,LondonW106UD
Tel:02089692817
MENCAP (support group and providers of services for people with
learning disabilities)
123GoldenLane,LondonEC1Y0RT
Tel:02074540454Fax:02076083254
Website:www.mencap.org.uk
AFASIC (UK charity representing children and young adults with
communication impairments working for their inclusion in society and
supporting parents and carers)
2ndFloor,50-52GreatSuttonSt,LondonEC1V0DJ
Helpline08453555577Fax02072512834
Website:www.afasic.org.uk
Email:[email protected]
��
Contact-a-Family
209-211CityRoad,LondonEC1V1JN
Helpline:08088083555
Website:www.cafamily.org.uk
Epilepsy Action
NewAnsteyHouse,GateWayDrive,LeedsLS197XY
Helpline08088005050Fax01133910300
Website:www.epilepsy.org.uk
The National Autistic Society (NAS)
393CityRoad,LondonEC1V1NE
Tel:02078332299Fax:02078339666
Website:www.nas.org.uk
Email:[email protected]
Dyspraxia Foundation
8WestAlley,Hitchin,HertsSG51EG
Helpline01462454986Fax01462455052
Website:www.dyspraxiafoundation.org.uk
Email:[email protected]
Hyperactive Children’s Support Group
71WhykeLane,ChichesterPO192LD
Tel:01903725182Fax:01903734726
Website:www.hacsg.org.uk
Email:[email protected]
��
Parent Network (offers courses on parenting skills)
Room2,WinchesterHouse,KenningtonPark,
11CranmerRoad,LondonSW96EJ
Tel02077351214(parentenquiry)Tel02077354596(admin)
Fax02077354692
Skill (National Bureau for Students with Disability)
Providesinformation,adviceandpublicationsregardingpost16
education,trainingandemploymentforpeoplewithdisability
ChapterHouse,18-20CrucifixLane,LondonSE13JW
InformationserviceTel:08003285050Text:08000682422
Website:www.skill.org.uk
Email:[email protected]
Independent Panel for Special Education Advice (IPSEA)
Providesadviceandinformationtoparentswhosechildrenhavespecial
educationalneeds.ProfessionaladviceforparentsappealingtoSEN
tribunal
6CarlowMews,Woodbridge,Suffolk1P121EA
Helpline:08000184016Fax:01394380518
Website:www.ipsea.org.uk
Department of Education and Employment (DfEE) Publications Centre
(forcopiesoftheCodeofPracticeandotherDfEEpublications)
Tel08456022260
Advisory Centre for Education (ACE) Ltd
1bAberdeenStudios,22HighburyGrove,London,N52DQ
Helpline:08088005793Fax:02073549069
Website:www.ace-ed.org.uk
��
Makaton Vocabulary
The Makaton Vocabulary Development Project
31FirwoodDrive,Camberly,Surrey,GU153QD
Tel0127661390
Website:www.makaton.org
Email:[email protected]
The Paget-Gorman Society
2DowlandsBungalows,DowlandsLane,Smallfield,Surrey,RH69SD
Tel01342842308
Website:www.pgss.org
The Royal College of Speech and Language Therapists
2WhiteHartYard,LondonSE11NX
Tel:02073781200
Website:www.rcslt.org
Email:[email protected]
��
Further reading
BishopD(1985)Ageofonsetand
outcomein‘acquiredaphasia
withconvulsivedisorder’(Landau
Kleffnersyndrome).DevMedChild
Neurol27(6),705-712
DeWijngaertE,GommersK
(1993)LanguageRehabilitation
intheLandau-KleffnerSyndrome:
ConsiderationsandApproaches.
Aphasiology(7)475-480
LeaJ(1979)Language
developmentthroughthewritten
word.ChildCareHealthand
Development569-74
LeesJA(1993)Childrenwith
AcquiredAphasia.Whurr
PublishersLondon(secondedition
duelate2004)
NevilleBGR,BurchV,CassHetal
(1998)MotordisordersinLandau-
Kleffnersyndrome(LKS).[abstract],
Epilepsia39(Suppl6)p123
NevilleBGR,BurchV,CassHand
LeesJ(2000)TheLandau-Kleffner
syndrome,inJ.Oxbury,C.Polkey
andMDuchowny(eds).Intractable
FocalEpilepsy:MedicalandSurgical
Treatment,London:WBSaunderspp277-284
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Commonly encountered medical concepts
LKSmayalsobetermed:
• acquiredaphasiaofchildhood
withseizures
• epilepticaphasia
• receptiveepilepticaphasia
–(lossofcomprehension)
• epilepticverbalauditoryagnosia
• epilepticpureworddeafness
Aphasiameansdisturbanceinthe
abilitytouselanguage.Receptive
referstounderstandingor
comprehension,expressiverefersto
useofspokenlanguage.
Agnosiameansthepersonis
unawareoftheirfailureto
recognise,orunderstand.
Child development
Childdevelopmentistheprocess
bywhichchildrenchangeand
increaseintheirabilitiesin
allareas(forexample,motor,
language,social)overtime.Itis
viewedasacontinuousprocess
thatdependsonmaturationof
thechild’sbrain.Thebrainisnot
fullydevelopedatbirthandgrows
andmakesimportantconnections,
‘wiring’,throughouttheearlyyears
oflife.Generallychildrenfollowa
predictablesequence(forexample,
sitbeforetheywalk)althoughat
differentspeeds.Fortheyounger
child,developmentisoften
assessedbyconsideringskillsin
differentareassuchasgrossmotor
(forexample,sitting,walking),
finemotor(forexample,hand
manipulation),vision,language,
cognitiveability(forexample,
puzzlesandproblemsolving)and
personal-socialskills.Forolderand
moreablechildren,itiscommon
toconcentrateonlanguageand
cognitive(non-verbalintelligence)skills.
�0
Delaymeansthatachild’s
developmentisnotasadvancedas
wouldbeexpectedfortheirage
(henceitisoftenreportedasan
‘ageequivalent’)andthisnormally
occurswhenthechild’srateof
developmentisslowerthanusual.
Catch-up
Parentsoftenthinkthatachildcan
bestimulatedtocatch-upandthen
performatthesamelevelasother
childrenofasimilarage.Thisdoes
notgenerallyhappen,asitrequires
developmentatafasterratethan
normal.Mostdelayedchildren
makesteadyprogressataslower
ratethanotherchildrenofthe
sameage,andmakepredictable
gainsinlearning,butnever‘catch-
up’.
ThecaseforchildrenwithLKSis
different.Thesechildrengenerally
hadnormalearlydevelopment,
andwereincreasingtheirskills
atthenormalrate.Followinga
periodofregression,theymay
wellappearto‘catch-up’and
learnatanincreasedrate,often
inresponsetosteroids.Whatis
actuallyhappening,however,
isrecoveryoftheirprevious
developmentalpath.
Unfortunatelythisisnotalways
thecaseinLKS,andattheendof
theactivephaseofthedisease,
childrenoradolescentsareoften
leftwithresidualimpairments.
Theymaythenmakesteady
developmentalprogressbutnever
regaintheirpreviousrateof
learning.Howeverthereissome
evidenceofcontinuingrecovery
ofskillsintotheir20’s,hencethey
shouldhavepriorityforcontinuing
furthereducation.
Regressionisthelossofpreviously
acquiredskills,sothechildappears
tohavereturnedtoanearlier
stageintheirdevelopment.Itcan
beuneven,andleavethechild
withretainedisolatedskillsfrom
theirpreviousdevelopmentallevel,
whichcanmasktheirlosses.
Epilepsy
Thisisaconditionwhereaperson
hasaseriesofseizures.
��
Seizures
Thesehappenwhenpartofthe
braindevelopsuncontrolled
electricalactivityordischarges,
whichstopsthenormalfunction
ofthatpartofthebrainand
producesthefeaturesthatoccur
intheclinicalseizure.TheEEG
recordingswillpickupdischarges
andabnormalitiesoverthearea
ofbrainaffected,orevenoverthe
wholebrainiftheseizurebecomes
generalised.
Inclinicalseizuresthereisan
obviouschangethatoccursforthe
person,duringtheseizure.This
changejustdependsonwhatpart
ofthebrainishavingtheseizure
andthepersonmaytwitchand
jerk,orgoblankforafewseconds
orevenexperienceastrangetaste
orsmell.
Insubclinicalseizureactivity
thereisnoobviouschangesuch
asjerking,eventhoughthe
EEGrecordselectricalseizures.
Thisdoesnotmeantheseizures
arenothavinganeffectonthe
person,butthiseffectmaybeon
acquiredskillssuchaslanguage,
socialcommunicationorabstract
thought.InLKS,themainseizures
aresubclinicalandoccurduring
sleep.
Todd’s paresis
Thisreferstotemporaryweakness
thatsometimesfollowsaseizure.
Convulsive status epilepticus
Thisiswhereaseizurethatcauses
convulsions,(whenthemuscles
ofthebodymoveoutofcontrol),
continuesforalongtime(e.g.
morethanthirtyminutes),or
whenoneseizurefollowsanother
withoutthepersonregaining
consciousnessin-between.Itis
dangerousandneedsurgent
treatmentifaseizurelasts4-5
minutestotrytopreventalarger
attack.
Non-convulsive status
Thisalsooccurswhenseizuresare
veryprolonged,orfollowoneupon
anotherwithoutbreak.Howeverin
��
thiscase,theseizuresdonotcause
convulsionsbuttypicallycause
fluctuationsinawarenessandjerks.
Epilepsy with electrical status epilepticus during sleep (ESES)
Thisisaspecialtypeofnon-
convulsivestatusinwhich
continuousdischargesoccupymost
ofsleep.Itisparticularlyassociated
withtheactivephaseofLKSand
isassociatedwithintellectual
deteriorationandlossof
language.Itmayalsobereferred
toascontinuousspikeandwave
dischargesduringsleep(CSWS).
Thiselectricalactivitycanpersist
formonthsorevenyears.
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Notes
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©GOSHTrustFebruary2005Ref:2004F296CompiledbytheNeurodisabilityTeamincollaborationwiththeChildandFamilyInformationGroup.
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