Paediatric Epilepsy

7/30/2019 Paediatric Epilepsy

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PAEDIATRIC EPILEPSY

Dr.AlvinYeeShing CHANMBBS(HK),MRCP(UK),DCH(GLASG),FHKCPaed,FHKAM(Paediatrics),MRCPCH,FRCP(Edin)

VicePresident,TheHongKongMedicalAssociation

CoChairman,HealthEducationCommittee

CentralCoordinator,HKMACommunityNetwork

CoChairman,InternationalAffairsCommittee


2/92

CAUSES Astructurallyabnormalareainthebrain

Asevere

metabolic

disturbance,

hypoxemia,

and

somedrugs

Fever

Geneticallyinherited

Channelopathy

Adefect

in

one

of

the

sodium,

potassium,

or

the

calciumchannelsortheionpumpsinthecell

membranethatmaycausecerebralneuronsto

discharge

abnormally 2


3/92

CRYPTOGENICEPILEPSY

Noevidencethatapatient'sseizureswere

causedby

amedical

disorder,

astructural

abnormalityinthebrain,oraninherited

disorder

Corticaldysplasia

Subtlestructuralabnormality

3


4/92

CONVULSIONvs SEIZURE

vs EPILEPSY

Convulsions seizures epilepsy

(motor) (cerebral

arrhythmia) (tendency

of

spontaneous

recurrentseizures)

SEIZURES Corticalneuronial discharge:

Involuntaryparoxysmal

Briefdisturbanceofbehaviour,emotion,

motororsensoryfunctionwithabruptonset,

dropin

awareness 4


5/92

EPIDEMIOLOGYOF

EPILEPSY

5%ofpopulationhasaseizureby20

yearsof

age

Incidence:2050per100,000/year

Prevalence:0.5

1%

of

population

5


6/92

Standardizedmortalityrateisincreased

23timescomparedtogeneralpopulationdueto:

Statusepilepticus

Accidentalinjury

Suicide

Suddenunexplaineddeath

6


7/92

CLASSIFICATIONOF

EPILEPSY

1. SymptomaticEpilepsy

Tumor

Congenitalmalformation

Infarction

Intracranialinfection

Hemorrhage

Traumaticbraininjury

2. Idiopathic

Epilepsy Geneticallyinheritedformsofepilepsy

Inallcasesofgeneticepilepsy,bydefinition,nostructural

abnormality

exists

in

the

brain

that

would

account

for

seizures. 7


8/92

EPILEPSY

vs

REACTIVESEIZURES

Requiredcorrectionof

underlyingcausesand

longterm

AED

often

not

necessarye.g.hypoxia,

fever,

hypoglycemia,

hyponatremia,drugs,drug

withdrawal,

encephalopathy 8


9/92

DDx OFSEIZURES

A.Systemicdisturbances:syncope,breath

holding,metabolic

disorders

B.Neurologicaldisturbances:e.g.

movementdisorders,

sleep

disorders

C.Psychogenicdisturbances:e.g.psychotic

hallucination,panic

attacks,

psychogenic

seizures

9


10/92

TYPESOF

SEIZURES

Thedifferencebetweenpartial(focal)

seizuresand

primary

generalized

seizures

Severalmedications

indicated

for

partialseizuresdonotcontrolormay

evenworsen

primary

generalized

seizures

10


11/92

PARTIALSEIZURES

Partialseizuresoriginatefromonlyonecerebralhemisphere.

Theyaresometimescausedbyastructuralabnormality

Apartial

seizure

usually

causes

signs

and

symptoms

reflecting

thefunctionoftheneuroanatomical regionfromwhichthe

seizurearises.

Signs

and

symptoms

may

include:1. arrestofspeech(seizureoriginatinginthelanguagecortex);

2. stereotypicmovementsofapartofthebody(motorareaseizure);

3. astereotypedthought,emotion,orbehaviour (prefrontalcortex

seizure);

4. theperceptionofoddnoises(auditorycortexseizure);

5. unilateralparesthesias ornumbness(sensorycortexseizure);

6. oradisturbance

of

vision

(visual

cortex

seizure).

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12/92

Partialmotorseizure

Todd'sparalysis:transientparalysis

oftheaffectedlimb

12


13/92

Partialseizuresthatdonotimpairthe

patient'sresponsiveness

during

the

seizureorthepatient'smemoryofthe

seizureare

called

simple

partial

seizures

Partialseizuresthatinterferewiththe

patient's

responsiveness

and

ability

to

recalltheeventsoftheseizurearecalled

complexpartialseizures.

13


14/92

COMPLEXPARTIALSEIZURES Temporal

lobe

seizures

Mayalsooriginatefromthefrontallobe

Precededbyaprodromal stage(aura)

Typicalsymptoms

during

the

aura

~ Unpleasantolfactorysensations

~ Arisingsensationintheabdomenasifcomingdowna

rollercoaster

~ Astereotypicthought

~ A"dejavu"spellorafeelingofimpendingdoomordread

Inall

such

cases,

the

aura

is

the

only

part

of

the

seizure

that

canlaterberecalledbythepatient

Astateofdiminishedresponsivenesslastingforseveral

minutes

or

longer

typically

follows,

during

which

time

the

patientdoesnotrespondandappearstobeinatrance. 14


15/92

Associatedbehavioursmayincludestaring,forced

eyedeviation

or

head

turning

to

one

side,

nystagmus,

blinking,lipsmacking,andsemipurposeful

movementsofthehands.

Automatisms Followingacomplexpartialseizure,oftenastateof

sleepinessorgrogginesslastingseveralminutesto

hours.This

postictal

state

is

an

important

clinical

sign

usefulindifferentiatingcomplexpartialseizuresfrom

absenceseizuresandalsofromsyncopalepisodes

andother

nonepileptic

events. 15


16/92

SECONDARYGENERALIZATION

Partialseizuresthatspreadwithinonecerebral

hemisphereandthen,viathecorpuscallosum,spreadto

theopposite

hemisphere

Generalizedtonicclonic("grandmal")seizureisoften

observed,manifestingascompletelossofconsciousness

andwhole

body

stiffening,

followed

by

rhythmic

jerking

ofthetorsoandextremities,clenchingofthejaw,eye

rolling,tonguebiting,cyanosis,andlossofbowelor

bladdercontrol

Usuallylasts1to2minutes;thepostictalstatethat

followsisoftenprolonged,sometimeslastingforhours

orevendays.Thepatientcannotrecallanyofthis,other

thanthe

aura. 16


17/92

PRIMARYGENERALIZEDSEIZURES

~ Absenceseizures

~ Myoclonic seizures

~ Tonicseizures

~ Clonic seizures~ Tonicclonic seizures

~ Atonic seizures

The

thalamus

or

brainstem

is

the

source

of

these

seizures,

althoughlesionsintheseareasdonotcauseseizures.A

possiblecauseisa"channelopathy".Theseseizuresare

presumedtooccurastheresultofageneticallyinherited

trait. 17


18/92

Differentiatingprimarygeneralizedseizures

fromsecondarily

generalized

seizures

Primarygeneralizedtonicclonic seizure Secondarilygeneralizedtonicclonic seizure

1. Noauraprecedingtheevent 1. Historyofanaurathatprecededthe

seizure

2. Nodeviationoftheeyestooneside 2. Eyedeviationtoonesideduringorafter

theseizure

3. Involvementof

both

sides

of

the

bodyfromthemomentofonset3. Tonic

or

clonic movements

mostly

of

one

sideofthebodyduringtheseizure

4. NoTodd'sparalysisaftertheseizure 4. Todd'sparalysisaftertheseizure

5. Ageneralized

spike

wave

electroencephalogram(EEG)tracing5. EEG

showing

epileptiform discharges

from

onlyonecerebralhemisphere

6. Afamilyhistoryofepilepsy 6. Afamilyhistoryofepilepsyislessoften

reported.18


19/92

PHYSICAL

EXAMINATION Theskin dysmorphicfeatures

Neurocutaneousdisorder

Cranialbruit

Oddbehavior

may

suggest

autism

Focalfindings

Signs

of

raised

intracranial

pressure

(papilledema),especiallyinthecaseofanew

onset,partialorpoorlycharacterizedseizure

19


20/92

Diagnostictestsforpatientsseenaftera

firstnonfebrile

seizure:

MRIBrain

Mesial temporalsclerosisreferstoscarringoratrophy

withinthe

hippocampus

that

often

causes

complex

partialseizures

Agenesisofthecorpuscallosum isassociatedwith

difficultto

control

seizures,

developmental

delay,

and

otherstructuralabnormalitiesofthecentralnervous

system

Lissencephaly Hemimegalencephaly

Polymicrogyria

Schizencephaly 20


21/92

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23


24/92

EEG

(electroencephalogram)

(I)1. TheprimarypurposeoftheEEGistodetectepileptiform

dischargessuggestingthatpartofapatient'sbrainis

electrophysiologicallyabnormalandthataseizuremayoccur

asaresult.ApersondoesNOThavetohaveaseizureduring

theEEGrecordingfortheEEGtorevealepileptiformactivity

2. Asingle,

30

minute

EEG

demonstrates

epileptiform

activity

in

about50%ofallpatientswithahistoryofepilepsy.The

sensitivityoftheEEGvariesmarkedlyfrompatienttopatient.

TheEEGofsomepatientswithepilepsyisalwaysabnormal,

whiletheEEGofotherpatientswithepilepsyisneverabnormal.Inthelattergroupofpatients,epileptiform

dischargesarethoughttooriginatefromanareaofthebrain

toodistant

from

the

recording

electrodes

to

cause

arecordableabnormality

24


25/92

CLINICAL

USE

OF

EEG

(II)EEGrecordstheelectricalactivityofthebrainatscalpsurface

1. An

interictal EEG

shows

epileptic

form

discharges

in

50%

of

epilepticpatients

2. 20%ofpatientsdonotshowEEGabnormalfindings

3. SomenormalindividualsmayhaveusualEEGdischarges

4. RoutineEEGcan'tproveordisproveepilepsy

5. EEGabnomality canhelpindiagnosisandclassificationof

theseizuretype

6. Anictal EEG

with

video

recording

can

usually

help

in

diagnosisandclassificationinmostcases,particularlyuseful

indiagnosingpsychogenicseizuresandpaediatric seizures

25


26/92

Myclonic

3%

Others

8%

Unclassified

3%

Simple14%

Complex

36%

Unknown

7%

Generalizedtonicclonic

23%

Absence

6%

Generalized

(43%)

Partial*

(57%)*includessecondary

generalisation

26


27/92

CLINICAL

USE

OF

EEG

(III)7. TheEEGmayormaynotnormalizefollowinginitiationof

antiepilepticdrug(AED)therapy.Inmostcases,ifthe

patient'sseizuresarecontrolledbytheAED,itdoesnotmatterthattheEEGremainsabnormal.Thetreatmentof

certainconditions,suchasinfantilespasms,isexpectedto

normalizethe

EEG

8. TheEEGisveryhelpfulindistinguishingprimarygeneralized

seizuresfromsecondarilygeneralizedseizures

9. The

diagnosis

of

some

forms

of

epilepsy

requires

specific

EEG

findings.TheseconditionsincludebenignRolandicepilepsy,

absenceepilepsy,juvenilemyoclonicepilepsy,infantile

spasms,andLennoxGastautsyndrome

27


28/92

CLINICAL

USE

OF

EEG

(IV)10. CommonlyreportedEEGabnormalitiesinclude:anabnormally

slowbackgroundrhythm,duetoencephalopathyofalmostany

cause(e.g.,

concussion,

overmedication,

sepsis,

postictal

state,

coma,etc.);focalslowwavesfromonehemisphereduetoa

structurallesion(e.g.,tumor,infarct,etc);focalsharpwavesor

spike

discharges

from

one

hemisphere,

typical

in

cases

of

partialseizureandgeneralizedspikewaveorgeneralized

polyspikedischarges,whichareseenincasesofprimary

generalizedepilepsy,includingabsenceepilepsyandjuvenile

myoclonicepilepsy.

11. Hypsarrhythmiaisassociatedwithinfantilespasms.Thisterm

referstoagrosslydisorganized,highamplitudeEEGcomprised

ofunsynchronizeddeltawavesandspikedischarges.

28


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34/92

Whentotreatapatientwhohas

hadaseizure

Manypediatricneurologistsdonotinitiatetreatmentwithan

AEDifonlyonenonfebrileseizurehasoccurred,sinceatleast

50%of

these

patients

never

experience

arecurrence.

MRIstructuralabnormality

EEGepileptiformdischarges

Historyof

neurodevelopmental

delay

Recurrencegenerallyhighifdiagnosedwithidiopathic

(geneticallyinherited)epilepsycharacterizedbyrecurrent

seizures. Ifthepatienthashadtwoormoreunprovokedseizures,the

riskofrecurrenceisgreaterthan70%,andmostneurologists

willinitiatetreatment.34


35/92

However,manyparentsdonotwanttheirchildtreatedwithanAED

eveniftherehavebeentwoormoreseizures.

c Thedanger

of

avery

prolonged

seizure

(status

epilepticus)

that

candamagethebrainorevencausedeath;formostpatients

withepilepsy,thechanceofthisoccurringissmall,although

neverzero

d The"kindling"model,basedonanimalstudies,impliesthat

ongoingepileptiform activityinthebrainmakesfutureseizures

morelikelytooccurandalsoprovidessomejustificationsfor

treating

a

patient

with

a

history

of

multiple

seizurese Evidencethatmultiplecomplexpartialseizuresmightcause

progressivememoryimpairment

f Thesocialimplications,particularlyforanolderchild,alsoneed

tobe

considered 35


36/92

ANTIEPILEPTIC

DRUGS

TheAEDstraditionallyprescribedto

patientswith

partial

epilepsy

are

phenytoin,earbamazepine,and

phenobarbital

AEDsmorerecentlyapproved

includevalproate,

gabapentin,

levetiracetam,topiramate,

zonisamide,and

oxcarbazepine 36


37/92

PatientstakingoneofthenewerAEDs(gabapentin,

levetiracetam,

topiramate,

zonisamide,

and

oxcarbazepine)donotrequireroutinebloodwork

(completebloodcountsandliverfunction

monitoring).Levetiracetamandgabapentindonot

undergosignificanthepaticmetabolismanddonot

bindtoplasmaproteins,makingthesetwoAEDs

preferableforpatientswithahistoryofaliver

diseaseorwhoaretakingadrugthatisplasmaproteinbound.

37


38/92

Drug Indication Dose Importantadverse

reactions

Phenytoin (Dilantin,

ParkeDavis)PS,

SGS Maintenance:

5to10mg/kg

dividedbid.

Loading forstatus

epilepticus:

15to

20

mg/kg

intravenously(not

intramuscular,as

routecancause

tissuenecrosis).

AR,CBS,

gum

hyperplasia,course

facialfeatures,HLA.

Zeroorder kinetics

athigherdoses.

Cardiacarrhythmia

whengiven

intravenously.

Fosphenytoin

(Injectiononly)

(Cerebyx,Eisai)

Statusepilepticus Asabove;mayalso

beadministeredby

intramuscular

injection.

Generallywell

tolerated;fewer

adversereactions

thanphenytoin.

38


39/92


reactions

Phenobarbital PS,SGS Maintenance:

3to

6

mg/kgdivided

dailybid.

Loadingforstatus

epilepticus:15 to

20mg/kg

intravenously.

AR,CBS

(especially

inyoungpatients),

HLA.

AVOIDGIVING

WITHVALPROATE.

Carbamazepine

(Tegretol,Novartis;

Carbatrol,

Shire)

PS,SGS Maintenance:10to

15mg/kgdivided

bid

or

tid.

Adolescents:initial

dose, 200mgbid;

increaseasclinically

indicated.

AR,HLA,SIADH

(usuallymild),CBS,

aplastic anemia

(R).

39


40/92


reactions

Oxcarbazepine(Trileptal,Novartis)

PS,SGS 150

to

300

mg

bid;

mayincrease to

maximumof1800

mgdaily.

Loweredplasma

sodium, CBS.

Valproate

compounds(divalproex sodium,

valproic acid)

(Depakate,

Depakene,

Depakone;Abbott)

PS,SGS,PGS Maintenance:15to

60mg/kgdividedbidortid.

Adolescents:initial

dose,250 mgbid;

increase

as

clinically

indicated.

Depakone canbe

loaded

intravenously.

AR,HLA,CBS,

weightgain,tremor,

hairloss,hepatic

failure(R),

pancreatitis (R).

USUALLY

NOT

GIVENTOPATIENTS

UNDER2YEARSOF

AGE.

40


41/92


reactions

Gabapentin(Neurontin,Parke

Davis)

PS, SGS 100 to300

mg

bid

ortid;mayincrease

tomaximumof900

mgtid.

CBS

Levetirecetam

(Keppra, UCB)

PS,SGS,PGS 250mgbid;may

increasetomaximumof1500

mgbid.

CBS,behavioural

disturbances.

Zonisamide

(Zonigran,Elan)

PGS,PS, SGS 100to400 mgonce

daily.

CBS, hematological

abnormalities,renal

stones,

oligohidrosis (R).

41


42/92


reactions

Topiramate(Topamax, Ortho

McNeil)

PS, SGS,PGS Initial dose,

15

to

25

mgdaily;gradually

increaseto50to100

mgbid.

CBS (canbe

marked),

appetiteloss,renal

stones(R),glaucoma

(R),oligohidrosis (R).

Lamotrigine

(Lamictal,

GlaxoSmithKline)

PS, SGS,PGS 0.6to1.2mg/kg

dividedbid;

increase

graduallyto

maximumof5to15

mg/kgdividedbid.

IFUSEDWITH

VALPROICACID,

CONSULT

MANUFACTURER'S

RECOMMENDED

DOSAGE

AR,StevensJohnson

syndrome

(uncommon),CBS.

42


43/92


reactions

Ethosuximide(Zarontin,Parke

Davis)

Absenceseizures

(only)250

mg

tid;

maximum dose,

500mgtid.

AR,stomach

pain,

HLA,luptuslike

reaction(R).

Benzodiazepines

(diazepam,

clonazepam,

Iorazepam,

midazolam)

Status epilepticus;

occasionallya

maintenanceAED;

rectaldiazepam

usedforfebrile

seizures

SeePhysician's

DeskReferencefor

dosing.

Highlikelihoodof

developing

medication

tolerance,

somepolence,

respiratory

depression

(R);

reboundseizures

whendrugtapered

off.

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Phenytoin,phenobarbital,andgabapentin

generallyare

not

effective

for

the

long

term

controlofprimarygeneralizedseizures.

Carbamazepineandoxcarbazepinemaycause

anincrease

in

the

frequency

of

primary

generalizedseizure,especiallyabsenceand

myoclonicseizure,andshouldneverbe

prescribedto

patients

with

primary

generalizedepilepsy.

44

TITRATION MAINTENANCE AND


45/92

TITRATION,MAINTENANCE,AND

DURATIONOF

TREATMENT

Tominimizeadverseeffects,treatmentwithan

AEDis

usually

initiated

at

about

one

quarter

to

onehalfofthenormalmaintenance dose,and

thedoseisthengraduallyincreased

Ifseizures

still

occur

after

the

target

dose

has

beenattained,itisreasonabletofurtherincrease

thedoseaslongasthepatientdoesnotcomplain

ofadverse

effects.

If

good

control

without

significantadverseeffectscannotbeachieved,

theAEDshouldbequicklytaperedoffwhile

anotherAED

is

introduced. 45


46/92

WhenapatientwhoistakinganAEDpresentswith

lethargy,

vomiting,

or

ataxia,

AED

toxicity

should

be

suspected.Ifthepatient'ssymptomsaresevere,the

patientmayneedtobesenttotheemergencyroom

orhospitalized.Liverfunctiontestsandaplasma

ammonialevelshouldbeorderedifthepatientis

takinganAED(especiallyvalproate)thatundergoes

hepaticmetabolism.TheAEDlevelshouldbe

measured.The

drug

should

be

withheld

until

the

patientbecomesasymptomatic.Oncethesymptoms

oftoxicityabate,theAEDmayberestartedatalower

dosethan

the

patient

originally

took. 46


47/92

MostAEDshavebeenfoundtocausecongenitalmalformations.

Fetalhydantoinsyndromeisacraniofacialanddigital

malformationresulting

from

prenatal

exposure

to

phenytoin.

Valproatecompoundsincreasetheriskoffetalneuraltube

defects.

Serious

injury

to

the

fetus

can

result

if

the

mother

has

a

generalizedtonicclonicseizureduringpregnancy.Ifawoman

withgeneralizedtonicclonicseizureswishestobecome

pregnant,herAEDshouldnotbediscontinued.Valproatebased

drugsshould

be

avoided.

Myoclonic,absence,andnongeneralizedpartialseizuresarenot

likelytobeinjurioustothefetus,andawomanwithoneof

theseseizuresmayelecttodiscontinueherAEDbefore

becomingpregnant. 47


48/92

HEPATICANDRENALINSUFFICIENCY

TheplasmalevelofAEDsthataremetabolized

bythe

liver

will

tend

to

be

higher

if

the

patient

hasahistoryofhepaticinsufficiency,andthe

AAEDlevelshouldbecarefullymonitored.The

ammonialevel

should

also

be

checked.

The

dosesofAEDsthatareprimarilymetabolized

bythekidney(e.g.,leveltiracetam,

gabapentin)may

require

adjustment

in

cases

ofrenalinsufficiency.

48


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COGNITIVEIMPAIRMENT

Themostcommonadverseeffectreportedby

patientstaking

an

AED

is

mild

cognitive

impairment,includingdrowsiness,mental

fatigue,lightheadedness,anddifficulty

concentrating.Treatment

with

an

AED

should

bestartedatalowdosetohelpminimize

thesesymptoms.Manypatientsreportthat

theirsymptoms

become

less

noticeable

after

afewweeks,butinsomecases,thedrugmust

be

discontinued

and

another

AED

substituted.49


50/92

ALLERGICRASH

Phenytoin,carbamazepine,lamotrigine,

phenobarbital,and

occasionally

other

AEDsmaycauseanallergicrash,whichis

often

accompanied

by

fever.

The

AED

mustbeimmediatelydiscontinuedto

avoidprogressiontoStevensJohnson

syndrome.

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HEMATOLOGICALABNORMALITIES

Patientstakingcarbamazepineorvalproateoftenare

foundtohavemildhematologicalabnormalitiessuchas

anemia,neutropenia,

or

thrombocytopenia.

Adjustment

oftheAEDdoseisnotusuallynecessarysincethereare

noclinicalmanifestations.

Aplasticanemia

is

an

extremely

rare

but

life

threatening

complicationoftreatmentwithseveralAEDs,in

particularcarbamazepineandthecompletebloodcell

countis

usually

monitored

every

3to

6months

when

apatientistakingcarbamazepine.Felbamate(Felbatol,

Wallace),anAEDforLennoxGastautsyndrome,causes

aplastic

anemia

in

a

larger

percentage

of

cases. 51


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HEPATICEFFECTS

ValproatebasedAEDscancauseliverfailurewithanaberrant

metaboliteofvalproatethatactsasamitochondrialpoison.The

incidenceof

liver

failure

has

been

estimated

at

1per

600

in

children

undertheageof2yearswhoaretakingvalproate,buttheincidenceis

only1per100,000amongadults.Valproateisprescribedtopatients

under2yearsofageonlytotreatinfantilespasmsorLennoxGastaut

syndromeand

only

when

other

AEDs

have

not

been

effective.

Levocarnitine(Carnitor,SigmaTau),50to100mgperday,is

sometimesprescribedwithvalproatetohelppreventhepatotoxicity.

Pancreatitisandpolycysticovariansyndromealsooccasionallyresult

fromtreatment

with

valproate.

Monitorliverfunctiontests6monthlyiftakingvalproate.Mild

elevationsofthehepatictransaminasesarecommonbutdonotsignify

hepaticfailure.Sometimesmildhyperammonemia,causing

somnolence,also

is

noted. 52


53/92

Valproatemaycauseweightgain,hairloss,

tremor,and

mood

swings.

Multivitaminswithzincandseleniumoften

helppreventhairlossandtremorsfrom

valproate.

Phenytoin,carbamazepine,andphenobarbital)

occasionallycause

allergic

hepatitis.

The

AED

isusuallydiscontinued,andanotherAED,

preferablyadrugthatdoesnotunderhepatic

metabolism,is

substituted. 53


54/92

Patientswhotakephenytoin(Dilantin,ParkeDavis)often

developgingivalhyperplasiaandcoarsefacialfeatures.

Phenobarbital,the

AED

most

often

prescribed

to

neonatesandinfants,oftencausesirritability.The

infant'smoodusuallyimproveswithinafewweeksof

startingtreatment

but

may

temporarily

deteriorate

when

thedrugistaperedoff.

Benzodiazepines(lorazepam,diazepam,andclonazepam)

rapidly

induce

tolerance,

and

seizures

may

recur

soon.

If

thedoseisthenincreased,somnolenceandeven

suppressioncentralrespiratorydrivemayresult;ifthe

doseisthenreduced,theseizuresagainbecome

frequent. 54


55/92

Gabapentinalmostnevercausesseriousadverseeffects

butoftencausesmildneurocognitiveimpairment

(lightheadedness,fatigue,

and

difficulty

concentrating).

Topiramatealsomaycausecognitiveslowing

Topiramateandzonisamidemaycausethepatientto

developkidneystonesandmaycauseappetitesuppressionand,rarely,oligohidrosis(decreased

sweating)resultinginheatintolerance.

Monitoredduring

warm

weather

Levetiracetammaycauseachangeinmentalstatus(e.g.,

agitation,confusion).55


56/92

Patientswithepilepsyshouldnotswimunless

theyhave

infrequent

seizures,

they

wear

alife

vest,andanadultwhoisagoodswimmeris

presenttowatchthem

Ahelmet

should

be

worn

when

riding

a

bicycle.Thepatientwithepilepsyshouldnot

operateheavymachineryorworkwithhigh

voltageelectricalequipment.

Teamandindividualsportsarepermitted

56


57/92

Frequentlyprescribedmedicationsthatmaycausea

nonepilepticpatienttohaveaseizureorprovokea

seizurein

an

epileptic

patient

include

antipsychotic

drugs,

tricyclicantidepressants,buproprion.

Methylphenidatehas,atmost,aweakepileptogenic

effect.

Tricyclicantidepressantsandbuproprionshouldbe

avoided,butpatientswithepilepsyandADHDoftentake

astimulant

like

methylphenidate,

and

patients

with

epilepsyandapsychiatricdisorderoftenneed

antipsychoticdrugs(inbothcases,withanAED)usually

withno

exacerbation

of

seizures. 57


58/92

AsingleAEDpreventsseizuresinmorethan70%of

patientswithepilepsy.Theremaining30%have

seizuresmoredifficulttocontrol.

TheAEDwithbestcontrolofseizuresandfewest

adverse

effects

is

continued. Addingseconddrugresultsingoodcontrolforabout

50%ofpatientswhoseseizureswerenotcontrolled

by

monotherapy. Whenmorethantwodrugsarerequired,ketogenic

dietorepilepsysurgeryshouldbeconsidered.

58

BENIGN ROLANDIC EPILEPSY


59/92

BENIGNROLANDIC EPILEPSY

Themostcommonseizuredisorderofchildhood.

Onset:4to12yearsofage.

Partial

seizures

during

sleep

&

awakening

inability

to

speak,

andclonicmovementsofonearmorsideoftheface.

Thenocturnalseizuresoftensecondarilygeneralize;rarely

duringthedayaswell.Theremaybefamilyhistoryand

asymptomaticrelatives

may

have

EEG

abnormalities.

Centrotemporalspikes

Themainissueconcernstreatment.

Sincethe

seizures

are

eventually

outgrown,

some

physicians

donotinitiatetreatment.

Iftheseizuresarefrequentoroccurduringthedaytime,an

AEDis

more

often

prescribed. 59

BENIGN OCCIPITAL EPILEPSY


60/92

BENIGNOCCIPITALEPILEPSY

Causesvisualsymptoms,suchasflashing

lightsorlossofvision,lossof

consciousness;and

sometimes

tonic

clonicmovementsandisdiagnosedon

thebasis

of

localized

occipital

lobe

EEG

discharges

Occasionally

persist

into

adult

life ControlwithanAEDforpartialseizures

60

PANAYIOTOPOULOS SYNDROME


61/92

PANAYIOTOPOULOSSYNDROME

EarlyonsetbenignChildhoodoccipital

epilepsy:autonomic

epilepsy:

vomiting

andotherautonomicsymptoms

Control

with

an

AED

for

partial

seizures

61

SYMPTOMATIC PARTIAL SEIZURES


62/92

SYMPTOMATICPARTIALSEIZURES

1. Neurocysticercosis (moreinadults)

Thepork

tapeworm,

aparasitic

nematode

Unexplainedseizures

2. Intracranialworm

infestation:

larvae

of

diphilobothrium mansoni

Ingestedwithfrogorsnakes

3. Congenitalcerebralmalformations

62


63/92

63


64/92

64


65/92

65

COMMONPRIMARYGENERALIZED


66/92

EPILEPSYSYNDROMES

Absenceepilepsy

Middle

childhood Occurmanytimesaday.Theseizurestake

theformofbrief(5to30seconds)staring

spells.Blinking,

head

nodding,

and

semi

purposefulhandmovements(automatisms)maybenotedduringtheseizures.Incontrast

to

complex

partial

seizures,

which

are

somewhatsimilarinappearance,apostictalstateisneverdescribed

afamily

history 66


67/92

Physicalexaminationusuallyrevealsnoabnormalities.Hyperventilationfor3minutes

oftenprovokes

an

absence

seizure.

The

EEG

includesintermittentgeneralized3Hzspikewavedischarges

Childhoodabsence

seizures

usually

stop

by

the

endofadolescence.Absenceseizuresforaminuteorlongerandareassociatedwith

prominentautomatisms

(atypical

absence

seizures)andabsenceseizuresfirstnotedduringadolescence(juvenileabsenceseizures)typically

continuethroughout

adult

life. 67


68/92

Amyoclonicjerkisalightningfastmovement,usuallyoftheupperextremities.

Juvenilemyoclonic

epilepsy

(JME)

during

adolescence.

Myoclonicjerksofthearmsareoftenthefirstsign.Myoclonusofthelowerextremities,resultinginfalls.

Oftenhave

generalized

tonic

clonic

seizures

as

well

as

myoclonicseizures.

Physicalexamination:noabnormalfindings

EEG:

4

to

6

Hz

generalized

spike

wave

or

polyspike

discharges.

Byvalproate,Topirimate,LamotrigineorZonisamide

LifelongtreatmentwithanAED68


69/92

Infantilespasms

Usually,during

the

first

year

Multipleepisodesofrepetitiveheadtrunk,orbilateralupperextremityflexion,

or

hyperextension

Abriefcry,staringoreyerolling,ora

transientloss

of

consciousness

often

occur

69


70/92

EEG

Hypsarrhythmia:adisorganizedbackgroundwithcontinuoushighamplitudeslowwavesandmultifocalspikeactivity

Cryptogenicinfantilespasms

Symptomaticinfantilespasms

Halfhaveanunderlyingconditionaffectingbrain

development.E.g:

prenatal

cerebral

infarction,

cerebral

malformation,tuberoussclerosis,chromosoma anomaly,metabolicdisorder

Prognosisbetterwithcryptogenicinfantilespasms,butboth

typesmay

lead

to

other

forms

of

epilepsy

and

developmental

delays.Westsyndrome:triadofinfantilespasms,developmentaldelay,andahypsarrhythmic EEG

MRI,karyotype,genetictesting

Metabolicstudies 70


71/92

Standardtreatment

Aseriesofintramuscularinjectionsofadrenocorticotropichormone,orACTH(ActharGel,Questcor)

Controllingthe

seizures

Developmentalprognosis

PromptlytreatingcryptogenicinfantilespasmswithACTH,resultsinanimprovedprognosis

ACTHinjectionsarestartedinhospital

Theinitialdosehasneverbeenstandardized;20to80IUdailyistheusualrange

Baram

et

al.

suggest

that

a

dose

of

150

IU/m2

(body

surface

area) Ifthereisnoimprovement,withinafewdays,thedosemaybe

doubled.Ifthereisstillnoimprovement,thedrugshouldberapidly

taperedoff.

71


72/92

Adverseeffectsfromlongtermsteroidtherapy:cushingoid

features,hypertension,cardiomyopathy,gastriculcers,diabetes

mellitus,and

infection.

Monitorbloodpressure,plasmaandurineglucose,electrolytes,

stooloccultbloodandinfectionsshouldbetreated.

Infantilespasmscausedbyanunderlyingconditionwithpoor

prognosis,such

as

asevere

brain

injury

or

malformation:

ACTH

may

notbejustified

Benzodiazepines

Topirimate

Valproate

Vigabatrin:visualdisturbancesandcerebralwhitematterchangeswerereportedduringclinicaltrialsofvigabatrininthiscountryandprecludedFDAapproval

72

LENNOXGASTAUT SYNDROME (LGS)


73/92

LENNOX GASTAUT SYNDROME(LGS)

Oneofthemostsevereandrefractoryformsofchildhoodepilepsy

TomeetcriteriaforLGS,apatientmusthave(a)seizuresofmultipletypes(includingmyoclonic,absence,tonicclonic,and/oratonicseizures);(b)ahistory

of

development

delay

or

mental

retar

dation;

and

(c)

an

EEG

showingageneralizedslow(1.5to2Hz)spikewavepattern

Manypatientshaveahistoryofinfantilespasms

Neurocutaneousdisorders(especiallytuberoussclerosis)orinheritedand

metabolicdefects

affecting

neurological

development

Difficulttocontrol,andtreatmentwithatleasttwoAEDsisalmostalwaysrequired

Levitiracetam/benzodiozepine

Valproate

Felbamate

Aplasticanemiaandhepatotoxicity

Everilimus/sirolimuscouldbetriedforpatientswithtubersclerosis

73


74/92

Febrileseizures 5%ofchildren

Theageofonsetisusuallybetween6monthsand6yearsofage.

Increasedchanceofepilepsywithnonfebrile seizures:

Familymemberswithepilepsy

complexfebrileseizure(e.g:duration>20minutes,unilateral

motor

activity

or

lateral

eye

deviation

during

the

seizure,

or

postictal Toddsparalysis)

delayeddevelopment

lateonsetoffirstfebrileseizuresolderthan3years

Asingle,

brief,

uncomplicated

febrile

seizure

does

not

require

CT,

MRI,

orEEG.Recurrentorcomplexfebrileseizures,ahistoryofabnormalneurologicaldevelopment,andafamilyhistoryofepilepsy:toconsiderfurthertesting

Brainimagingusuallynormalevenincomplexfebrileseizures74


75/92

Withafirstfebrileseizure,thechildinemergencyroomstillfebrile,meningitisisoften

considered.

A

lumbar

puncture

(LP)

is

appropriateifthechilddoesnotrecoverquicklyfromtheseizureorwasnotedtobeunusually

irritable

or

lethargic

before

the

seizure.

For

additional

febrile

seizures,

an

LP

should

be

deferredunlessthereisastrongclinicalsuspicionofmeningitis

75


76/92

Adiazepamrectalsuppository2.5to10mg,canbegiventoabortafebrileseizure

Givento

the

family

of

the

child

with

ahistory

of

febrileseizures.

Timelyadministrationmaypreventemergencyvisit

Paracetamolor

ibuprofen

every

4to

6hours

Epilim,PhenobarbitaloranotherAED:preventivetreatmentforfebrileseizuresdoesnotdecreasethe

likelihoodto

develop

epilepsy

Majoritystopoccurringwellbefore6yearsofage,andinmostcases,before3yearsofage

76

OTHERFREQUENTLYSEEN


77/92

SEIZURESIN

CHILDHOOD

Viralgastroenteritis

A

vaccination

may

be

followed

by

fever

andtherefore,byafebrileseizure

Childsvaccinationscheduleshouldnotbe

significantly

modified

because

of

a

historyoffebrileseizures

Pertussis

Vaccination

related

seizures

77

EPILEPSYSURGERY


78/92

Consideredincasesofintractablepartialseizures.Patientswithidiopathicprimarygeneralizedepilepsy

arenever

surgical

candidates

because

their

seizures

donotoriginatefromaresectablepartofthebrain

Commissurotomy,ordividingthecorpuscallosum,is

ofteneffective

in

reducing

the

severity

of

seizures

in

casesofrefractoryepilepsy.Theprocedurepreventsthespreadofepilepticdischargesfromonecerebrohemispheretotheoppositehemisphere

Cognitiveimpairment

Patienthasapriorhistoryofsignificantmentalretardationorothersevereneurologicalimpairments

78

KETOGENIC DIET


79/92

Ifthe

response

to

multiple

AEDs

in

combination

has

been

unsatisfactoryandepilepsysurgeryisnotpossible(asincasesofidiopathicprimarygeneralizedepilepsy)orhasnotresultedinbettercontrol.Theketogenic dietisalmostcompletelycarbohydratesfree

andsubstitutes

fats.

Anutritionistwithappropriatetrainingandexperiencemustplanthediet.Someofthemealsroutinelyrecommendedaspartoftheketogenic dietmayseemstrange(e.g:astickofbutter,andaglassofheavycreamforbreakfast).Theketogenic dietrequiresgettingusedtoand

ahigh

level

of

commitment

on

the

part

of

the

patient

and

family,

butitcanbebeneficial

Inmanycases,oneormoreofthepatientsAEDscanbetaperedoff,andoccasionally,theseizuresstoprecurringaltogether

VAGUS NERVESTIMULATION Batterypoweredstimulatorimplantedinthepatientsneck.An

electricalstimulus

is

delivered

the

nerve

at

set

intervals 79

QUESTIONSPARENTSOFTEN


80/92

ASKABOUT

SEIZURES

1)Canmychilddiefromaseizure?

Theansweristhatdeathfromaseizureoccursextremelyrarely,usually

asthe

result

of

status

epilepticus.

There

are

also

rare

reports

of

patientswithepilepsywhodiemysteriouslywhileasleep,perhapsas

theresultofaseizurethatcausedairwayobstructionortriggereda

fatalcardiacarrhythmia.Thevastmajorityofseizuresarenotlife

threatening2)Doesmychildhaveepilepsy?

Epilepsyisdefinedasahistoryofatleasttwoseizuresthatwerenotprovokedbyageneralmedicalcause(e.g:fever,hypoglycemia,sideeffectofadrugetc.).Ifapersonhasanunprovokedseizureat3yearsof

age

and

asecond

unprovoked

seizure

at

6years

of

age,

the

child

technicallyhasepilepsy.However,thischildslifeisdifferentfromthatofthepatientwhohasaseizureeveryweek.Thepointtomaketoparentsisthatepilepsyisabroadtermthatdoesnotsuggestthe

impactof

the

disorder

on

any

given

patient's

life. 80


81/92

3)Arepatientswithepilepsymentallyretarded?Ismychild

goingtobecomebraindamagedifhehasmoreseizures?

Asignificant

brain

injury

often

results

in

cognitive

impairmentandincreasesthechanceofafutureseizure.

Thusmanychildrenwithahistoryofabraininjury

(patientswith

cerebral

palsy)

are

mentally

retarded

or

otherwiseneurologicallyimpairedandalsohaveseizures,

leadingmanypeopletoassociatementalretardation

with

epilepsy However,mostpatientswithepilepsyareofnormal

intelligence.Statusepilepticus maycausepermanentneurologicaldamage;frequentcomplexpartialseizures

maybe

mildly

deleterious

to

apatients

memory 81


82/92

4)Willtheseseizureshappenagainandifso,willmychildgrowoutofthem?

seizuresare

more

likely

to

recur

in

cases

of

abnormal

neurologicaldevelopmentandwhentheMRIorEEGisabnormal.Incasesofanidentifiableepilepsysyndrome,therecurrenceriskisoftenwelldescribed

Aseizurecausedbyamedicaldisorder,suchashypoglycemia,isunlikelytorecurifthemedicalconditionthatcausedtheseizureisappropriatelymanaged

BenignRolandic

epilepsy

is

always

eventually

out

grown,

whereasjuvenilemyoclonicepilepsyislifelongcondition

Cryptogenicepilepsyprognosisaremoredifficultto

predict 82


83/92

5)WhatshouldIdoifmychildhasanother

seizure?Nothingshouldbeputintothepatients

month.Itisnotpossibleforapatientto

swallowhis

tongue

during

aseizure

(or

ever).

Apersonwhoputshisorherfingerintothe

mouthofapatientwhoishavingageneralized

tonicclonic

seizure

risks

losing

his

or

her

fingerbecause,duringtheseizure,the

patients

jaw

clenches83


84/92

~Thank

You!!

~

Forstayingawaketillnow.

84


85/92

Timeofonset

Observethepatientduringtheseizure

Beforethe

seizure

started,

was

the

patient

ill

or

acting

strangely?Duringtheseizure,wasonesideofthebodymostlyinvolved(didonearmshakeorstiffenmorethantheotherarm)?Didthepatientseyesrollbackorgotooneside,

ordid

the

head

turn

to

the

right

or

left?

Did

the

patient

turn

blue?Wasthepatientgroggyorunarousableaftertheseizureended?Didthepatientbitehisorhertongueorlosecontrolofhisorherurineorfeces?

AttendA&E

if

the

seizure

lasted

more

than

5minutes

Longerseizuresorrecurrentseizuresarereasonstocallforan

ambulance


86/92

Syncopalepisodes

Breathholding

spells

Tremor


87/92

Autism,ADHD,bipolardisorder

Behaviordisorders

Aggressiveor

agitated

behavior

may

be

noted

during

or

after

acomplexpartialseizure,butcomplexpartialseizuresareprimarily

characterizedbydiminishedresponsiveness,automations, toniceyedeviationorunilateralclonicmovements,andaposticalstate

In

many

cases

of

seizures

presenting

with

behavioral

manifestations,thepatientsbehaviorsareusuallystereotypic

Ahistoryofbehavioraloutburstsandnootherhistorysuggestiveof

acomplexpartialseizureandthepatientsbehaviorsaredifferent

duringeach

episode,

it

is

unlikely

that

these

episodes

are

seizures

TheEEGinafairlysignificantnumberofpatientswhodonothave

seizuresisabnormal


88/92

Nonepilepticseizures(NES)

Pseudoseizures Episodesofapparenttonicclonicactivityon

bothsidesofthebody,inapatientwhois

awake,are

unlikely

to

be

epileptic

seizures

Alackofapostictalstatefollowinganapparentgeneralizedtonic,clonicseizureis

highlysuggestive

of

an

NES


89/92

Apatientwithahistoryofmanyapparent

generalizedtonicclonicseizureswhohasnotbitten

hisor

her

tongue

or

become

incontinent

during

any

oftheseeventsmaybehavingNES

NESoftenincorporateexaggeratedbehaviorssuchas

flailing,thrashing

and

pelvic

thrusting.

If

apatient

hasahistoryofeventsofthiskindandmultipleEEG

tracingsarenormal,thepossibilityofNESshouldbe

considered.However,

frontal

and

temporal

lobe

epilepticseizurescanalsocausebizarremovements


90/92

Consent

Thepatientisaskediftheyarewillingtobegivenadrugthat

mayelicit

aseizure

so

that

the

seizure

can

be

observed

by

aphysician.Theyarealsotoldthat,ifaseizureisprovoked,they

willreceiveanantidotetostoptheseizure.Ifthepatientaggress,thefirstdrugs(aninjectionofnormalsaline

solutionor

an

alcohol

swab

rubbed

on

the

neck)

is

administered.Thepatientisthenobserved;ifaseizureoccurs,itisassumedtobenonepileptic.Theantidote(alsonormalsalineoranalcoholswab)isthengiven;andaborted

seizurefurther

supports

the

diagnosis

of

NES.

Another

techniquetodiagnoseNESistomeasuretheplasmaprolactin,levelimmediatelyafteraseizure(39).Theprolactinlevelisoftenelevatedafterepilepticseizuresbutnotafter

NES


91/92

Malingeringreferstoamedicalcomplaintormedicalsignfakedfora

specificpurpose(e.g:missingwork,obtainingbenefitsorcompensation,

skippingatestinschool).Afakedseizurecanbeaformofmalingering

Provocationtesting

is

auseful

tool

in

the

evaluation

of

these

patients

Aconversionsymptomisaphysicalsymptomorsignproduced

unconsciouslyindirectresponsetoapsychologicalstressor

Forexample,apersonmightcomplainthathehassuddenlybecome

unableto

move

his

arm

afew

days

after

losing

his

job.

The

neurological

examinationrevealsfindings(e.g:reflexesthataresymmetrical)thatare

inconsistentwithanorganiccause,andtheresultofdiagnostictesting,

includingMRIandEEG,isnormal.Thesymptomoftenimprovesovertime

andwith

the

aid

of

psychotherapy

Asomatizationdisorderreferstoalengthyhistoryofmultiplephysical

complaintsthat,aftercarefulinvestigation,arenotfoundtohavea

medicalcause


92/92

Munchausensyndromereferstotheconsciousproductionofactualphysicalsigns(i.e.,bythepatient

injuringor

infection

himself)

because

the

patient

derives

enjoymentfromtheexperienceofreceivingmedicalattention.Munchausensyndromebypraxyisanotoriousdisorderofparentswhoproduceactualdiseaseintheir

childand

then

take

the

child

to

the

medical

community

toinvestigatethecause.Theparentspresumablederivesatisfactionfromtheexperienceofworkingwithmedicalprofessionalstodiagnosetheirchildsmedicalproblem.

Anappropriate

legal

authority

must

be

contacted

if

Munchausensyndromebyproxyissuspected.

Documents

Paediatric Epilepsy