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Seizures in Childhood`
Epilepsy in children
Epileptic seizure
→ clinical manifestation of abnormal &
excessive discharge of a set of neurons in the brain
.
Epilepsy is a chronic neurological condition characterized by
recurrent,
unprovoked
seizures (no fever, no acute cerebral
insult), occurrence of at least 2 unprovoked seizures 24 hours
apart.
Most Seizures are provoked by infection, fever, head trauma,
hypoxia, toxin, fatigue, hyperventilation alkalosis and drugs like
( INH, penicillin, theophylline …etc).
The incidence of epilepsy is 3% and more than 50% of cases
begin in childhood
An epileptic syndrome is a disorder that manifests 1 or more
specific seizure types and has a specific age of onset and a
specific prognosis.
Classification of epileptic seizures:
Old classification
1) Focal seizures (F.S.)(Partial seizures)40%:-
Motor
a) F.S. Simple partial seizures
Sensory
b) Complex partial seizures ( CPS).
Autonomic
2) Generalized seizures:-
Typical
a) Absence seizures
Atypical
Tonic
Clonic
b) Generalized tonic – clonic seizures
Myoclonic
Atonic
Infantile Spasm
3) Unclassified seizures.
50% of childhood seizures can be classified into specific syndromes (e.g):- West
syndrome and lennox – Gastaut syndrome.
Pediatric neurology lecture
By Dr. Russul Feihan
Assist professor
Babylon medical college
Part one
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*ILAE 2010 CLASSIFICATION
GENERALISED SEIZURES:-
(Discharge arises from both hemisphere)
Absence seizures
Myoclonic seizure
Tonic
Tonic clonic
Atonic seizures
FOCAL – SEIZURES
:-
(Arise from one or part of one hemisphere)
Frontal seizures
Temporal lobe seizures
Occipital seizures
Parietal lobe seizures
*international league against epilepsy
PARTIAL SEIZURE - FOCAL SEIZURE
Begin in a r el a t iv el y s ma l l gr oup of dy s f unct iona l neur ones
in one of t he cer ebr a l hemis pher es .
Ma y be her a l ded by a n a ur a (s ens or y s eizur e) which
r ef l ect s t he s it e of t he or igin
Ma y or ma y not be a s s ocia t ed wit h cha nge in cons cious nes s
or mor e gener a l ized mot or j er king.
Frontal seizure
seizures
• Involve the motor cortex
• May lead to clonic movements
→ travel proximally→
(Jacksonian) March
from face to arm to leg.
• Asymmetrical tonic seizures
→ bizarre, hyperkinetic & easily
dismissed as non –epileptic event (psychiatric disorder, sleep
disorder).
Frontal lobe seizures often occur at night and can be
very numerous and brief, but other complex partial seizures
from other areas in the brain can also occur at night, too. There
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is often contralateral dystonic posturing of the arm and, in some
cases, unilateral or bilateral tonic arm stiffening.
Temporal lobe seizure
Benigen epilepsy syndrome with focal seizures: benign childhood
epilepsy with centrotemporal spikes: (BECTS)
• The mos t common epil ept ic s y ndr ome
• This t y pica l l y s t a r t s dur ing chil dhood (a ges 3-10 y r )
wit h r emis s ion in a dol es cence.
• The chil d t y pica l l y wa kes up a t night owing t o a f oca l
(s impl e pa r t ia l ) s eizur e ca us ing bucca l a nd t hr oa t
t ingl ing a nd t onic or cl onic cont r a ct ions of 1 s ide of t he
f a ce, wit h dr ool ing a nd ina bil it y t o s pea k but wit h
pr es er v ed cons cious nes s a nd compr ehens ion.
• Dy s cognit iv e f oca l (compl ex pa r t ia l ) a nd s econda r y
gener a l ized s eizur es ca n a l s o occur .
• EEG s hows t y pica l br oa d-ba s ed cent r ot empor a l s pikes
t ha t a r e ma r kedl y incr ea s ed in f r equency dur ing
dr ows ines s a nd s l eep. MRI is nor ma l .
• Pa t ient s r es pond v er y wel l t o a nt iepil ept ic dr ugs (AEDs )
s uch a s ca r ba ma zepine.
• In s ome pa t ient s who onl y ha v e r a r e a nd mil d s eizur es
t r ea t ment might not be needed.
Benign epilepsy with occipital spikes
•
Ca n occur in early childhood a nd ma nif es t s wit h complex
partial seizures with ictal vomiting, or t hey a ppea r in later
childhood (Gastaut type) wit h complex partial seizures, visual
auras, and migraine headaches.
• Bot h a r e t y pica l l y r es ol v e in a f ew y ea r s .
Ma nif es t a t ions ma y incl ude v is ua l ha l l ucina t ions a nd
pos t ict a l hea da che (epil eps y –migr a ine s equence).
Generalized seizures:
Typical absence seizures: - pet it ma l
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• It us ua l l y starts at 5-8 yr. of age a nd a r e of t en, ov er l ooked
by pa r ent s f or ma ny mont hs ev en t hough t hey ca n occur
up t o hundr eds of t imes per da y .
• They do not have an aura, us ua l l y l a s t f or onl y a few
seconds, a nd a r e a ccompa nied by eye lid flutter or upward
rolling of the eyes, (a bs ence s eizur es ca n ha v e simple
automatisms l ike l ip-s ma cking or picking a t cl ot hing a nd
t he hea d ca n minima l l y f a l l f or wa r d).
• Abs ence s eizur es do not have a postictal period a nd a r e
cha r a ct er ized by immedia t e r es umpt ion of wha t t he
pa t ient wa s doing bef or e t he s eizur e.
• Hy per v ent il a t ion f or 3-5 min ca n pr ecipit a t e t he s eizur es
a nd t he a ccompa ny ing 3 Hz spike–and–slow-wave discharges.
• The pr es ence of per ior bit a l , l id, per ior a l or l imb
myoclonic jerks wit h t he typical absence seizures us ua l l y
predicts difficulty in controlling the seizures with medications.
•••
Early onset absence seizures (bef or e t he a ge of 4 y r ) s houl d
trigger evaluation for glucose transporter def ect t ha t is of t en
a s s ocia t ed with low CSF glucose levels a nd a n a bnor ma l
s equencing t es t of t he t r a ns por t er gene.
Atypical absence seizures:
Ha v e a s s ocia t ed myoclonic components a nd tone changes of the
head (head drop) and body a nd a r e a l s o usually more difficult to treat.
They a r e precipitated by drowsiness a nd a r e us ua l l y a ccompa nied
by 1-2 Hz s pike–a nd–s l ow-wa v e dis cha r ges .
Generalized motor seizures: Grand mal
• The mos t common gener a l ized mot or s eizur es a r e
gener a l ized t onic–cl onic s eizur es t ha t ca n be eit her
pr ima r il y gener a l ized (bil a t er a l ) or s econda r il y
gener a l ized f r oma unil a t er a l f ocus .
If t her e is no
pa r t ia l component , t hen t he s eizur e us ua l l y s t a r t s
wit h l os s of cons cious nes s a nd, a t t imes , wit h a
s udden cr y , upwa r d r ol l ing of t he ey es , a nd a
generalized tonic contraction wit h f a l l ing, a pnea , a nd
cy a nos is .
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• In s ome, a cl onic or my ocl onic component pr ecedes t he
t onic s t if f ening.
• The t onic pha s e is followed by a clonic phase t ha t , a s t he
s eizur e pr ogr es s es , s hows s l owing of t he r hy t hmic
cont r a ct ions unt il t he s eizur e s t ops us ua l l y 1-2 min
l a t er .
Tonic phase
The t onic pha s e begins wit h f l exion of t he t r unk a nd
el ev a t ion a nd a bduct ion of t he el bows . Subs equent ext ens ion
of t he ba ck a nd neck is f ol l owed by ext ens ion of a r ms a nd l egs .
Pier cing cr y ma y be pr es ent due t o pa s s a ge of a ir t hr ough
cl os ed v oca l cor ds .
Aut onomic s igns a r e common dur ing t his pha s e a nd incl ude
incr ea s e in pul s e r a t e a nd bl ood pr es s ur e, pr of us e s wea t ing
This s t a ge l a s t s f or
10-20 seconds.
Clonic phase
t r emor occur s a t a r a t e of 8 t r emor s per s econd, which ma y
s l ow down t o a bout 4 t r emor s per s econd.
The cl onic pha s e l a s t s f or
30 sec. to 1minute.
• Incont inence a nd a pos t ict a l per iod of t en f ol l ow. The
l a t t er us ua l l y l a s t s f or 30 min t o s ev er a l hour s
wit h s emicoma a nd pos t ict a l s l eepines s , wea knes s ,
a t a xia , hy per - or hy por ef l exia , a nd hea da ches .
• Ther e is a r is k of a s pir a t ion a nd inj ur y . Fir s t a id
mea s ur es incl ude pos it ioning t he pa t ient on his or her
s ide, cl ea r ing t he mout h if it is open, l oos ening t ight
cl ot hes or j ewel r y , a nd gent l y ext ending t he hea d a nd,
if pos s ibl e ins er t ion of a n a ir wa y by a t r a ined
pr of es s iona l .
•
The mout h s houl d not be f or ced open wit h a f or eign
obj ect (t his coul d dis l odge t eet h, ca us ing a s pir a t ion)
or wit h a f inger in t he mout h (t his coul d r es ul t in
s er ious inj ur y t o t he exa miner ’s f inger ).
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Benign myoclonic epilepsy of infancy cons is t s of t he ons et of
my ocl onic a nd ot her s eizur es dur ing t he 1st yr of life, wit h
gener a l ized 3 Hz s pike–a nd–s l ow-wa v e dis cha r ges .
Of t en, it is init ia l l y dif f icul t t o dis t inguis h t his t y pe f r om
mor e-s ev er e s y ndr omes , but f ol l ow-up cl a r if ies t he dia gnos is .
Juvenile myoclonic epilepsy (Janz syndrome) is t he mos t common
gener a l ized epil eps y in young adults, a ccount ing f or 5% of all
epilepsies.
It ha s been l inked t o mutations in many genes.
Ty pica l l y , it starts in early adolescence wit h 1 or mor e of t he
f ol l owing ma nif es t a t ions : myoclonic jerks in the morning, of t en
ca us ing t he pa t ient t o dr op t hings ; generalized tonic–clonic or
clonic–tonic–clonic seizures upon awakening; a nd juvenile absences.
The EEG us ua l l y s hows gener a l ized 4-5 Hz pol y s pike–a nd–
s l ow
wa v e dis cha r ges .
It ma y r es pond t o Na v a l pr oa t e which
is r equir ed l if el ong.
SEVERE GENERALIZED EPILEPSIES:
West syndrome: Starts bet ween t he a ges of 2 a nd 12 mo a nd
cons is t s
of a t r ia d of *Infantile epileptic spasms t ha t us ua l l y occur in
cl us t er s
(pa r t icul a r l y in dr ows ines s or upon a r ous a l ), **developmental
regression,
a nd a typical EEG picture called ***hypsarrhythmia.
Hy ps a r r hy t hmia is a high-v ol t a ge, s l ow, cha ot ic ba ckgr ound
wit h mul t if oca l s pikes .
Pa t ient s wit h cryptogenic ( idiopa t hic) Wes t s y ndr ome ha v e
nor ma l
dev el opment bef or e ons et , whil e pa t ient s wit h symptomatic West
s y ndr ome ha v e pr eceding dev el opment a l del a y owing t o
per ina t a l encepha l opa t hies , ma l f or ma t ions , under l y ing
met a bol ic dis or der s , or ot her et iol ogies . In boy s , Wes t
s y ndr ome ca n a l s o be ca us ed by ARX gene mut a t ions (of t en
a s s ocia t ed wit h a mbiguous genit a l ia a nd cor t ica l migr a t ion
a bnor ma l it ies ). Wes t s y ndr ome, es pecia l l y in cr y pt ogenic
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ca s es , is a medica l emer gency beca us e dia gnos is del a y ed f or 3
wk or l onger ca n a f f ect l ong-t er mpr ognos is .
} ACTH gel
} Vigabatrin: Its principal side effect is its retinal toxicity.
Valproate, nitrazepam, and clonazepam, pyridoxine, ketogenic
diet, and (IVIG).
Lennox-Gastaut syndrome t y pica l l y s t a r t s bet ween t he ages of
2
and 10 yr a nd cons is t s of a t r ia d of : developmental delay, multiple
seizure types t ha t a s a r ul e incl ude a t y pica l a bs ences ,
my ocl onic,
a s t a t ic, a nd t onic s eizur es a nd the third component is the EEG
findings 1-2 Hz s pike–a nd–s l ow wa v es , pol y s pike bur s t s in
s l eep, a nd a s l ow ba ckgr ound in wa kef ul nes s .
Pa t ient s commonl y ha v e s eizur e t y pes t ha t a r e difficult to
control, a nd mos t are left with long-term cognitive impairment and
intractable seizures despite multiple therapies.
Landau-Kleffner syndrome is a rare condit ion of unknown ca us e
cha r a ct er ized by loss of language skills a t t r ibut ed t o auditory
agnosia
in a pr ev ious l y nor ma l chil d. At l ea s t 70% have associated
clinical
seizures, but s ome do not . The s eizur es when t hey occur a r e of
several
types.
CT a nd MRI s t udies t y pica l l y y iel d nor ma l r es ul t s .
The approach and therapy :
Va l pr oic a cid is of t en t he a nt iconv ul s a nt t ha t is us ed f ir s t
t o t r ea t t he
cl inica l s eizur es a nd ma y hel p t he a pha s ia .
Some chil dr en r es pond t o cl oba za m, t o t he combina t ion of
v a l pr oic a cid a nd cl oba za m, or t o l ev et ir a cet a m.
Long-t er mt her a py is of t en needed ir r es pect iv e of wha t t he
pa t ient r es ponds t o.
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If t he s eizur es a nd a pha s ia per s is t a f t er dia zepa ma nd
s t er oids t r ia l s , t hen a cour s e of intravenous immunoglobulins
s houl d be cons ider ed.
Diagnosis of seizures
History :- f ul l des cr ipt ion of t he s eizur e a nd t he pos t
ict a l s t a t e incl uding t he t iming, dur a t ion, pr ecipit a t ing
f a ct or s , a ur a ,
1) per s ona l it y cha nges or int el l ect ua l det er ior a t ion which
ma y s ugges t a degener a t iv e dis ea s e of CNS wher e a s
v omit ing a nd FTT might indica t e a met a bol ic dis or der or a
s t r uct ur a l l es ion.
2) Physical examination :- t o s ea r ch f or or ga nic ca us e, B.P,
wt , l engt h a nd H.C s houl d be r ecor ded a nd pl ot t ed on a
gr owt h cha r t . Look f or a ny unus ua l f a cia l f ea t ur es or
a s s ocia t ed hepa t o – s pl enomega l y which ma y indica t e a
met a bol ic or s t or a ge dis ea s e. Sea r ch f or cut a neous
l es ion ( neur ocut a neous s y ndr omes ), ey e exa mina t ion f or
( r et ina l pha koma , pa pil l odema , r et ina l hemor r ha ge,
chor io r et init is ), hy per v ent il a t ion f or ( 3-4) min pr oduce
a bs ence s eizur e.
3) Investigation :- in t he f ir s t a f ebr il e s eizur e
we mus t do
a ) ( FBS, Ca
++
, mg
++
a nd el ect r ol y t e ) es t ima t ions .
b) EEG ( nor ma l EEG s een in 40% of pa t ient s ), s o we
ma y do a ct iv a t ion pr ocedur es . (hy per v ent il a t ion,
ey e
cl os ur e,
phot ic
s t imul a t ion
a nd
s l eep
depr iv a t ion), which wil l ↑t he pos it iv e r es ul t s .
Seizur es dis cha r ges a r e mor e l ikel y t o be r ecor ded
in inf a nt s a nd chil dr en t ha n in t he a dol es cent or
a dul t .
c) CT a nd MRI : indica t ed if t her e is s us picion of *int r a
– cr a nia l
l es ion, *pr ol onged pa r t ia l
s eizur e,
*f oca l
neur ol ogica l
def icit , *no r es pons e t o
a nt iconv ul s a nt a nd *ev idence of ↑ I.C. P.
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d) CSF exa mina t ion :- if t her e is s us picion of inf ect ion,
s ub-
a r chnoid
hemor r ha ge
or
demy el ina t ion
dis ea s es .
e) Specif ic met a bol ic t es t s .
Treatment of epilepsy
* Be s ur e t ha t t he pa t ient ha s s eizur e dis or der a nd not a
condit ion t ha t mimic epil eps y .
* Af t er
a f ir s t s eizur e, a nd t he pa t ient ha s
nor ma l
neur odev el opment a l s t a t us , EEG, a nd MRI , t hen t r ea t ment is
us ua l l y not s t a r t ed.
* If t he pa t ient ha s a bnor ma l EEG, MRI, dev el opment , a nd/or
neur ol ogic exa m a nd/or a pos it iv e f a mil y his t or y of epil eps y ,
t hen t he r is k is higher a nd of t en t r ea t ment is s t a r t ed.
The choice of anti- epileptic drug depends on: (t y pe of s eizur e a nd
epil eps y
s y ndr ome, pot ent ia l
f or
pa r a doxica l
s eizur e
a ggr a v a t ion, a dv er s e ef f ect s , cos t a nd a v a il a bil it y , ea s e of
init ia t ion, dr ug int er a ct ions , t he pr es ence of
comor bid
condit ions , t he coexis t ing s eizur es , mecha nis m of dr ug a ct ions ,
ea s e of us e, a bil it y t o monit or t he medica t ion a nd a dj us t t he
dos e, pa t ient 's a nd f a mil y 's pr ef er ences a nd t he t er a t ogenic
pr of il es ).
Initiating and monitoring therapy: In nonemer gency s it ua t ions or
when l oa ding is not neces s a r y , t he ma int ena nce dos e of t he
chos en AED is s t a r t ed. For exa mpl e, t he s t a r t ing dos e of
ca r ba ma zepine
is
us ua l l y 5-10 mg/kg/da y . Incr ement s
of
5 mg/kg/da y ca n be a dded ev er y 3 da y s unt il a t her a peut ic
l ev el is a chiev ed a nd a t her a peut ic r es pons e is es t a bl is hed
or unt il una ccept a bl e a dv er s e ef f ect s occur .
Titration: If a t her a peut ic l ev el needs t o be a chiev ed f a s t er , a
l oa ding dos e ma y be us ed. For v a l pr oa t e it is 25 mg/kg, f or
pheny t oin it is 20 mg/kg, a nd f or phenoba r bit a l it is 10-20 mg/kg.
Onl y one dr ug s houl d be us ed init ia l l y a nd t he dos e incr ea s ed
unt il compl et e cont r ol is a chiev ed or unt il s ide ef f ect s ha d
a ppea r ed. Then a not her dr ug be a dded a nd t he init ia l one
s ubs equent l y t a per ed.
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Monitoring: Bef or e s t a r t ing t r ea t ment , ba s el ine l a bor a t or y
s t udies incl uding CBC, l iv er enzy mes , a nd pos s ibl y kidney
f unct ion t es t s a nd ur ina l y s is a r e of t en obt a ined a nd r epea t ed
per iodica l l y . Al l er gic hepa t it is a nd a gr a nul ocy t os is a r e
mor e l ikel y t o occur in t he f ir s t 3-6 mont hs of t her a py , s o
t hes e l a bor a t or y s t udies a r e checked once or t wice dur ing t he
f ir s t mont h, t hen ev er y 3 t o 4 mont hs t her ea f t er .
Additional treatment: St er oid, Int r a v enous ga mma gl obul in (IVIG).
Ket ogenic diet a nd Sur ger y .
Discontinuation of Therapy: is us ua l l y indica t ed when chil dr en
a r e f r ee of s eizur es f or a t l ea s t 2 y r . Mos t chil dr en who
ha v e not ha d a s eizur e f or ≥2 y r a nd who ha v e a nor ma l EEG
when wit hdr a wa l is init ia t ed ha d r ema ined f r ee of s eizur es
a f t er dis cont inuing medica t ion, a nd mos t r el a ps es occur wit hin
t he f ir s t 6 mo.
Risk factors for seizure relapse:
• Abnor ma l EEG bef or e medica t ion is dis cont inued.
• Sy mpt oma t ic epil eps y .
• Abs ences s eizur e.
• Thos e who t r ea t ed wit h v a l pr oa t e.
• Ol der a ge of epil eps y ons et .
• Longer dur a t ion of epil eps y .
• Pr es ence of mul t ipl e s eizur e t y pes .
• Need t o us e mor e t ha n one AEDs .
Ther a py s houl d be dis cont inued ov er a per iod of 3-6 mont hs
beca us e a br upt dis cont inua t ion ca n r es ul t in wit hdr a wa l
s eizur es or s t a t us epil ept icus .
Wit hdr a wa l
s eizur es
a r e
es pecia l l y
common
wit h
phenoba r bit a l a nd benzodia zepines .
Seizur es t ha t occur mor e t ha n 2 - 3 mont hs a f t er AEDs a r e
compl et el y dis cont inued indica t e r el a ps e, a nd r es umpt ion of
t r ea t ment is us ua l l y wa r r a nt ed.