SEIZURES IN CHILDHOOD

FOR C-1
 Introduction
• Definition:
paroxysmal, time-limited change in motor activity and/or behavior that
results from abnormal electrical activity in the brain.
Paroxysmal synchronized discharge of cortical neurons resulting in
alteration of function (motor , sensory , cognitive, autonomic).

• Epilepsy : first episode seizure + second such seizure or enough EEG

and clinical information to convincingly demonstrate an enduring
predisposition to develop recurrences.
Two or more unprovoked seizures which occur >24 hr apart
A condition in which Sz is triggered recurrently from within the brain
without obvious precipitants
• Seizure disorder is a general term that is usually used to include any one of several disorders
including epilepsy, febrile seizures, and possibly single seizures and seizures secondary to
metabolic, infectious, or other etiologies
• An epileptic syndrome is a disorder that manifests one or more specific seizure types and has a
specific age of onset and a specific prognosis.
• An epileptic encephalopathy is an epilepsy syndrome in which the severe EEG abnormality is
thought to result in cognitive and other impairments in the patient.
• Genetic (Idiopathic) epilepsy is an epilepsy syndrome that is genetic or presumed genetic and in
which there is no underlying disorder affecting development or other neurologic function (e.g., petit
mal epilepsy).
• Structural/metabolic (Symptomatic) epilepsy is an epilepsy syndrome caused by an underlying
brain disorder (e.g., epilepsy secondary to tuberous sclerosis).
• Unknown (cryptogenic epilepsy (presumed symptomatic epilepsy)) is an epilepsy syndrome in
which there is a presumed underlying brain disorder causing the epilepsy and affecting neurologic
function, but the underlying disorder is not known.
• Unprovoked seizure : implies no closely associated concurrent illness ,
fever , or acute brain injury like head injury or drug intoxication
• Provocative factors
- High fever , Infection , Head trauma ,Hypoxia, Toxins or dugs
,Cardiac arrhythmias
Status epilepticus:
 Continuous convulsion lasting for > 30 min
OR
 Serial convulsions for > 30 min b/n which there is no return of
consciousness .
• Approximately 4-10% of children experience at least 1 seizure in
the first 16 yr of life.
• most are provoked by somatic disorder originating outside the brain:
• high fever, infection, hypoxia, toxins, arrhythmias, or electrolyte
abnormalities (Na+, Ca++);
• Less than one third of seizures in children are caused by epilepsy .
• The cumulative lifetime incidence of epilepsy is 3%, and more than
half of the cases start in childhood.
• The annual prevalence is 0.5-1%.
• Genetic factors account for at least 20 % of all cases of epilepsy
 Etiology
Any insult to the cerebral cortex can cause Sz,
Typically arise from the neocortical gray matter & the limbic
system, some may arise from subcortical regions.
The thalami, basal ganglia, and posterior fossa structures, including
the cerebellum, are considered incapable of generating seizures
 Idiopathic/cryptogenic, vascular, traumatic, developmental,
infectious, neoplastic, & degenerative causes respectively are
among the common causes.
Mechanisms of Sz-
- precise mechanism unknown.
• Two hypotheses :
1) Inhibitory neurons are selectively damaged and remaining principal
excitatory neurons become hyperexcitable .
2) Aberrant excitatory circuits are formed as part of reorganization after
injury
• Underdeveloped brain (young age) more susceptible.
• Sz may arise from areas of neuronal death, abnormal tissue (tumors,
AVMs, gliosis).
• Genetic factors
International Classification of Epileptic Sz
• Focal (Partial Sz):
- Simple partial (consciousness retained)
. motor, sensory, autonomic, psychic
- focal dyscognitive (Complex partial (consciousness impaired))
. Simple partial, followed by impaired consciousness
. Consciousness impaired at onset
- Partial Sz with secondary generalization
Classification cont’d
• Generalized Sz
- Absences- typical, atypical
- Generalized tonic-clonic, tonic, clonic
- Myoclonic
- Atonic
- Infantile spasms

• Unclassified Sz
-Neonatal seizures
Partial Sz
• Most common Sz type in all age groups, >50% of all Sz in children;
• Simple partial Sz (SPS)
- motor activity most common, clonic/tonic, involve face, neck, &
extremities,
- no automatism, some may have aura,
- average duration- 10-20 sec, conscious,
- no post-ictal event.
- EEG- spikes/sharp waves uni-/bi/ multifocal.
• Complex partial Sz (CPS)

- may begin with SPS +/- aura (a third ), may coincide or be

followed by impaired consciousness,
- brief blank stare, sudden pause
- period of impaired consciousness brief & few,
- automatism common (50-75% of cases), develop after loss of
consciousness & may persist into the post-ictal state: include lip
smacking, chewing, & swallowing,
 Cont’d
In older children- picking& pulling at clothes walking/running-
nondirective, repetitive;
- CPS can result in 2º generalization,
- duration average 1-2min,
- EEG- anterior temporal lobe sharp waves/ focal spikes, & multifocal
spikes;
- CT&MRI- abnormality in the temporal lobe sclerosis, hamartoma,
gliosis, glioma, AVMs.
 Generalized Sz
Absence Sz:
- Simple (typical) absence (Petit mal)
-Sudden cessation of motor activity or speech with a blank facial expression &
flickering of the eyelids, no loss of body tone,
-uncommon before 5 yr of age; girls>boys;
-usually <30sec, no aura/ post-ictal state,
-Not associated with a postictal state (resume preseizure activity
-countless Sz daily, automatism frequent,
-Hyperventilation for 3-4 min produces it.
EEG- Typical 3/sec spike & generalized wave discharge
Complex (atypical) absence seizures
-Have associated motor components consisting of myoclonic movement of the face,
fingers, or extremities and, on occasion, loss of body tone.
-These seizures produce atypical EEG spike and wave discharges at 2–2.5/sec
• Generalized Tonic-Clonic Sz (Grand mal)
- most common generalized Sz, may follow a partial Sz or occur de novo;
- may be ass’ed with aura (focal origin);
- duration- few minutes;
- Patients suddenly lose consciousness and, in some cases, emit a shrill, piercing cry.
- Eyes roll back, entire body musculature undergoes tonic contractions, and they
rapidly become cyanotic in association with apnea.
• The clonic phase of the seizure is heralded by rhythmic clonic contractions
alternating with relaxation of all muscle groups.
• During the seizure, children may bite their tongue but rarely vomit.
• Loss of sphincter control, particularly the bladder, is common during a
generalized tonic-clonic seizure.

• Postictally, children are initially semicomatose and typically remain in a deep

sleep from 30 min to 2 hr. .
• The postictal phase is often associated with vomiting and an intense bifrontal
headache.
• Myoclonic Epilepsies of Childhood
- repetitive Sz consisting of brief, often symmetric muscular contractions,
loss of body tone & falling/slumping forward
- 5 distinct sub groupings:
. Benign Myoclonus of Infancy
. Typical Myoclonic Epilepsy of early childhood
. Complex Myoclonic Epilepsies
. Juvenile Myoclonic Epilepsy
. Progressive Myoclonic Epilepsies
• Infantile Spasms
- begin between 4-8 mo of age;
- brief, symmetric, contractions of the neck,trunk, and extremities;
- 3 types: flexor,extensor, & mixed;
. Flexor- sudden flexion of neck, arms, & legs,
. Extensor- extension of the trunk & extremities,
. Mixed- most common.
. Clusters or volleys of seizures may persist for minutes with brief intervals b/n each
spasm
EEG- hypsarrhythmia – bilateral
• Classification :
1) Cryptogenic (10-20%)
- uneventful pregnancy and birth history
- normal dev’tal milestones before onset of seizures
- normal neurologic examination , CT and MRI of brain
2) Symptomatic (80-90%)
• Severe prenatal , perinatal and postnatal factors
Prenatal and perinatal factors: HIE with PVL, Congenital infections, Inborn errors of
metabolism, Neurocutaneous syndromes e.g. tuberous sclerosis, Congenital brain
anomalies, Prematurity
Postnatal factors: HIE,CNS infections,head trauma (subdural hematoma ,IVH)
 Cont’d

Prognosis
• Cryptogenic - good
• Symptomatic - 80-90% chance of mental retardation
- other neurologic sequelae based on underlying
CNS disorder
Evaluation of the first Sz

• ACUTE EVALUATION AND STABILIZATION

• In the Hx-
- look for features suggesting meningitis, sepsis, head trauma, ingestion of drugs,
toxins;
- details of pregnancy& delivery;
- detailed description of the Sz type:
.focal/generalized, duration,
.consciousness,
.sensory/motor (if motor: tonic, tonic-clonic, clonic, myoclonic, spasm, atonic,
etc),
 Cont’d…
.preceding aura (epigastric discomfort ,pain),
.posture of the patient, loss of sphincter control.
• On examination-
- ABC,
- Temperature, BP
- HC, length/ ht, Wt
- papiledema, retinal hemorrhage,
- focal signs- subtle hemiparesis/ hyperreflexia,
 Cont’d
- cutaneous signs of neurocutaneous syndromes
Investigations- depending on suspected cause.
- LP- has limited value,
- EEG- recommended
- for Dx of event, prediction of recurrence risk, for Dx of focal
abnormality.
Neuroimaging- generally not recommended unless neurological
examinations indicate a possible abnormality.
anticonvulsants: not recommended for only one Sz.
• CT SCAN
• MRI should be considered in:
1. child with a significant cognitive or motor impairment of unknown
etiology,
2. unexplained abnormalities on neurologic or psychiatric examination,
3. a seizure of focal onset with or without secondary generalization,
4. an EEG that does not represent a benign partial epilepsy of
childhood or primary generalized epilepsy, or I
5. n children younger than 1 year of age
 Cont’d
• Recurrent Sz
- epileptic with risk of recurrence,
- type of Sz, frequency, precipitating factors(1st convulsion may be with a
viral illness),
- behavioral abnormalities before several days,
- prolonged personality change/ intellectual deterioration (degenerative
CNS disease),
- vomiting,failure to thrive (structural lesion),
- hx of previous Rx, drugs that potentiate Sz.
 Cont’d
On examination-
- neurologic, ophthalmologic, & general exam,
- ↑ ICP, neurocutaneous synd., structural brain abnormalities ( injuries,
malformations, infections, tumors).
Neuroimaging-
- esp. for those with neurological deficits or partial Sz, or focal
neurological abn.)
- MRI- more sensitive than CT for detecting brain malformations &
dysplastic lesions.
 Cont’d
EEG,
. EEG can be abnormal in 3-5% of normal children.
. Interictal EEG recording is normal in 40% of patients
To increase yield , activation procedures are used :
1- Hyperventilation
2- Sleep deprivation
3- Photic stimulation
4- Eye closure
5- Special electrode placement
serum electrolyte( Mg, Ca, Na), glucose. Metabolic testing
LP (occasionally- chronic infection, metabolic abn.)
Note: 75% of these patients with two or more unprovoked seizure have additional seizure
Febrile Seizures
• Most common Sz disorder in childhood;
• Age dependent- common b/n 9mo-5y, peak age~14-18mo;
• Dx by exclusion
• Incidence- 3-4% of young children;
• Possible genetic predisposition;
• Generally-excellent prognosis, but may also signify serious underlying infection,
e.g. meningitis;
• Recurrence : in 30-50 % of cases
• Usu. generalized tonic clonic, rarely focal;
Clinical manifestations
Types
1) Simple
2) complex or complicated\
Simple Febrile Seizures
1) Age 9 months – 5 years
2) Core T0 ≥ 39 0C
3) GTC Sz
4) Duration : few seconds -15 minutes
• A complex febrile seizure
1. more prolonged (>15 min),
2. is focal, and/or
3. recurs within 24 hr.
Complex febrile seizures may have an approximately 2-fold long-term increase in mortality
• Febrile status epilepticus is a febrile seizure lasting >30 min.

• These factors ↑ the risk to ~9% (Vs 1%) of epilepsy:

- atypical, family history, 1st episode at < 9mo,
- delayed developmental milestones,
- preexisting neurological abnormality,
RISK FACTORS FOR OCCURRENCE OF SUBSEQUENT EPILEPSY
RISK FACTOR RISK FOR SUBSEQUENT EPILEPSY
Simple febrile seizure 1%
Neurodevelopmental abnormalities 33%
Focal complex febrile seizure 29%
Family history of epilepsy 18%
Fever <1 hr before febrile seizure 11%
Complex febrile seizure, any type 6%
Recurrent febrile seizures 4%
Work-Up
• ?Lumbar Puncture
• Electroencephalogram
• first simple febrile seizure and is otherwise neurologically healthy, an EEG need not
normally be performed
• Blood studies (serum electrolytes, calcium, phosphorus, magnesium, and
complete blood count [CBC]) are not routinely recommended
• CT or MRI is not recommended in evaluating the child after a first simple febrile
seizure.
• The work-up of children with complex febrile seizures needs to be
individualized. This can include EEG and neuroimaging
 Rx of febrile convulsion
• ABCs,
• Arrest convulsion using diazepam,
• Careful search for the cause of the fever,
• Control the fever- paracetamol & sponging;
• ?Anti epileptics
• Reassurance of parents
- fever control at home,
- possible recurrence with febrile episodes,
- goes away by 5-6 yr of age.
• ?Screening and treatment of iron deficiency
Rx of Epilepsy
• 1ststep- ensure that the patient has a Sz disorder and not pseudoseizures
Neonates Infants Children
Apnea Breath-holding spells Breath-holding spells
Jitteriness Benign myoclonus of infancy syncope
Benign neonatal sleep Shuddering attacks Migraine
myoclonus
Hyperkplexia Sandifer syndrome Benign paroxysmal vertigo
Benign torticollis in infancy Staring spells
Abnormal eye movements Tic disorders
Rhythmic movement disorder Rhythmic movement disorder
(head banging)
Parasomnias
• 2nd step- choosing an anticonvulsant when indicated. Depends on type of
the Sz.
principle/goal
1.use of only one drug with fewest side effects
2.the drug is increased slowly until seizure is controlled or undesirable
side effect develop
success rate 75-90%
3, Add additional drugs if indicated
• Drugs of choice
• Partial seizures:
- carbamazepine , phenytoin , phenobarbitone , primidone , clonazepam ,
valproic acid , lamotrigine
• GTC seizure :
- phenytoin , phenobarbitone , primidone , valproic acid , carbamazepine ,
lamotrigine
• Absence seizures :
- Ethosuximide , clonazepam , Nitrazepam , valproic acid , lamotrigine
• Myoclonic :
- Clonazepam , Nitrazepam , valproic acid , lamotrigine
• Infantile spasms :
- ACTH , Prednisolone , clonazepam , nitrazepam , vigabatrin
• Akinetic : valproic acid , clonazepam
• Adjuvant drugs : Lamotrigine ( Lamictal ), Gabapentin , clobazam ,
Tiagabine , topiramate ( Topimax ) , vigabatrin
• Duration of drug therapy
• If complete seizure control is accomplished by anticonvulsant,a minimum
of 2 seizure free years is adequate and safe period for patient with no risk
factors
• Complete seizure control for 2 years & no risk factors-chance of
recurrence 20-25% particularly in the first 6 months
• Recurrence is greater than 50% in patients with risk factors
• Weaning from drugs 3-6 months
• Rapid tapering of therapy precipitates seizures
• Prominent risk factors
i. a.age greater than 12 years at onset
ii. b.neurologic dysfunction
iii. c.Histrory of prior neonatal seizure
iv. d.Numerous seizure before control of seizure
v. e.Mixed seizures(partial & generalized)
vi. f.Failure of EEG to normalize during treatment
 Neonatal seizures
Is common indicator of significant neurologic dysfunction in the neonatal
period.
The immature brain has more excitable and more likely to develop seizures
• delay in Na+, K+-ATPase maturation 
• increased NMDA and AMPA receptor density
• the specific types of these receptors that are increased are those that are
permeable to calcium (GLUR2 AMPA receptors)
• delay in the development of inhibitory GABAergic transmission (GABA in
the immature brain has an excitatory function)
immature brain appears to be more resistant to the deleterious effects of
seizures
• Types
1) Focal clonic seizures
- consist of rhythmic twitching of muscle groups, particularly those of the extremities and
face.
- often associated with localized structural lesions as well as with infections and
subarachnoid hemorrhage
2) Multifocal clonic seizures
- convulsions are similar to focal clonic seizures but differ in that many muscle groups are
involved.------ non jacksonian march
3) Tonic seizures
- can be focal or generalized (generalized are more common).
-characterized by rigid posturing of the extremities and trunk
- sometimes associated with fixed deviation of the eyes.
4) Myoclonic seizures
- Brief focal, multifocal or generalized jerks of the extremities or body that tend to
involve distal muscle groups.
-can be distinguished from clonic seizures by the rapidity of the jerks and by their lack
of rhythmicity.
5) Subtle seizures
• Consist of :
- Chewing motions, excessive salivation
- Alterations in the respiratory rate including apnea
- Blinking, nystagmus
- Bicycling or pedaling movements
- Changes in color .
Subtle seizures occur more commonly in premature than in full-term infants.
• Spasms, focal clonic or tonic, and generalized myoclonic seizures are, as a rule,
associated with electrographic discharges (epileptic seizures)
• the subtle, generalized tonic and other myoclonic seizures are usually not
associated with discharges

• Seizures vs. Jitteriness

• Jitteriness can be defined as rapid motor activities, such as a tremor or shake,
that can be ended by flexion or holding the limb
• Unlike most seizures, is usually induced by a stimulus. Also unlike jitteriness,
seizures often involve eye deviation and autonomic changes.
 Causes of neonatal seizures
AGES 1–4 DAYS   
• Hypoxic-ischemic encephalopathy  
•   Drug withdrawal, maternal drug use of narcotic or barbiturates  
•   Drug toxicity: lidocaine , penicillin   
• Intraventricular hemorrhage   
• Acute metabolic disorders     
- Hypocalcemia      
- Hypoglycemia
-  Hypomagnesemia     
- Hyponatremia or hypernatremia      
• Inborn errors of metabolism     
• Pyridoxine deficiency (must be considered at any age)
AGES 4–14 DAYS   
• Infection   
• Metabolic disorders   
- Hypocalcemia, Hypoglycemia, persistent
•  Drug withdrawal
• Benign neonatal convulsions ( familial and nonfamilial   )
• Kernicterus , hyperbilirubinemia
AGES 2–8 WEEKS   
• Infection     
• Head injury
•   Inherited disorders of metabolism   
• Malformations of cortical development   
  - Lissencephaly, Focal cortical dysplasia, Tuberous sclerosis, Sturge-Weber syndrome
   
N.B. GTC convulsions tend not to occur in the 1st mo of life:
- The arborization of axons and dendritic processes as well as
myelination is incomplete in the neonatal brain.
- A seizure discharge, therefore, cannot readily be propagated
throughout the neonatal brain to produce a generalized seizure.
Investigations
• A lumbar puncture is indicated in virtually all neonates with
seizures, unless the cause is obviously related to a metabolic disorder
such as hypoglycemia or hypocalcemia .
• EEG is indicated in all cases , if possible .
• Treatment
• Phenobarbital
• Phenobarbital is considered by many to be the drug of first choice in
neonatal seizures.
• The usual loading dose of is 20 mg/kg.
• If this dosage is not effective, then additional doses of 5 to 10 mg/kg can
be given until a dose of 40 mg/kg is reached.
• Twenty-four hours after starting the loading dose, maintenance dosing can
be started at 3-6 mg/kg/day usually administered in 2 separate doses.
• Phenytoin and Fosphenytoin
• If a total loading dose of 40 mg/kg of phenobarbital was not effective, then a
loading dose of 15-20 mg/kg of phenytoin can be administered intravenously.
• The rate at which the dose should be given must not exceed 0.5-1 mg/kg/min
in order to prevent cardiac problems
• Fosphenytoin, which is a phosphate ester prodrug, is preferable.
• It is highly soluble in water and can be administered very safely
intravenously and intramuscularly, without causing injury to tissues.
• Lorazepam
• The initial drug used to control acute seizures is usually lorazepam.
• This can be used either as the initial drug or as second-line in a newborn who did not
respond to treatment with phenobarbital and phenytoin (15-40% of cases).
• Lorazepam is distributed to the brain very quickly and exerts its anticonvulsant effect in
<5 min.
• It is not very lipophilic and does not clear out from the brain very rapidly. Its action can
last 6-24 hr.
• Usually, it does not cause hypotension or respiratory depression. The dose is
0.05 mg/kg (range, 0.02-0.10 mg/kg) every 4-8 hr.
• Diazepam
• Diazepam is highly lipophilic, so it distributes very rapidly into the brain and then is
cleared very quickly out, carrying the risk of recurrence of seizures.
• Like other intravenous benzodiazepines, it carries a risk of apnea and hypotension
 Prognosis
- For children with epilepsy ,the prognosis is generally good
- 10-20% of children with epilepsy have persistent seizures refractory
to drugs .

Childhood Epileptic Syndromes with Generally Good Prognosis

• Benign neonatal familial convulsions
• Febrile convulsions
• Infantile familial convulsions
• Petit mal absence epilepsy
• Juvenile myoclonic epilepsy
• Benign myoclonic epilepsy of infancy
Thank you