Childhood seizure disorders

Ayalew Moges ,M.D.

 Definitions
• Seizure (Convulsion):
1) Paroxysmal synchronized discharge of cortical neurons resulting
in alteration of function (motor , sensory , cognitive, autonomic).

2) A paroxysmal , time-limited change in motor activity and/ or

behavior that results from abnormal electrical activity in the brain .

• Epilepsy : Two or more unprovoked seizures which occur >24

hr apart
Status epilepticus:
Continuous convulsion lasting for > 30 min
OR
Serial convulsions for > 30 min b/n which
there is no return of consciousness .

Idiopathic seizure : is a term applied when the cause of a

seizure cannot be ascertained
(50-60 % of cases in pediatrics)

Aura :- suggests a focal origin of the epileptiform discharge

• Unprovoked : implies no closely associated concurrent illness ,
fever , or acute brain injury like head injury or drug intoxication

• Some provocating factors leading to reflex seizures are permitted

(unless extreme):

- Patterns and flashes from video terminals in photosensitive

epilepsy ( Photic stimulation)
- Stresses related to personal activity
- Sleep deprivation
- Hyperventilation
- severe emotional distress or excessive fatigue
• Provocative factors
- High fever , Infection , Head trauma ,Hypoxia, Toxins or dugs
,Cardiac arrhythmias
 Seizure type and epilepsy syndrome
• Seizure types , age of onset , cause , severity , co-morbid
conditions , response to medication ,and clinical course vary
widely.
• 1) Age at onset of seizures
2) Cognitive development and neurologic examination,
3) Description of seizure type, and
4) EEG findings, including the background rhythm,
help to classify ≈50% of childhood seizures into specific
syndromes .
• Two main ways of grouping patients to bring an ordered
approach of classification , treatment and prognosis :
1) Seizure type
2) Epilepsy syndrome
Etiology
• Seizure can arise from any site in the brain, but
typically localized to the neocortical gray matter
and the limbic system ( hippocampus &
amygdala ).

• Sub cortical structures have neuromodulatory

influences or serve as relay stations for spread
of seizure.
 Epidemiology
• cumulative lifetime incidence of epilepsy is
3%; more than half of cases begin in
childhood
- Genetic factors account for at least 20 % of
all cases of epilepsy
• Seizures are common in the pediatric age
group and occur in 10 % of children .
• Less than one third of seizures in children are
caused by epilepsy .
 History
• Perinatal problems (asphyxia , jaundice ,trauma,meningitis,etc.)
• Developmental milestones(motor ,speech , language)
• Seizure characteristics (during ictal period)
- Seizure type , time of day ,frequency
- Duration of seizure
- State of consciousness (retained or impaired)
- Posture of the patient, vocalizations
- Presence and distribution of cyanosis
- Loss of sphincter control (especially bladder)
- Postictal state (sleep ,headache , hemiparesis )
- Presence of fever
• Headache ,vomiting
• Precipitating factors
• Presence of aura preceding the convulsion
- Epigastric discomfort or pain
- Feeling of fear , head ache ,chest pain
• Behavior of the child immediately preceding the seizure
• Past Hx of meningitis
• Past Hx of seizure disorder & use of anticonvulsants
• Trauma
• Poisoning
• Previous therapy
• Family Hx of seizures
• Any evidence of active CNS infection
 Physical examination
• The physical , ophthalmologic , and neurologic examination
provides information about the presence of :
1) Increased ICP
2) Neurocutaneous syndromes
3) Structural brain abnormalities
- Brain malformations , injuries , infections , tumors
• BP
• HC,length,weight
• Fundoscopy
- Papilledema , retinal hemmorrhages ,
coloboma , chorioretinitis , macular changes
• Unusual facial features & hepatosplenomegaly
- Metabolic or storage diseases
• Skin lesions
- Shagreen patch , multiple cafeau-lait spots
• Localizing neurologic signs
 Mechanisms of seizures
• Mechanism of seizures : 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

Phenomenon of kindling
In experimental animals, repeated subconvulsive
stimulation of the brain (amygdala) ultimately leads to a
generalized convulsion by changes in synapses.This synaptic
mechanism may also occur in humans.
 Classification of seizures
• EEG + Clinical
• EEG is an important adjunct in classification
of seizures
• Classifying the seizure type :
- Provides a clue to the cause of the
seizure disorder
- Allows a firm basis for making a
prognosis
- Helps in choosing the most appropriate
treatment
  International Classification of Epileptic
Seizures
1) PARTIAL SEIZURES
a) Simple partial
(consciousness retained)  
- Motor  
- Sensory  
- Autonomic  
- Psychic
b) Complex partial
(consciousness impaired)  
 - Simple partial, followed by
impaired  consciousness
- Consciousness impaired at onset

c) Partial seizures with secondary

generalization (Jacksonian march)
2 ) GENERALIZED SEIZURES
a) Absences ( Petit mal)
- Typical (simple) 
- Atypical (complex)
b) Generalized tonic - clonic (grand mal)
c) Tonic
d) Clonic
e) Myoclonic
f) Atonic
g) Infantile spasms
3 ) UNCLASSIFIED SEIZURES
- Neonatal seizures

N.B. Classification of Epilepsies and Epileptic

Syndromes in children
*** SPECIAL SYNDROMES
- Situation-related seizures  
- Febrile convulsions  
- Isolated seizures or isolated status epilepticus
- Acute symptomatic seizures: e.g., alcohol withdrawal seizures,
eclampsia , uremia
 Infantile spasms
• Age of onset : 4 - 8 months
• Characterized by brief symmetric contractions of the neck ,trunk
, and extremities

• Types : flexor , extensor (least common form ), mixed (most

common type)

• Clusters or volleys of seizures may persist for minutes with brief

intervals b/n each spasm

• EEG : chaotic pattern of high voltage ,bilaterally asynchronous ,

slow-wave activity ( hypsarrhythmia) or modified
hypsarrhythmia pattern
• 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)
Prognosis
• Cryptogenic - good
• Symptomatic - 80-90% chance of mental retardation
- other neurologic sequelae based on underlying
CNS disorder
Treatment
- Exogenous ACTH and glucocorticoids suppress CRH synthesis
• ACTH ( preferred drug )
- 20 U/day IM for 2 weeks ; if no response 30-40 U/day IM for
additional 4 weeks
• Prednisolone 2 mg/kg/day for 2 weeks ; if no response same
dose for additional 4 weeks
 Neonatal seizures
• Types
1) Focal 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 .
3) Tonic seizures
- characterized by rigid posturing of the extremities and trunk

- sometimes associated with fixed deviation of the eyes.

4) Myoclonic seizures
- Brief focal or generalized jerks of the extremities or body that
tend to involve distal muscle groups.

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 .
 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
 PAROXYSMAL NONEPILEPTIFORM DISORDERS   
• Jitteriness   
• Benign neonatal sleep myoclonus

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 .
 Febrile seizures
• Most common cause of seizures in childhood
• Excellent prognosis
• Dx by exclusion
• Incidence : ≈ 3-4% of young children
• Recurrence : in 30-50 % of cases
• Genetic predisposition
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
5) R/o .CNS infections (bacterial ,viral)
( Do LP if any doubt !!!!! )
.Sepsis
* Risk of epilepsy = 1%
 Complex or Complicated febrile
seizures
1)Duration >15 min
2)Repeated convulsions in 24 hrs
3)Focal seizure activity
4)Focal findings during postictal period
** LP must be done if no C/I !
Seizure with fever
-Children with a chronic seizure disorder with
more seizures during fever
 Treatment
• Search for cause of fever
• Reassurance & education of parents
• Control fever
-Antipyretics : not shown to prevent seizure
recurrence
• Long term anticonvulsants not indicated !!
• Diazepam oral 0.3mg/kg Q 8hourly
(1mg/kg/24hr)
for duration of illness(2-3 days)
 SIMPLE PARTIAL SEIZURES (SPS)
• Motor activity is the most common symptom of SPS.
- clonic or tonic movements
- Tend to involve the face, neck, and extremities.
- Versive seizures consisting of head turning and conjugate
eye movements .
• Automatisms do not occur with SPS
• Some patients complain of aura (chest discomfort, headache)
• The average seizure persists for 10–20 sec
• Patients remain conscious
• No postictal phase
• EEG : spikes or sharp waves unilaterally or bilaterally or a
multifocal spike pattern.
 COMPLEX PARTIAL SEIZURES (CPS)
• Other names :Psychomotor or temporal lobe seizure
• Occurs with or without an aura
• The average duration of a CPS is 1–2 min
• Automatisms are a common feature of CPS in infants and
children, occurring in ≈50–75% of cases
- Alimentary automatisms : lip smacking,
chewing, swallowing, and excessive salivation.
- Automatisms develop after the loss of consciousness
and may persist into the postictal phase .
- Automatic behavior in older children consists of semi-
purposeful, incoordinated , and unplanned gestural
automatisms.
• EEG : anterior temporal lobe sharp waves or focal spikes, and
multifocal spikes are a frequent finding.
• Around 20% of infants and children have a normal routine
interictal EEG.
• Some children have interictal sharp waves or spikes
originating from the frontal, parietal, or occipital lobes.
• CT scanning and especially MRI most likely identify an
abnormality in the temporal lobe of a child with CPS :
- Mesial temporal sclerosis
- Hamartoma
- Postencephalitic gliosis
- Subarachnoid cysts
- Infarction
- Arteriovenous malformations, and
- Slow-growing glioma
 ABSENCE ( petit mal ) SEIZURES

1) Simple ( typical ) absence seizures

• Characterized by a sudden cessation of motor activity or
speech with a blank facial expression and flickering of the
eyelids.
• Uncommon before age 5 yr
• More prevalent in girls
• Never associated with an aura
• Rarely persist longer than 30 sec
• Not associated with a postictal state (resume preseizure
activity)
• May occur too frequently daily
• NO loss of body tone, but head may fall forward slightly.
• Automatic behavior frequently accompanies simple absence
seizures.
• Hyperventilation for 3–4 min routinely produces an absence
seizure.
• The EEG shows a typical 3/sec spike and generalized wave
discharge .

2) 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 SEIZURES
• Common
• May follow a partial seizure with a focal onset (secondary
generalization) or occur de novo.
• May be associated with an aura, suggesting a focal origin of the
epileptiform discharge.
• 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.
• The clonic phase slows toward the end of the seizure, which
usually persists for a few minutes, and patients often sigh as
the seizure comes to an abrupt stop.
• 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

• Characterized by repetitive seizures consisting of brief, often

symmetric muscular contractions with loss of body tone and
falling or slumping forward, which has a tendency to cause
injuries to the face and mouth.

• Myoclonic epilepsies include a heterogeneous group of

conditions with multiple causes and variable outcomes.

• At least five distinct subgroupings can be identified.

 1 ) Benign Myoclonus of Infancy

• Begins during infancy

• Consists of clusters of myoclonic movements confined to the
neck, trunk, and extremities.
• The myoclonic activity may be confused with infantile spasms;
however, the EEG is normal in patients with benign myoclonus.
• The prognosis is good, with normal development and the
cessation of myoclonus by 2 yr of age.
• An anticonvulsant is not indicated.
 2 ) Typical Myoclonic Epilepsy of Early Childhood
• Near normal before the onset of seizures, with an unremarkable
pregnancy, labor, and delivery and intact developmental
milestones.
• The mean age of onset is ≈2 yr, but the range spreads from 6
mo to 4 yr.
• The frequency of myoclonic seizures varies; they may occur
several times daily, or children may be seizure-free for week
• A few patients have febrile convulsions or generalized tonic-
clonic afebrile seizures that precede the onset of myoclonic
epilepsy.
• Approximately half of patients occasionally have tonic-clonic
seizures in addition to the myoclonic epilepsy.
• The EEG shows fast spike wave complexes of ≥2.5 Hz and a
normal background rhythm in most cases.
• At least one third of the children have a positive family history of
epilepsy, which suggests a genetic etiology in some cases.
• The long-term outcome is relatively favorable.
• Mental retardation develops in the minority, and >50% are
seizure-free several years later.
• Learning and language problems and emotional and behavioral
disorders occur in a significant number of these children and
require prolonged follow-up by a multidisciplinary team.
 3 ) Complex Myoclonic Epilepsies
• A heterogeneous group of disorders with a uniformly poor
prognosis.
• Focal or GTC seizures beginning in the 1st yr of life typically
antedate the onset of myoclonic epilepsy.
• The generalized seizure is often associated with an upper
respiratory tract infection and a low-grade fever and frequently
develops into status epilepticus.
• Approximately one third of these patients have evidence of
delayed developmental milestones.
• A history of HIE in the perinatal period and the finding of
generalized UMN and extrapyramidal signs with microcephaly
constitute a common pattern among these children.
• A family history of epilepsy is much less prominent in this group
compared with typical myoclonic epilepsy.
 4 ) Juvenile Myoclonic Epilepsy (Janz Syndrome)
• Usually begins between the ages of 12 and 16 yr and accounts
for ≈5% of the epilepsies.
• A gene locus has been identified on chromosome 6p21.
• Patients note frequent myoclonic jerks on awakening, making
hair combing and toothbrushing difficult.
• The myoclonus tends to abate later in the morning .
• A few years later, early morning GTC seizures develop in
association with the myoclonus.
• The EEG shows a 4–6/sec irregular spike and wave pattern,
which is enhanced by photic stimulation
• The neurologic examination is normal, and the majority
responds dramatically to valproate, which is required lifelong.
• Discontinuance of valproate causes a high rate of recurrence of
seizures.
 5 ) Progressive Myoclonic Epilepsies

• Heterogeneous group of rare genetic disorders

• Uniformly has a grave prognosis.
• Includes :
- Lafora disease
- Myoclonic epilepsy with ragged-red fibers (MERRF)
- Sialidosis type 1 , ceroid lipofuscinosis
- Juvenile neuropathic Gaucher disease
- Juvenile neuroaxonal dystrophy.
 Laboratory tests
• Serum electrolytes (Na , Ca,Mg)
• Toxicology screening (urine and serum )
• Metabolic testing (Urine and serum )
• Serum glucose * *
• EEG * *
• Lumbar puncture
• Skull X-Ray
• Neuroimaging (CT AND MRI)
- Focal neurologic deficits
- MRI of the brain is recommended over CT
scanning.
 Diagnosis
• Clinical features : Eye witness
• EEG abnormalities during the ictal or interictal period
- Interictal EEG recording is normal in 40% of patients
So , normal EEG does not preclude the Dx of epilepsy
- To increase yield , activation procedures are used :
1- Hyperventilation
2- Sleep deprivation
3- Photic stimulation
4- Eye closure
5- Special electrode placement (zygomatic leads)
• Imaging (CT or MRI)may be abnormal which would be
supportive
• Routine lab
 Disorders that mimic childhood epilepsy
1) Confused with GTC seizure
- Pallid syncope (reflex anoxic seizure)
- Vasodepressor syncope (reflex anoxic seizure)
- Cyanotic breath-holding attacks
- Collapsing attacks with cardiac dysrhythmias
- Cataplexy
2) Confused with generalized absence seizure
- Behavioral staring attacks
- Complex partial seizures
- Tic disorder
3) Confused with complex partial seizures
- Self-stimulatory behavior , especially in children with autistic
spectrum disorders
- Sleep walking
- Night terrors
- Temper tantrums with amnesia for the rage event
- Benign paroxysmal vertigo
- Migraine-related disorders
4) Confused with epileptic myoclonus
- Physiologic hypnagogic myoclonus
- Benign infantile sleep myoclonus
- startle disease
 Management
1) Establish diagnosis
- correctly determine seizure type,epileptic
syndrome,etiology and precipitating factors.

2) Principles of pharmacologic therapy

a) Begin monotherapy with drug of choice
- Monotherapy should be tried best before dual
therapy . Polytherapy is used occasionally .
b) Push the first drug tried
c) Add additional drugs
 3) 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
 Factors increasing risk of recurring seizures
1) Evidence of structural lesion
-strongest predictor
2)Abnormal EEG
3) Partial seizure type
4) Positive family Hx
5) Postictal motor paralysis
N.B. - persons with no risk factor have < 50%
(usually 30%)chance of 2nd seizure within 2 yrs
- persons with 2 or more risk factors have a
100% chance of seizure recurrence within 2
yrs….probably should be treated.
4) Drug interactions
a) B/n antiepileptic drugs
b) B/n antiepileptic drugs & and other types of drug

5) Antiepileptic drug plasma conc. Determinations

(Blood levels)
6) Discontinuing therapy
- Anticonvulsant Rx could be discontinued 2 years after patient
has been free of seizures.
- Drugs should be withdrawn slowly: elimination of one pill per
day (or 25% of daily dosage) every five elimination half-lives is
probably the optimal regimen . The weaning process may take
2-3 months up to 3-6 months ( especially benzodiazepines
e.g. clonazepam and barbiturates e.g. phenobarbitone )
- Rapid tapering of therapy precipitates seizures
7) Medically intractable epilepsy
- Causes :
a) improper Dx of seizure type (resulting in use
of improper antiepileptic drugs)
b)failure to push the drugs used to the maximal

dosage
c) failure to use all available antiepileptic drugs
8) Ketogenic diet
-Lennox-Gastaut syndrome refractory to
standard drug therapy
-progressive myoclonic epilepsy
• The diet contains a high proportion of fats and
small amounts of CHO & protein(typically ratio of
4:1 )
• Brain uses ketone bodies as primary fuel when it
is deprived of glucose as an energy
source(Ketosis)….acetoacetate & b-hydroxy
butiric acid act as anticonvulsant drugs
• Effective in 1/3 -1/2 of children
9) Surgery
a) Temporal lobectomy
b) Nontemporal resections
c) Corpus callostomy
d) Hemispherectomy
 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