1. Dr. Parag Moon
Senior resident,
Dept. of Neurology
GMC Kota.

2.  Definition:
 An epileptic disorder characterized by a
cluster of signs and symptoms customarily
occurring together; these include type of
seizure, etiology, anatomy, precipitating
factors, age of onset, severity, chronicity,
diurnal and circadian cycling and prognosis.
 Must involve more than just a seizure type.

3.  Neonatal period
 Benign familial neonatal epilepsy (BFNE)
 Early myoclonic encephalopathy (EME)
 Ohtahara syndrome
 Infancy
 Epilepsy of infancy with migrating focal seizures
 West syndrome
 Myoclonic epilepsy in infancy (MEI)
 Benign infantile epilepsy
 Benign familial infantile epilepsy
 Dravet syndrome
 Myoclonic encephalopathy in non-progressive
disorders

4.  Childhood
 Febrile seizures plus (FS+) (can start in infancy)
 Panayiotopoulos syndrome
 Epilepsy with myoclonic atonic (previously astatic) seizures
 Benign epilepsy with centrotemporal spikes (BECTS)
 Autosomal dominant nocturnal frontal lobe epilepsy
(ADNFLE)
 Late-onset childhood occipital epilepsy (Gastaut type)
 Epilepsy with myoclonic absences
 Lennox–Gastaut syndrome
 Epileptic encephalopathy with continuous spike-and-wave
during sleep (CSWS)*
 Landau–Kleffner syndrome (LKS)
 Childhood absence epilepsy (CAE)

5.  Adolescence–adult
 Juvenile absence epilepsy (JAE)
 Juvenile myoclonic epilepsy (JME)
 Epilepsy with generalized tonic-clonic seizures
alone
 Progressive myoclonus epilepsies (PME)
 Autosomal dominant epilepsy with auditory
features (ADEAF)
 Other familial temporal lobe epilepsies
 Less specific age relationship
 Familial focal epilepsy with variable foci
(childhood to adult)
 Reflex epilepsies

6.  Onset days 2–15, commonly in first week.
 Family history, autosomal dominant inheritance.
 Mutations of potassium channel genes KCNQ2
and KCNQ3, and a nicotinic cholinergic receptor
channel gene.
 Cluster of focal clonic seizures, often secondarily
generalized or apneic.
 No specific EEG pattern, interictal background
may be normal.
 Spontaneous recovery with favorable outcome.
 Can occur as late as 3.5 months and occur later
in premature infants.

7. EEG showing focal or multifocal sharp waves or
“theta pointu alternant” pattern

8.  Onset of erratic myoclonus before 3 months
(usually first 30 days).
 Massive myoclonus, focal seizures and late-
onset tonic spasms also occur.
 Developmental arrest.
 Suppression-burst EEG pattern.
 Refractory to antiepileptic therapies.
 Poor outcome; 50% mortality in first year.
 Associated with inborn errors of metabolism,
especially glycine encephalopathy,

9. EEG showing diffuse suppression burst pattern

10.  Early infantile epileptic encephalopathy with
suppression bursts
 Onset of tonic spasms before 3 months (usually first
10 days).
 Developmental arrest.
 Suppression-burst EEG pattern.
 Refractory to antiepileptic therapies.
 Frequent progression West syndrome.
 Poor outcome; severe neurological impairment,
death.
 Distinguished from EME by absence of erratic
myoclonus, presence of tonic spasms at onset
 Frequently accompanied by structural lesions.

12.  Malignant migrating partial epilepsy in infancy
 Onset 6 months.
 Clusters of severe, polymorphous focal seizures,
frequently evolving into generalized.
 Progressive decline in psychomotor development
 Within weeks to months, patients enter a “stormy
phase” with frequent polymorphous focal
seizures that become virtually continuous.
 EEG shows multifocal discharges, typically
rhythmic theta activity, that progressively expand
to adjacent cortical areas
 Ictal and interictal EEGs become indistinguishable
 Prognosis is poor

13.  Onset before 1 year, peak 4–7 months.
 Clusters of spasms.
 Spasms are myoclonic-tonic contractions and
can be either flexor, extensor, head or
combination.
 Developmental arrest and psychomotor
deterioration.
 Hypsarrhythmia interictal EEG pattern.
 Often refractory to antiepileptic therapies.

14. EEG showing hypsarrythmia

15.  Onset 3–20 months.
 Clusters of brief partial seizures.
 Normal development before onset.
 Responsive to antiepileptic therapies.
 Favorable outcome.
 Familial form based on a family history of
infantile convulsions without later development
of other forms of epilepsy
 Inherited as autosomal dominant.
 Peak age of seizure onset in familial form is 4–7
months

16. Ictal EEG showing seizure onset starting at O1, spreading to Cz and C3

17.  Onset 3 months to 6 years.
 Generalized tonic-clonic seizures (GCTS)
occurring with fever.
 Continuation of febrile GCTS after 6 years of age
or occurrence of afebrile GCTS.
 Family history of childhood febrile seizures.
 Normal interictal EEG.
 Favorable outcome.
 May experience febrile myoclonus
 Associated with mutations of the SCN1A sodium
channel gene.

18.  Previously called early-benign childhood seizures
with occipital spikes
 Childhood onset (peak 5 years).
 Focal autonomic seizures or autonomic status
epilepticus, frequently with emesis.
 Interictal EEG with shifting or multifocal high-
amplitude spikes, often with occipital predominance.
 Favorable outcome with remission in 1–2 years and
normal development.
 EEG spikes occur most commonly in the posterior
areas of the brain including the occipital lobe
 30% of patients show only extraoccipital discharges
or normal EEGs

19. EEG showing clone-like repetitive occipital spike-wave
discharges

20.  Formerly known as myoclonic astatic epilepsy of Doose
 Onset between 18 months and 5 years (peak 3 years).
 Myoclonic atonic seizures are primary seizure type, but
heterogeneous presentation.
 Initial massive myoclonic jerk followed immediately by
severe loss of muscle tone, often causing a fall and
referred to as a drop attack
 Most patients experience heterogeneous seizure
presentations
 Interictal EEG with 4–7 Hz spike and slow-wave or
polyspike and slow-wave complexes.
 Variable course and outcome.
 One half experience encephalopathic effects and suffer
from persistent GTCS, myoclonic-atonic status and
dementia.

21. Interictal EEG showing 4-7 Hz spike and slow wave pattern

22.  Defined as syndrome characterised by
multiple type of seizures including a nucleus
of brief tonic or atonic seizures, absence
seizures, myoclonic jerks(less common).
 Interictal EEG pattern of diffuse slow(less than
2.5 hz) spike and wave complexes.
 Mental retardation common(90%).
 Non convulsive status epilepticus common.

23.  More common in males
 Peak age of onset between3-5yrs
 More frequent during sleep
 Two thirds to three-fourths-secondary or
symptomatic
 Cortical malformations- B/L perisylvian and
central dysplasia, diffuse subcortical laminar
heterotopias, focal cortical dysplasia.

24.  Tonic seizures-most characteristic
 Occur during non REM sleep for avg.10 secs
 Axial subtype-B/L symmetrical contraction of
axial muscles
 Axorhizomelic-abduction & elevation of arms
 Global tonic attacks-affect most muscles.
 May be associated with autonomic
phenomenon

25. Discharge of high amplitude fast rhythms lasting for about 10 secs followed by
Polyspikes and spike and wave complex

26. EEG showing spike-wave of 2.5 hz or less.

27.  Atypical absence seizures- 13 to 100%
 Burst of spike-wave of 2.5 hz or less seen.
 Not precipitated by hyperventilation or
photic.
 Myoclonic- less common
 Atonic seizures-26-56%
 Non convulsive status epilepticus-50-75%
 Consist of subcontinuous atypical absence
periodically interrupted by brief tonic
seizures.

28.  Predictors of Prognosis
1. Age of onset
2. Frequency of tonic seizures
3. Repeated episodes of non convulsive status
4. Constant slow EEG background.

29.  Treatment
 Refractory to treatment
1. AED-Benzodiazapines, Sodium valproate,
Felbamate, Lamotrigine, Topiramate
2. Ketogenic diet
3. Vagal nerve stimulation
4. Surgical resection

30.  Onset 5mth to 5yrs.
 May be preceded with febrile convulsion.
 Myoclonus may be axial or generalised.
 Myoclonus increased in drowsiness.
 Triggered by sudden tapping or acoustic stimuli.
 Interictal EEG may be normal.
 Sleep record-burst of generalised fast SW or
polySW.
 Favorable prognosis
 Often controlled with valproate monotherapy.

31.  Benign rolandic epilepsy or sylvian epilepsy
 Onset between 2 and 13 years (peak 9–10 years).
 Normal development before onset and during
course of epilepsy.
 Autosomal dominant inheritance.
 Focal seizures with motor signs often hemifacial
without impairment of consciousness.
 Interictal EEG with high-voltage centrotemporal
spikes on a normal background.
 Favorable outcome with recovery in adolescence.

32. EEG during drowsiness and sleep showing frequent bilateral
synchronous/independent biphasic spikes followed by slow waves in
the centro-temporal regions.

34.  Childhood/adolescent onset (mean 11 years).
 Autosomal dominant inheritance due to nicotinic
acetylcholine receptor
 (AChR) channelopathy.
 Focal sensory-motor seizures occurring in NREM
sleep.
 Variable manifestations including prominent
motor features such as jerking, dystonia, and
automatisms, as well as vocalizations, and non-
specific auras
 Ictal EEG with frontally dominant slow discharges.
 Prognosis is typically favorable.

36.  Formerly grouped with Panayiotopoulos
syndrome as childhood occipital epilepsy, late
onset Gastaut type
 Childhood onset (mean 8–9 years).
 Occipital seizures, primarily visual manifestations
including hallucinations and temporary
blindness.
 Interictal EEG with occipital spike-waves upon
eye closure and with attenuation upon eye
opening.
 Responsive to antiepileptic therapies.
 Favorable prognosis with remission in
adolescence.

37. EEG showing B/L occipital spike and wave complexes with right
dominance.

38.  Recognized by Tassinari and colleagues
 Childhood onset (mean 7 years).
 Myoclonic absence seizures: loss of
consciousness with severe, rhythmic
myoclonic jerks.
 Myoclonias are bilateral and rhythmic,
maximally involving proximal limb muscles,
and may be associated with a tonic
contraction associated with raising the arms

39.  Ictal EEG showing bilateral, synchronous
spike and slow-wave complexes at 3 Hz
associated with myoclonus.
 Interictal EEG is variable and ranges from
normal to background slowing and
generalized spike and slow-wave activity
 Variable course and outcome.
 Many resistant to drug therapy
 Less favorable outcomes associated with poor
seizure control.

40.  Childhood onset (peak 4–7 years).
 Various generalized and focal seizures.
 Cognitive deterioration and behavioral
disturbances.
 EEG with continuous spike and slow wave seen in
at least 85% of slow-wave sleep.
 Characterized by a hallmark EEG presentation,
called continuous spike and-wave during sleep
(CSWS) or electrical status epilepticus of slow-
wave sleep (ESES) accompanied by seizure activity
and neuropsychological deficits.

41. EEG showing continuous spike and-wave during sleep (CSWS) or
electrical status epilepticus of slow-wave sleep.

42.  2–5 years after seizure onset, CSWS pattern
emerges & is temporally associated with
emergence of neuropsychological and
behavioral disturbances as well as onset of
atypical absence seizures in wakefulness.
 No associated brain pathology
 Typically some improvement in neurological
status once epileptiform activity has resolved.

43.  Severe myoclonic epilepsy or severe
polymorphic epilepsy of infants.
 Onset at 2-12months of age.
 Early appearance of convulsive seizures which
are prolonged (10 to 90 mins) and often
lateralised.
 Related to fever in two third to three-fourth
 Myoclonic seizure occur during second and
third year of life.

44.  Massive myoclonias
 Axial muscles-> falls
 Predominate on awakening
 Precipitated by variation in ambient light
intensity
 EEG-burst of irregular polyspike-wave
 Segmental or erratic myoclonia
 Distal limbs or face
 More palpable than visible
 Common during period of severe convulsion
 Not associated with EEG paroxysm.

45.  Atypical absence-40%
 Focal seizures-1/2 to 3/4th
 Initial development normal, progress slows
down in second and third year and comes to
standstill
 Ataxia-59%
 EEG-generalised discharges of fast spike-
wave or polyspike wave in burst or isolation
 Photic stimulation 40%
 Theta rhythm of 5-6Hz in central and vertex
95%

46. 17 m. child with SW induced by opening and closing eyelids
Recurrence of diffuse SW during sleep

47.  Treatment
 Treatment of febrile diseases
 Avoid hot baths
 AED increased during vaccination
 Valproate, benzodiazapines
 Stiripentol

48.  Onset between 3 and 8 years (peak 5–7 years).
 Acquired aphasia (verbal auditory agnosia).
 Continuous spike and wave discharges on EEG, activated in
sleep.
 Resolution of EEG abnormalities in adolescence.
 Deterioration or significant fluctuation in language are
indications to evaluate for LKS.
 Generalized or focal seizures occur in up to 80% of
children and may precede or follow the onset of aphasia
 Seizures commonly resolve before age 15 years
 Neuropsychological deficits tend to persist.
 many epileptologists consider CSWS and LKS on a common
syndromic spectrum and consider LKS a specific
presentation of epilepsy with CSWS

50.  Also called “pyknolepsy”
 Onset between 4 and 10 years in a previously
healthy child.
 Frequent typical absence seizures.
 Maintenance of neurological status and
development during course of epilepsy.
 Ictal EEG: generalized, high-amplitude 3 Hz spike
and slow-wave complexes, lasting 4–20 s.
 Generally responsive to antiepileptic drug (AED)
usually with ethosuximide or valproate.
 One-half of patients develop convulsive seizures,
associated with a worse prognosis.

52.  Onset 7–17 yrs (peak 10–12 yrs) in previously
healthy child.
 Typical absence seizures.
 Secondary seizure type: GTCS.
 Ictal EEG: generalized, high-amplitude spike and
slow-wave complexes ≥3.5 Hz, typically >4 s
duration.
 Absence seizures in JAE are more sporadic.
 EEG is slightly faster with generalized spike wave
paroxysms of 3.5–4 Hz.
 Usually controlled with AEDs
 Prognosis is favorable.

53.  Onset 8 to 26 years (peak 12–18, mean 14
years).
 Bilateral myoclonic jerks, most frequently
upon awakening.
 Secondary seizure types including GTCS and
typical absence seizures.
 Ictal EEG with generalized high-amplitude
polyspike-and-wave.
 Usually demonstrate a life-long
predisposition to generalized seizures.

55.  Severe myoclonias.
 Epilepsy with generalized seizures, especially tonic-clonic,
clonic-tonic-clonic, and clonic.
 Progressive course including dementia and cerebellar
manifestations.
 EEG typically shows progressive background slowing, generalized
and multifocal abnormalities, and photosensitivity
 Unverricht–Lundborg disease (ULD) and Lafora disease.
 Onset of ULD occurs between 7 and 16 years of age (peak 9–13
years)
 Characterized by severe myoclonias, generalized clonic-tonic-
clonic seizures, and cerebellar ataxia.
 ULD has a slow progression with little to no cognitive impairment
 Caused by a mutation in cystatin B gene

56.  Lafora disease presents at a similar age
 Has a severe prognosis
 Rapid progression to dementia and nearly
constant myoclonus
 Death in 2–10 years.
 Autosomal recessive inheritance
 Caused by mutations in enzyme laforin.
 Other PMEs include neuronal ceroid
lipofuscinoses, sialidosis, and myoclonic
epilepsy with ragged-red fibers (MERRF).

57.  Onset 1-14 yrs of age
 Focal onset motor seizures: simple partial or
evolve into complex partial or secondary
generalisation
 Seizures start in same hemisphere
 Progressive hemiatrophy with lesser atrophy
on other side
 More pronounced in perisylvian region
 T/T-IVIg, corticosteroids
 Hemispherectomy for resistant cases.

58. Ictal EEG showing nearly continuous sharp waves and spikes in the
left frontal-temporal region

59. Thanks

60.  Epilepsy in Children and Adolescents:Editor James W.
Wheless:Wiley-Blackwell:2013.
 Aicardi's Epilepsy in Children;Third edition:Dec2003
 Epileptic Syndromes in Childhood: Clinical Features,
Outcomes and Treatment:Peter Camfield, Carol
Camfield:Epilepsia,43(Suppl. 3):27–32, 2002
 EEG in Benign and Malignant Epileptic Syndromes of
Childhood:Ivo Drury:Epilepsia 43(Suppl. 3):17–26, 2002
 Freedman SB, Powell EC. Pediatric seizures and their
management in the emergency department. Clin Ped
Emerg Med. 2003;4:195-206.