Dr. Parag MoonSenior resident,

Dept. of NeurologyGMC Kota.

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.

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

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)

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

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.

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

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,

EEG showing diffuse suppression burst pattern

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.

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

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.

EEG showing hypsarrythmia

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

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

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.

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

EEG showing clone-like repetitive occipital spike-wave discharges

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.

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

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.

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.

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

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

EEG showing spike-wave of 2.5 hz or less.

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.

Predictors of Prognosis

1. Age of onset

2. Frequency of tonic seizures

3. Repeated episodes of non convulsive status

4. Constant slow EEG background.

Treatment

Refractory to treatment

1. AED-Benzodiazapines, Sodium valproate, Felbamate, Lamotrigine, Topiramate

2. Ketogenic diet

3. Vagal nerve stimulation

4. Surgical resection

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.

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 hemifacialwithout impairment of consciousness.

Interictal EEG with high-voltage centrotemporalspikes on a normal background.

Favorable outcome with recovery in adolescence.

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

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.

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.

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

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

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.

Childhood onset (peak 4–7 years).

Various generalized and focal seizures.

Cognitive deterioration and behavioraldisturbances.

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.

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

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.

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.

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.

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%

17 m. child with SW induced by opening and closing eyelidsRecurrence of diffuse SW during sleep

Treatment

Treatment of febrile diseases

Avoid hot baths

AED increased during vaccination

Valproate, benzodiazapines

Stiripentol

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

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.

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.

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.

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

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 ceroidlipofuscinoses, sialidosis, and myoclonicepilepsy with ragged-red fibers (MERRF).

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.

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

Thanks

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 PedEmerg Med. 2003;4:195-206.