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Dr. Parag Moon
Senior resident,
Dept. of Neurology
GMC Kota.
 Definition:
 An epileptic disorder characterized by a
cluster of signs and symptoms customarily
occurring together; the...
 Neonatal period
 Benign familial neonatal epilepsy (BFNE)
 Early myoclonic encephalopathy (EME)
 Ohtahara syndrome
 ...
 Childhood
 Febrile seizures plus (FS+) (can start in infancy)
 Panayiotopoulos syndrome
 Epilepsy with myoclonic aton...
 Adolescence–adult
 Juvenile absence epilepsy (JAE)
 Juvenile myoclonic epilepsy (JME)
 Epilepsy with generalized toni...
 Onset days 2–15, commonly in first week.
 Family history, autosomal dominant inheritance.
 Mutations of potassium chan...
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 ...
EEG showing diffuse suppression burst pattern
 Early infantile epileptic encephalopathy with
suppression bursts
 Onset of tonic spasms before 3 months (usually first
...
 Malignant migrating partial epilepsy in infancy
 Onset 6 months.
 Clusters of severe, polymorphous focal seizures,
fre...
 Onset before 1 year, peak 4–7 months.
 Clusters of spasms.
 Spasms are myoclonic-tonic contractions and
can be either ...
EEG showing hypsarrythmia
 Onset 3–20 months.
 Clusters of brief partial seizures.
 Normal development before onset.
 Responsive to antiepilepti...
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 GC...
 Previously called early-benign childhood seizures
with occipital spikes
 Childhood onset (peak 5 years).
 Focal autono...
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 ...
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, ab...
 More common in males
 Peak age of onset between3-5yrs
 More frequent during sleep
 Two thirds to three-fourths-second...
 Tonic seizures-most characteristic
 Occur during non REM sleep for avg.10 secs
 Axial subtype-B/L symmetrical contract...
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 hyperventilati...
 Predictors of Prognosis
1. Age of onset
2. Frequency of tonic seizures
3. Repeated episodes of non convulsive status
4. ...
 Treatment
 Refractory to treatment
1. AED-Benzodiazapines, Sodium valproate,
Felbamate, Lamotrigine, Topiramate
2. Keto...
 Onset 5mth to 5yrs.
 May be preceded with febrile convulsion.
 Myoclonus may be axial or generalised.
 Myoclonus incr...
 Benign rolandic epilepsy or sylvian epilepsy
 Onset between 2 and 13 years (peak 9–10 years).
 Normal development befo...
EEG during drowsiness and sleep showing frequent bilateral
synchronous/independent biphasic spikes followed by slow waves ...
 Childhood/adolescent onset (mean 11 years).
 Autosomal dominant inheritance due to nicotinic
acetylcholine receptor
 (...
 Formerly grouped with Panayiotopoulos
syndrome as childhood occipital epilepsy, late
onset Gastaut type
 Childhood onse...
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
conscious...
 Ictal EEG showing bilateral, synchronous
spike and slow-wave complexes at 3 Hz
associated with myoclonus.
 Interictal E...
 Childhood onset (peak 4–7 years).
 Various generalized and focal seizures.
 Cognitive deterioration and behavioral
dis...
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
...
 Severe myoclonic epilepsy or severe
polymorphic epilepsy of infants.
 Onset at 2-12months of age.
 Early appearance of...
 Massive myoclonias
 Axial muscles-> falls
 Predominate on awakening
 Precipitated by variation in ambient light
inten...
 Atypical absence-40%
 Focal seizures-1/2 to 3/4th
 Initial development normal, progress slows
down in second and third...
17 m. child with SW induced by opening and closing eyelids
Recurrence of diffuse SW during sleep
 Treatment
 Treatment of febrile diseases
 Avoid hot baths
 AED increased during vaccination
 Valproate, benzodiazapi...
 Onset between 3 and 8 years (peak 5–7 years).
 Acquired aphasia (verbal auditory agnosia).
 Continuous spike and wave ...
 Also called “pyknolepsy”
 Onset between 4 and 10 years in a previously
healthy child.
 Frequent typical absence seizur...
 Onset 7–17 yrs (peak 10–12 yrs) in previously
healthy child.
 Typical absence seizures.
 Secondary seizure type: GTCS....
 Onset 8 to 26 years (peak 12–18, mean 14
years).
 Bilateral myoclonic jerks, most frequently
upon awakening.
 Secondar...
 Severe myoclonias.
 Epilepsy with generalized seizures, especially tonic-clonic,
clonic-tonic-clonic, and clonic.
 Pro...
 Lafora disease presents at a similar age
 Has a severe prognosis
 Rapid progression to dementia and nearly
constant my...
 Onset 1-14 yrs of age
 Focal onset motor seizures: simple partial or
evolve into complex partial or secondary
generalis...
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;Thir...
Pediatric epilepsy syndromes
Pediatric epilepsy syndromes
Pediatric epilepsy syndromes
Pediatric epilepsy syndromes
Pediatric epilepsy syndromes
Pediatric epilepsy syndromes
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Pediatric epilepsy syndromes

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Pediatric epilepsy syndromes

  1. 1. Dr. Parag Moon Senior resident, Dept. of Neurology GMC Kota.
  2. 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. 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. 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. 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. 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. 7. EEG showing focal or multifocal sharp waves or “theta pointu alternant” pattern
  8. 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. 9. EEG showing diffuse suppression burst pattern
  10. 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.
  11. 11.  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
  12. 12.  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.
  13. 13. EEG showing hypsarrythmia
  14. 14.  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
  15. 15. Ictal EEG showing seizure onset starting at O1, spreading to Cz and C3
  16. 16.  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.
  17. 17.  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
  18. 18. EEG showing clone-like repetitive occipital spike-wave discharges
  19. 19.  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.
  20. 20. Interictal EEG showing 4-7 Hz spike and slow wave pattern
  21. 21.  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.
  22. 22.  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.
  23. 23.  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
  24. 24. Discharge of high amplitude fast rhythms lasting for about 10 secs followed by Polyspikes and spike and wave complex
  25. 25. EEG showing spike-wave of 2.5 hz or less.
  26. 26.  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.
  27. 27.  Predictors of Prognosis 1. Age of onset 2. Frequency of tonic seizures 3. Repeated episodes of non convulsive status 4. Constant slow EEG background.
  28. 28.  Treatment  Refractory to treatment 1. AED-Benzodiazapines, Sodium valproate, Felbamate, Lamotrigine, Topiramate 2. Ketogenic diet 3. Vagal nerve stimulation 4. Surgical resection
  29. 29.  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.
  30. 30.  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.
  31. 31. EEG during drowsiness and sleep showing frequent bilateral synchronous/independent biphasic spikes followed by slow waves in the centro-temporal regions.
  32. 32.  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.
  33. 33.  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.
  34. 34. EEG showing B/L occipital spike and wave complexes with right dominance.
  35. 35.  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
  36. 36.  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.
  37. 37.  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.
  38. 38. EEG showing continuous spike and-wave during sleep (CSWS) or electrical status epilepticus of slow-wave sleep.
  39. 39.  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.
  40. 40.  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.
  41. 41.  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.
  42. 42.  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%
  43. 43. 17 m. child with SW induced by opening and closing eyelids Recurrence of diffuse SW during sleep
  44. 44.  Treatment  Treatment of febrile diseases  Avoid hot baths  AED increased during vaccination  Valproate, benzodiazapines  Stiripentol
  45. 45.  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
  46. 46.  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.
  47. 47.  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.
  48. 48.  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.
  49. 49.  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
  50. 50.  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).
  51. 51.  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.
  52. 52. Ictal EEG showing nearly continuous sharp waves and spikes in the left frontal-temporal region
  53. 53. Thanks
  54. 54.  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.

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