Pediatric Epilepsy

Question 1

Name all Neonatal onset Epilepsy Syndromes (In utero to 1 month of life)

Answer 1

There are 4 syndromes to know

a. Benign (idiopathic) neonatal seizures (fifth day fits)
b. Benign Familial neonatal epilepsy
c. Ohtahara syndrome (Early Infantile epileptic encephalopathy)
d. Early Myoclonic encephalopathy

Question 2

Benign (idiopathic) neonatal seizures

Answer 2

Benign (idiopathic) neonatal seizures (fifth day fits)
Usual Timeframe: DOL 4-6
Main type of seizure: unifocal clonic, rarely focal tonic
Prognosis: Excellent, usually resolves within 2 days
Differentiating feature: Otherwise healthy with normal neuro exam between fits and no Fhx. Can have Theta Pointu alternant ictal pattern on EEG.

Question 3

Benign Familial neonatal epilepsy

Answer 3

Benign Familial neonatal epilepsy
Usual Time frame:DOL 2-3
Main type of seizure: Hypertonia, apnea, facial movements, Clonus. Can have myoclonus or tonic clonic but less likely.
Prognosis: usually remits by 6 months of age with 10% chance of epilepsy.
Differentiating feature: Family history of neonatal seizures, autosomal dominant. Normal neuro exam between seizures. Can test for KCNQ mutation.

Question 4

Early-onset Epileptic Encephalopathies

Answer 4

1. Ohtahara syndrome (Early Infantile epileptic encephalopathy)
2. Early Myoclonic encephalopathy

Question 5

Ohtahara syndrome

Answer 5

Ohtahara syndrome (Early Infantile epileptic encephalopathy)
Timeframe: First 10 days of life
Main type of seizure: Tonic seizure > myoclonic with persistent burst suppression on EEG
Prognosis: May progress to West Syndrome or Lennox-Gastaut
Differentiating feature: Seizure type and usually has structural cause
Treatment:

Question 6

Early Myoclonic encephalopathy

Answer 6

Early Myoclonic encephalopathy
Timeframe: Neonatal period
Main type of seizure: Myoclonic > tonic with burst suppression on EEG (more with sleep)
Prognosis: May progress to West Syndrome or Lennox-Gastaut
Differentiating feature: Seizure type and usually a metabolic cause.
Treatment:

Question 7

Infantile Onset Epilepsy Syndromes

Answer 7

Infantile Onset (1 month to 1 year).

There are 7

a. Benign Myoclonic Epilepsy in infancy
b. Benign Epilepsy of infancy/Benign familial infantile epilepsy
c. Epilepsy of infancy with migrating focal seizures
d. Hemiconvulsion-hemiplegia-epilepsy syndrome
e. West syndrome (infantile spasms, hypsarrhythmia)
f. Dravet syndrome (severe myoclonic epilepsy of infancy)
g. Myoclonic encephalopathies in non progressive disorders

Question 8

Myoclonic encephalopathies in non progressive disorders

Answer 8

Myoclonic encephalopathies in non progressive disorders
Timeframe: 1 month and 6 years
Main type of seizure: Myoclonic
Prognosis: Progressive decline.
Differentiating feature: Often with Angelman, Prader-Willi, Rett, or Wolf-Hirschhorn syndromes. Neurodevelopmental impaired at baseline.
Treatment:

Question 9

Dravet syndrome (severe myoclonic epilepsy of infancy)

Answer 9

Dravet syndrome (severe myoclonic epilepsy of infancy)
Timeframe: Around 6 months of age
Main type of seizure: Myoclonic but can have anything except tonic.
Prognosis: poor, often medically intractable with cognitive decline and motor deficits.
Differentiating feature: Hemiconvulsive seizure that alternates sides. Very sensitive to higher temps, both fever and ambient.
Treatment: Valproate, clobazam, ketogenic diet. Is caused by a sodium channel mutation so sodium channel active AEMs can make it worse (phenytoin,lamotrigine,carbamazepine, gabapentin,etc)

Question 10

West syndrome

Answer 10

West syndrome (infantile spasms, hypsarrhythmia)
Timeframe: Most common epilepsy syndrome in infancy
Main type of seizure: Myoclonus
Prognosis: poor, high risk of cognitive impairment and Lennox Gastaut.
Differentiating feature: Triad of epileptic spasms, hypsarrhythmia on EEG (chaotic high voltage slow waves), and neurodevelopmental arrest or regression
Treatment: ACTH, Prednisolone, or Vigabatrin

Question 11

Hemiconvulsion-hemiplegia-epilepsy syndrome

Answer 11

Hemiconvulsion-hemiplegia-epilepsy syndrome
Timeframe: 1 month to 4 years
Main type of seizure: Super-Refractory unilateral convulsive seizures (often greater than 24 hours)
Prognosis: Variable based upon response to treatment.
Recently improved with vaccines and status txs.
Differentiating feature: Often triggered by febrile illness, EEG variable. Imaging with edema over involved hemisphere with abnormal white matter and cortex signals.
Treatment:

Question 12

Epilepsy of infancy with migrating focal seizures

Answer 12

Epilepsy of infancy with migrating focal seizures
Timeframe: 1 month to 6 months
Main type of seizure: “Ping pong” migratory seizure (starts at one focus then goes to another as the first stops)
Prognosis: Uniformly Poor
Differentiating feature: Ping pong seizures
Treatment:

Question 13

Benign Epilepsy of infancy/Benign familial infantile epilepsy

Answer 13

Benign Epilepsy of infancy/Benign familial infantile epilepsy
Timeframe: 1 month to 1 year
Main type of seizure: staring, AMS, eye deviation, head turning, apnea, automatisms. Can progress to multifocal or generalized.
Prognosis: excellent with pharmacotherapy. Usually complete remission.
Differentiating feature: neurodevelopmentally normal, autosomal dominant so positive family history.
Treatment:

Question 14

Benign Myoclonic Epilepsy in infancy

Answer 14

Benign Myoclonic Epilepsy in infancy
Initially found by Dravet.
Timeframe: 4 months and 3 years of age
Main type of seizure: Myoclonic seizures triggered by Sensory stimuli.
Characteristic very subtle at onset, head nods, or eye rolling.
Prognosis: Good but some cognitive and behavioral impairments occur
Differentiating feature: Family history of seizure with 3 Hz to 4 Hz generalized spike activity.
Treatment:

Question 15

Childhood Epilepsy Onset Syndromes

Answer 15

Childhood onset (1 year to puberty)

There are 11.

1. Genetic epilepsy with febrile seizures plus
2. Panayiotopoulos syndrome (Early-Onset Childhood Occipital Epilepsy)
3. Doose syndrome (Myoclonic-atonic epilepsy)
4. Benign Rolandic Epilepsy (Benign epilepsy with centrotemporal spikes)
5. Late-onset childhood occipital epilepsy
6. Tassinari syndrome (Epilepsy with myoclonic absences)
7. Lennox-Gastaut syndrome
8. Epileptic encephalopathy with continuous spike and wave in slow sleep
9. Landau-Kleffner syndrome (Acquired epileptic aphasia)
10. Childhood absence epilepsy (pyknolepsy)
11. Jeavons syndrome (Generalized epilepsy with eyelid myoclonia)

Question 16

Genetic epilepsy with febrile seizures plus

Answer 16

Genetic epilepsy with febrile seizures plus
Timeframe: 6 months and 6 years
Main type of seizure: Simple febrile to afebrile, prolonged, focal, and generalized.
Prognosis: Usually responsive to meds and remits by adolescence. Normal development.
Differentiating feature: Need two family members with disease. Autosomal dominant
Treatment: ***

Question 17

Panayiotopoulos syndrome (Early-Onset Childhood Occipital Epilepsy)

Answer 17

Panayiotopoulos syndrome (Early-Onset Childhood Occipital Epilepsy)
Timeframe: Most common Occipital epilepsy in childhood from 3 year to 6 years.
Main type of seizure:**
Prognosis: Usually achieve remission within 2 years of onset.
Differentiating feature: Seizure with recurrent vomiting and eye deviation with onset during sleep. Occipital spikes on EEG in half of patients.
Treatment: **

Question 18

Doose syndrome (Myoclonic-atonic epilepsy)

Answer 18

Doose syndrome (Myoclonic-atonic epilepsy)
Timeframe: 18 months and 5 years, peaks at age 3
Main type of seizure: Large amplitude symmetric jerks of arms, legs, neck and shoulders followed by loss of muscle tone and a fall. Can have all other types of seizure as well.
Prognosis: Usually can achieve remission but if not then intellectual impairment with intractable epilepsy likely.
Differentiating feature: Myoclonus followed by a fall
Treatment: Valproic acid, ethosuximide, benzos, topiramate, lamotrigine, rufinamide, levetiracetam, ketogenic diet. May need a helmet for falls.

Question 19

Benign Rolandic Epilepsy (Benign epilepsy with centrotemporal spikes)

Answer 19

Benign Rolandic Epilepsy (Benign epilepsy with centrotemporal spikes)
Timeframe: 4 to 11 years, peak around 7-8 with male predominance. 25% of all childhood epilepsies.
Main type of seizure: Upon awakening, ipsilateral facial jerking, and excessive drooling, can have one sided paresthesia of tongue or mouth, then dysarthria/gagging. May become generalized rarely.
Prognosis: excellent, usually normal cognition, remission by 14-16 yo.
Differentiating feature: Usually brief seizures that may only occur once in a lifetime. Centrotemporal spikes on EEG, High voltage followed by slow waves (Rolandic spikes, 0.7% of normal kids have these as well)
Treatment: If has recurrent frequent events (daytime secondary generalized seizures) or poor school performance, then consider carbamazepine, levetiracetam, lamotrigine, or valproate.

Question 20

Late-onset childhood occipital epilepsy

Answer 20

Late-onset childhood occipital epilepsy
Timeframe: 8 to 11 years
Main type of seizure: visual hallucinations, eye flutters, eye deviation, vision loss, ocular pain.
Prognosis: Remission in half of patients.
Differentiating feature: EEG with bilateral occipital spike-and-wave discharges with eye closure but disappear with eye opening.
Treatment:

Question 21

Tassinari syndrome (Epilepsy with myoclonic absences)

Answer 21

Tassinari syndrome (Epilepsy with myoclonic absences)
Timeframe: 1- 12 yo but peaks at 7 years with male predominance.
Main type of seizure: absence seizures with clonic activity. Can have generalized tonic-clonic
Prognosis: Usually have cognitive impairment and pharmacoresistant.
Differentiating feature: Clonus with absence, often has a + family history.
Treatment:

Question 22

Lennox-Gastaut syndrome

Answer 22

Lennox-Gastaut syndrome
Timeframe:1-8 yo but peak at 3-5 years with male predominance.
Main type of seizure: Primarily Tonic but multiple generalized seizure types.
Prognosis: Poor, usually not responsive to meds.
Differentiating feature: cognitive impairment with regression, slow background EEG with paroxysmal fast activity during sleep.
Treatment:

Question 23

Epileptic encephalopathy with continuous spike and wave in slow sleep

Answer 23

Epileptic encephalopathy with continuous spike and wave in slow sleep
Timeframe:3-5 years of age
Main type of seizure: Focal motor. Can have Absence, secondary generalized, or atonic.
Prognosis: Remission by adolescence but with persistent neurocognitive sequelae.
Differentiating feature: Electrical status epilepticus in slow sleep on EEG.
Treatment:

Question 24

Landau-Kleffner syndrome (Acquired epileptic aphasia)

Answer 24

Landau-Kleffner syndrome (Acquired epileptic aphasia)
Timeframe: 3 to 7 years
Main type of seizure: Focal and generalized.
Prognosis:
Differentiating feature: Loss of understanding of common words and language regression.
Treatment:

Question 25

Childhood absence epilepsy (pyknolepsy)

Answer 25

Childhood absence epilepsy (pyknolepsy)
Timeframe:4-10 years with peak of 5-7 years. Affects girls more than boys.
Main type of seizure: absence but can have secondary generalized.
Prognosis: Good, 50-75% seizure free with treatment. Lots of learning and social comorbidities.
Differentiating feature: More common in girls. Brought on by hyperventilation. 3 hz spike on EEG. Often has a + family history of absence seizures.
Treatment: Ethosuximide and valproic acid

Question 26

Jeavons syndrome (Generalized epilepsy with eyelid myoclonia)

Answer 26

Jeavons syndrome (Generalized epilepsy with eyelid myoclonia)
Timeframe: Childhood
Main type of seizure: Absence seizure with eyelid blinking and upward eye deviation. Can progress to generalized tonic clonic.
Prognosis: Variable depending on frequency. Long term remission not expected but possible.
Differentiating feature:
Treatment: Photoreflexsive so blue colored polarized lenses can help, otherwise generalized seizure meds like valproic acid, ethosuximide, and benzos.

Question 27

Adolescent to Adult Epilepsy Syndromes

Answer 27

Adolescence to Adult Onset

There are 7