Pediatric epilepsy

Question 1

Simple febrile seizures

Answer 1

6 months to 5 years
Generalized
<15 minutes
Occurs only once in 24 hours

Question 2

Complex febrile seizures

Answer 2

Focal features
>15 minutes
>1/24 hours

Question 3

Neonatal seizures

Answer 3

Neonatal period = < 1 month old
Etiology of neonatal seizures:
- 60% Hypoxic-ischemic injury
- 20% Hemorrhage or infarct
- 10% Infection
- 10% Other
Syndromes: Benign idiopathic neonatal convulsions, Early infantile epileptic encephalopathy*

Question 4

Benign Idiopathic Neonatal Convulsions
AKA “5th day fits”

Answer 4

Age: 1st week
Seizures: Focal clonic, focal tonic, a/w apnea
Normal exam between seizures
Normal inter-ictal EEG
Prognosis: Excellent, most outgrow seizures
Familial form: K+ channel mutation (KCNQ2/3), AD

Question 5

Early Infantile Epileptic Encephalopathy
AKA Ohtahara syndrome

Answer 5

Age: 1st month
Seizures: Generalized (tonic spasms) and focal
Exam: Encephalopathic
EEG: Burst suppression

Many etiologies
Overall poor prognosis: Ohtahara->West->LGS

Question 6

Infantile seizures

Answer 6

Infant = 1-12 mo
Infantile spasms
Dravet syndrome

Question 7

Infantile spasms
AKA West syndrome

Answer 7

Age: 3-12 months
Seizures: Infantile spasms (flexion/extension of neck, trunk, arms)
EEG: Hypsarrhythmia
Etiology: Many, including structural, metabolic, genetic.
- Most common identifiable etiology: Tuberous Sclerosis
- 1/3 idiopathic
Development: Delay, sometimes profound
Treatment: ACTH or vigabatrin (better for TS)
- early Tx improves long-term developmental outcomes

Question 8

Dravet syndrome

Answer 8

Age of onset: 5-8 months
Seizures: hemiclonic or generalized → multiple seizure types (generalized and focal)
Development: Normal development until seizures begin, after which development stalls or regresses. Autistic features common.
Exam: Hypotonia, ataxia, pyramidal signs
EEG: Slow, disorganized BG with epileptiform abnormalities (NB: can be normal at onset)
Etiology: Genetic (SCN1A), 20-30% idiopathic
Avoid Na-blockade

Question 9

Childhood seizures

Answer 9

Childhood = 1-10 years
Absence epilepsy*
Lennox Gastaut syndrome*
Benign rolandic epilepsy*
Febrile seizures
GEFS+
Benign occipital epilepsy (Panayiotopoulos)*
Acquired epileptic aphasia (Landau-Kleffner)
Rasmussen Encephalitis

Question 10

Myoclonic-Astatic Epilepsy (Doose)

Answer 10

Age: 6 months - 6 years
Seizure: Multiple (drop attacks, myoclonic, myoclonic astatic, generalized tonic-clonic)
Development: Often have developmental delay
EEG: Slowing, generalized discharges

Distinguish from Dravet
Dravet can have focal seizures
Drop attacks are more specific for Doose

Question 11

CAE

Answer 11

Age: 4-10 years
If younger: T/c GLUT1 deficiency
If older: T/c JME or JAE
Seizures: Staring, +/- automatisms (no aura, no post-ictal)
may occur in isolation or with other seizure types
If other types (i.e., GTCs) prominent, t/c JME/JAE
Development: Normal
Treatment: Ethosuximide – T type calcium channels
(but doesn’t treat other sz types)
-Other broad-spectrum AED’s: valproic acid (good for GTCs)
-Worsened by carbamazepine – absence status (RITE 2021)

The EEG pattern in CAE is an approximate 3-Hz bilateral synchronous, symmetric spike and slow wave,

Question 12

LGS

Answer 12

Dev: Intellectual disability
Sem: Multiple seizure types - tonic, atonic, tonic-clonic, absence, drop attacks,
EEG: Generalized slow (1.5-2 Hz) spike and wave
Etiology: Many, with identifiable cause in ~60%
Treatment: Broad spectrum
-CBZ can increase drop attacks

Question 13

Benign Epilepsy with Centro-Temporal Spikes
AKA Benign Rolandic Epilepsy

Answer 13

Seizures: Facial twitching → +/- secondary GTC, classically at night or upon awakening
EEG: Unilateral/bilateral centrotemporal spikes, increase with sleep
Prognosis: Resolves spontaneously within 2 years
Treatment: Not required (OXC if seizures persist)

Question 14

Generalized Epilepsy with Febrile Seizures plus (GEFS+)

Answer 14

• Recurrent seizures provoked by fever
• Age 6+; focal and generalized; prolonged >15 min
• Family history
• Na+ channel mutation (SCN1A, SCN1B)

Question 15

Benign Occipital Epilepsy (PanayiOtOpOulOs syndrome)

Answer 15

Seizures: AutOnomic, pale, flaccid, unresponsive, vOmiting
Typically nocturnal
Last >5 minutes
EEG: Occipital sharp waves (ictal can have varied abnormalities)

Compared to Benign Epilepsy with Centro-Temporal Spikes:
- Occipital not centro-temporal spikes
- Vomiting
- Length of episodes (>5 minutes)

Question 16

Acquired epileptic aphasia (Landau-Kleffner)/Epileptic encephalopathy with continuous spikes and waves during sleep (CSWS)

Answer 16

Question 17

Rasmussen Encephalitis

Answer 17

Age: 14 months - 14 years
Seizure: Frequent focal seizures → epilepsia partialis continua → neurologic deficits (hemiplegia + cognitive decline)
MRI: Unilateral cerebral atrophy
EEG: Unilateral slowing and spikes
Tx: Hemispherectomy/otomy

Question 18

Adolescent seizures

Answer 18

Adolescence: >10 yo
JME
Nocturnal frontal lobe epilepsy

Question 19

JME

Answer 19

Age: Adolescence
Myoclonic jerks upon awakening
Seizure: Generalized (absence, GTC), myoclonus
→ Provoked by sleep deprivation, alcohol
EEG: Bursts of fast (4-6 Hz) generalized polyspike-wave discharges
→ Abnormalities provoked by photic stimulation
Treatment: Depakote, lamotrigine, levetiracetam → other broad spectrum
→ Pregnancy: Lamotrigine
→ Lifelong treatment
→ PHT + CBZ worsen myoclonus

Question 20

AD Nocturnal Frontal Lobe Epilepsy

Answer 20

Seizures: Thrashing and complex movements during sleep associated with startle/arousal, vocalizations

DDx: Parasomnia, PNEE

Associated Gene: Mutation in Nicotinic ACh receptor