Epilepsy Syndromes

 With concomitant EEG findings; seizures

Neonatal Epilepsy

Ohtahara Syndrome Birth Brief tonic seizure, Cortical dysplasia Gene mutations: Pyridoxine Poorly controlled
myoclonic seizures,  ARX Zonisamide seizures
infantile spasm  STXBP1 ACTH Severe
 CDKL5 Prednisolone developmental
 SCN2A delay
 SPTAN1 Shortened life span
Pyridoxine 1st day of life Myoclonic Burst suppression Mutation: Pyridoxine or P5P Mild to severe
Dependent Epilepsy Generalized clonic ALDH71A Poorly responsive intellectual
to other AEDs impairment

Infancy and Early Childhood

West Syndrome 4-9 months Epileptic spasm Hypsarrythmia  Structural brain ACTH Developmental
abnormalities Prednisone delay
 Chromosomal Vigabatrin
disorders Pyridoxine
 Gene
abnormalities
 Metabolic
etiologies
Lennox Gastaut 1-7 years old Tonic (mostly Generalized 1-2 Hz Felbamate
Syndrome nocturnal) slow spike and Clobazam
Atonic wave Rufinamide
Myoclonus Generalized Topiramate
Atypical absence slowing
Generalized Tonic- Paroxysmal fast
Clonic activity during sleep
Childhood

Benign Rolandic 4-10 years old Focal nocturnal Interictal EEG: Self-limited
Epilepsy seizure High amplitude
(Nocturnal tonic- centrotemporal
clonic seizure with spikes & sharp
focal onset) waves with
dramatic activity
Semiology: during sleep
 Drooling
 Dysarthria
 Tingling or clonic
activity of
unilateral face
Childhood Absence 3-10 years Generalized Absence Interictal EEG: Ethosuximide May become
Peak at 6-7 years Seizure provoke by Generalized Valproic Acid Juvenile Myoclonic
Onset before 3 Hyperventilation symmetric 3 hz Epilepsy
years spike
Semiology:
 Staring
 Behavioral arrest
 Unresponsiveness
 Clonic jerks

Duration: Brief, ~ 10
seconds
Juvenile Myoclonic 12-18 years Myoclonic Generalized 4-6 hz Valproic Acid
Epilepsy Generalized Tonic- atypical spike Carbamazepine
Clonic
Absence *Spontaneous
remission
Febrile Seizure o Roseola infantum
o Noninfectious illness
 Most common neurologic emergency  Genetic predisposition:
 Occurs between the age of 6-60 months o Mode of inheritance is more likely polygenic or AD with
 With temperature of 38 C or higher reduced penetrance
 Not the result of CNS infection or any metabolic imbalance  Risk Factors:
 Occur in the absence of history of prior afebrile seizure o Major:
 <1 year
Types: Simple and Complex  Fever <24 hr
 38-39 C
Simple
o Minor:
 1 in 24 hours  Family history of febrile seizure
 Fever in child aged 6 months to 5 years  Family history of epilepsy
 Single seizure is generalized and lasts <15 minutes  Complex febrile seizure
 Day care
 Neurologically healthy without neurological abnormality by
 Male
examination or by developmental history
 Decreased serum Na
Complex
_____________________________________________________________
 Focal or prolonged (>15 minutes) multiple seizures in close
Lumbar Puncture
succession
 Strongly recommended for children <18 months for a first simple
FEBRILE STATUS EPILEPTICUS
febrile seizure.
 Febrile seizure lasting >30 minutes  For those >/= 18 months of age, LP should be performed in the
 Long term epileptic drugs for 2-3 years presence of clinical signs of meningitis
 3 features interact to bring a febrile seizure:
Neuroimaging
o Immature brain
o Fever  Should not be routinely performed
o Genetic disposition
 Causes of fever: Antipyretics
o URTI or pharyngitis
o Otitis media  Used to lower fever and should not be relied upon to prevent
o Pneumonia recurrence
o GE  Control fever and comfort of child
AEDs

 Not recommended for prevention of recurrent simple febrile

seizure

EEG

 Not routinely requested in children with first simple febrile seizure