11-08 Pedi Epilepsy

Question 1

infantile spasms

Answer 1

—Present <1 y/o
—multiple sz types (flexion, extension, head drops, mixed)
—sz come in clusters/volleys w/ irritability between
—assoc w/ intellectual disabilities and PKU, tuberous sclerosis, Aicardi syndrome, trisomy 21, pyridoxine dependency, many others
—EEG: flattening w/ sz
——TX: ACTH/prednisone

Question 2

Lennox-Gastaut syndrome

Answer 2

difficult-to-treat form of childhood-onset epilepsy that most often appears between the second and sixth year of life, and is characterized by frequent seizures and different seizure types; it is often accompanied by developmental delay and psychological and behavioral problems. [WIKI]

Question 3

Benign Rolandic Epilepsy

Answer 3

—common
—simple partial sz during sleep —> wake up —> can 2°ly generalize
—autosomal dominant w/ incomplete penetrance (some relatives of young age
—typically occur just during sleep
—usu. not treated
—high association with learning disabilities (can’t remember what you learned that day

Question 4

West Syndrome

Answer 4

—infantile spasms
—EEG showing “hypsarrythmia”
—developmental delay

Question 5

Febrile szs

Answer 5

—really common; 5% of kids
—presents 6mos-6yrs
—not actually epilepsy
—treat w/ benzos (not phenobarb as before b/c of neurotoxic effect on developing brain)

Question 6

Absence

Answer 6

—Rare
—Generalized
—3Hz spike waves
—usually 
—frontal lobe affected —> learning disability

Question 7

Dravet Syndrome

Answer 7

—A severe form of myoclonic epilepsy of infancy, often associated with intractable epilepsy and poor psychomotor developmental outcome
—A mortality risk in early life as high as 10 percent has been reported
— not much luck w/ tx; DON’T use carbamazepin or lamotrigine