Abstracts

Rationale: Absence epilepsies (AE) are characterized by brief, generalized seizures characterized by behavior arrest and impairment of consciousness.  The typical age range of childhood AE is 4-9 years old and have an excellent prognosis, however, there are rare cases reported of early onset childhood absence epilepsy that are less well described, in particular with a worsening of seizures with addition of valproic acid. Methods: Retrospective case review of patient with absence epilepsy in infancy and associated literature review. Results: This is a case of a 14 month old female who was seen in pediatric epilepsy clinic for recurrent spells of freezing, staring, and falling down, occurring six times per day and lasting one to two seconds.  Previous EEG records had shown 2.5 to 3 Hz generalized spike-wave discharges lasting up to five seconds. She was trialed on levetiracetam with only mild benefit, so patient was admitted to the pediatric epilepsy monitoring unit for further characterization. cEEG showed similar discharges occurring 4-7 times/hour with normal background with associated motor arrest No other seizure semiology was noted including myoclonus. Levetiracetam was switched to valproic acid, however the number of seizures increased to 10 times/hour. Valproic acid was withdrawn slightly and ethosuximide was added, which reduced the frequency to 4 times/hour, and eventually was discharged on ethosuximide alone. The diagnosis was made of a primary generalized epilepsy and classified as early-onset childhood absence epilepsy. Due to refractory nature and worsening with valproic acid a genetics referral was placed which is underway.  In the interim topiramate was added to ethosuxmide with pending results. Conclusions: Absence epilepsy can present in patients prior to the age of 2, and has a broad spectrum of clinical manifestations and prognosis.  Typical absence epilepsy occurs mostly between the age of 4 to 9, with few reports of prior to age 3. Current literature is sparse in this area, with two case reports (2001, 2013) showing worsening response to valproic acid in absence epilepsy. This case shows an early-onset absence epilepsy with an atypical presentation with no response to levetiracetam, paradoxical negative response to valproic acid, and a fair response to ethosuximide. Funding: No funding was received in support of this abstract.