Abstracts

Rationale: Syndromic and non-syndromic generalized epilepsies are common in children and include absence, myoclonic and generalized seizures. The aim of this case series is to study the efficacy and effectiveness of PER among children with refractory generalized epilepsy.

Methods: A case series.

Results: Case 1: A 7-year-old girl with history of platelet storage pool disease with epilepsy onset at age 5 years. Patient has dialeptic seizures, with behavior arrest, staring into space, upward eye deviation, and unresponsiveness lasting 6-16 sec with frequency of 6 seizures/ hr, and rare generalized tonic clonic seizures. Interictal EEG shows 3 Hz generalized spike and wave (GSW), generalized polyspike and OIRDA (Fig 1). Her MRI brain, genetic and metabolic workup were unremarkable. She failed Ethosuximide (ESM), clobazam (CLB), valproic acid (VPA), levetiracetam (LEV), Amantadine and Acetazolamide. She was seizure free for 9 months after adding PER (4 mg/d) followed by relapse in seizures (Table 1) with no benefit of dose increase after the relapse.

Case 2: An 8-year-old girl born at 30 weeks gestation with epilepsy onset at age 8 year. She presented with dialeptic seizures occurring 17-18 times/hr with staring and unresponsiveness; and infrequent focal seizure with impaired awareness with staring, drooling, slow gaze and face movement to the right side. These seizures lasted 10 to 25 seconds. Interictal EEG displayed 3 Hz GSW, polyspikes, left posterior quadrant rhythmic slowing and left hemisphere paroxysmal fast activity. Ictal EEG exhibited 3 Hz GSW complex and left focal seizure with bilateral synchrony (Fig 1). Her MRI brain was normal. She failed treatment on ESM and LVT and became seizure free on addition of PER with no further seizure in the last 10 months. Subsequent EEGs became normal using PER as an adjunct therapy to LEV however using PER alone showed rare epileptic activity.

Case 3: An 18-year-old girl with developmental delay and epilepsy onset at age 14 years. Her seizure semiology was behavior arrest, staring into space and oral automatism lasting 2-9 sec, 20-30 times/hr. Interictal EEG showed bilateral fronto-central and left frontal epileptic discharges, while ictal EEG exhibited 3 Hz GSW (Fig 1). She failed Lamotrigine and ESM and became seizure free on addition of PER with no further seizures for 6 months (Table 1).

PER was incremented to 4 mg/d in all 3 patients and seizure freedom was achieved within one month.

A 6 months seizure freedom was seen with addition of PER as an adjunctive therapy in all 3 cases of refractory absence-like epilepsies with exception of one case with rebound seizures after 9 months. These patients had almost normal to near-normal EEG findings after starting PER.

Conclusions: PER can be used as an adjunct therapy in children with refractory generalized epilepsies to achieve seizure free state as seen in two of our cases and considerable improvement in the first patient with absence epilepsy. Specific improvement in generalized spike and wave discharges and ictal patterns is consistent with a robust clinical and electrographic response.

Funding: Please list any funding that was received in support of this abstract.: None.