Abstracts

Rationale: Lennox-Gastaut syndrome (LGS) is a difficult to manage childhood-onset epilepsy syndrome characterized by intractable seizures of multiple types, developmental and intellectual impairment, and abnormal EEG patterns including poor background rhythms, multifocal spikes, and interictal slow spike wave discharges. Patients frequently sustain traumatic head injury from falls associated with atonic or myoclonic seizures. The anti-epileptic drug (AED) rufinamide, recently received FDA approval for the adjunctive treatment of seizures in LGS for children 4 years of age and older, and adults. Our goal is to report on our experience with rufinamide, as there are limited studies on its efficacy and tolerability in clinical practice. Methods: A retrospective chart review was performed on patients treated with rufinamide at Stony Brook University Medical Center from 1/01/09 to 6/01/09. Results: Fifteen patients were identified, 10 males, 5 females, ages 4-51 years, with 8 (53%) pediatric and 7 (47%) adults (>16 years old). Mean dose was 1,120 mg/day (range 400 - 2400 mg/day) divided BID. Mean treatment duration at the time of the study was 81 days (range 5-151 days). One-hundred percent of the patients were on polypharmacy, ranging from one to four AEDs, and three had a VNS implanted. Indications for treatment were intractable epilepsy with poor seizure control. Seizure types were generalized tonic-clonic (11), atonic (9), myoclonic (9), partial seizures with secondary generalization (2), atypical absence (1), and juvenile myoclonic epilepsy (1). Eight patients met the criteria for LGS. Four (27%) experienced adverse effects necessitating discontinuation of rufinamide; anorexia/nausea (2), and rash (2). One patient with rash evolved into DRESS (drug rash with eosinophilia and systemic symptoms). The adverse reactions resolved with discontinuation of rufinamide, however the patient with DRESS required high dose prednisone with taper. There were no cases of SUDEP. Seven patients (47%) had improved seizure control during treatment, 4 (27%) discontinued the medication due to adverse reactions, and 4 (27%) showed no improvement. EEGs before and during treatment were either unavailable for comparison or did not reveal unequivocal signs of electrographic improvement. Conclusions: Rufinamide is a relatively well tolerated and effective adjunctive medication for managing intractable epilepsy and seizures in LGS. Further analysis of clinical experience is warranted regarding the use of rufinamide in larger clinical study populations, including other generalized epilepsies, as well as in non-convulsive status.