Abstracts

Rationale: Lennox-Gastaut Syndrome (LGS) is a severe epilepsy, characterized by multiple seizure types, cognitive impairment, an abnormal electroencephalogram patterns. Seizures associated with LGS can be quite refractory to various medications and often require multiple medications to achieve meaning seizure control.  Although perampanel (PER) does not have specific indication for LGS in the United States, it has been used in LGS patients as an additional therapeutic agent in patients with refractory LGS. With its novel mechanism of non-competitive selective AMPA receptor antagonist, PER may be a useful in adjunctive therapy for various seizures types associated with LGS.  Methods: We retrospectively evaluated patients with LGS diagnosis who had exposure to PER as adjunctive treatment in order to characterize the response to PER within our patient population. Existing medical records were reviewed to obtain information regarding age, sex, weight change, current AED use at time of PER initiation, prior AED use, length of PER treatment, reported adverse effects and reasons for discontinuation.  Results: A total of 7 patients were identified for the purposes of this study. Patients were aged 20 to 58 years old (median age was 32) and 57.1% were female. The majority of patients had tried more than 5 AEDs prior to PER exposure. At the time of PER initiation, the average number of concomitant AEDs was 4 with Keppra and Onfi being the two most AEDs. PER was initiated at 2 mg/day in all patients which then titrated to effect. Three out of 7 patients reported noticeable or meaningful improvement in seizure frequency after taking PER. Retention rate at 6 months was 42.9%. Adverse effects leading to discontinuing PER included behavioral changes (mood swings and anger outbursts), weight gain, ataxia with associated falls, headache, dizziness, nausea and sedation. Five patients over the course of PER therapy reported weight gain of 4.6 kg.  Conclusions: When PER was used in patients with refractory LGS, less than half of patients (42.9%) had reported improvement in seizure control. Many of patients eventually discontinued PER due to inefficacy or various adverse effects in LSG population. Larger study is needed to evaluate the effectiveness of PER in patients with LGS. Funding: None