Efficacy of Felbamate in a Cohort of Patients with Epilepsy with Myoclonic Atonic Seizures (EMAS)
Abstract number :
2.142
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2022
Submission ID :
2204096
Source :
www.aesnet.org
Presentation date :
12/4/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:23 AM
Authors :
Laurel Reed, MD – University of Michigan; Michael Ciliberto, MD – University of Iowa; Katherine Nickels, MD – Mayo Clinic; Eric Kossoff, MD – John's Hopkins; Elaine Wirrell, MD – Mayo Clinic; Charuta Joshi, MBBS – University of Texas Southwestern
Rationale: Epilepsy with myoclonic atonic seizures (EMAS) is a rare childhood onset epileptic encephalopathy which is frequently refractory to medical therapy. Recent efforts to identify the best treatment options have resulted in the identification of the ketogenic diet as the most effective known therapy. The best medication for treatment (before diet usage or after dietary failure) remains unknown.
Methods: Five large pediatric epilepsy centers (University of Colorado, University of Michigan, University of Iowa, John’s Hopkins, and Mayo Clinic) performed a retrospective chart review on patients diagnosed with EMAS at their institutions and collected data on felbamate usage and efficacy. Responders were classified as patients who had a 50% or greater reduction in seizures with a given therapy. Analyses included descriptive statistics with percentiles, means, and medians. Differences between groups were assessed using Fisher’s exact tests and Student’s t-tests.
Results: There were 223 patients with EMAS in the studied cohort, of whom 27 (12%) were treated with felbamate. There were 18 (67%) responders to felbamate, eight of whom (44% of responders) reported seizure freedom. The responder rate was much higher for felbamate than for levetiracetam (15%, p < 0.001) or valproic acid (32%, p = 0.001). The responder rate for felbamate was similar to that seen with the ketogenic diet (69%, p = 0.8). On average patients were tried on 4.9 medications prior to felbamate, with felbamate treatment occurring an average of 37 months after seizure onset. The ketogenic diet was used in 77% of patients prior to use of felbamate. There was no significant difference in time to start felbamate between responders (39 months) and non-responders (34 months) (p=0.57). Felbamate was effective in 4/6 (67%) who were treated prior to the ketogenic diet and 7/20 (65%) who were treated after the ketogenic diet (p = 1).
Clinical Epilepsy