Abstracts

Rationale: In Juvenile Myoclonic Epilepsy (JME) cases, 10% of patients are medically refractory [1]. With a subset of antiepileptic drugs (AEDs) indicated for generalized epilepsies, these patients are limited in treatment options.  Moreover, indications are often for generalized tonic-clonic (GTC) seizures without reference to myoclonic and absence seizures that also occur.  These seizures can result in physical injuries and limitations for patients’ quality of life.  Perampanel (PER) is the newest FDA-approved AED with an indication for primary generalized tonic-clonic seizures [2].  This study was undertaken to determine whether PER use in refractory JME patients could affect other types of seizures. Methods: A retrospective review of the Banner University Medical Center - Phoenix Epilepsy Clinic electronic medical records from 2015-2017 was performed for refractory JME cases.  Inclusion criteria were: a diagnosis of JME with electroencephalographic findings consistent with the diagnosis (i.e. polyspike-wave discharges) and treatment failure after 2-3 appropriate AED trials. Exclusion criteria were: patients with concurrent nonepileptic seizures and lack of follow up records from initial PER institution. Responder rates were defined as >50% and 100% improvement from baseline seizure frequency. Results: A total of 7 patients with refractory JME were identified who started on PER.  One patient was unable to tolerate the medication due to side effects (dizziness and nausea).  One was lost to follow up.  Of the remaining 5 patients, the duration of use ranged from 13 to 54 weeks with an average of 37 weeks (SD 15).  Doses ranged from 6 to 12 mg daily with an average of 9 (SD 3).  In 2 patients, PER was taken as monotherapy.  The 3 other patients took 1 other AED (lamotrigine, clonazepam, or levetiracetam).  Three patients had vagal nerve stimulators (VNS) implanted.  Four patients had GTC seizures at baseline.  Frequency ranged from rare ( < 1 per year) to 2 per month. In all 4 patients, no GTC seizures occurred during PER use.  Four patients had myoclonic seizures; all reported multiple daily events prior to starting PER. Afterwards, 3 reported improvement, 1 with infrequent events (1 per 2 weeks).  One patient achieved complete control of events.  Of 2 patients with absence seizures prior to PER, both improved. One continued to have daily events but at a lesser abundance; the other had 1 event per month.  In 1 patient, PER was tolerated but ineffective in controlling myoclonic/absence seizures. Conclusions: Alternative treatments for refractory JME cases, especially for myoclonic events, are limited.  This study shows PER can be an option if other AEDs are ineffective.  It was well tolerated at standard daily doses and as monotherapy (although in all cases, this was not the initial intent). Efficacy in controlling GTC seizures was confirmed, and it was effective in control of myoclonic and absence seizures, resulting in complete control in 1 patient.  This was a small case series; as such multiple confounding factors were not controlled, such as PER titration and target dosing, VNS implantation and parameter adjustments, dosing of adjunctive AEDs, and natural epilepsy progression.  Prospective controlled studies could better elucidate rates of efficacy and characteristics to better predict success. French JA, Krauss GL, Wechsler RT, Wang XF, DiVentura B, Brandt C, Trinka E, O'Brien TJ, Laurenza A, Patten A, Bibbiani F.  Perampanel for tonic-clonic seizures in idiopathic generalized epilepsy A randomized trial. Neurology. 2015 Sep 15;85(11):950-7.Gelisse, P., Genton, P., Thomas, P., Rey, M., Samuelian, J.C., Dravet, C. Clinical factors of drug resistance in juvenile myoclonic epilepsy. J Neurol, Neurosurg Psychiatry. 2001; 70: 240–243 Funding: None