Abstracts

Rationale: We present two patients with newly diagnosed Juvenile Absence Epilepsy (JAE) who demonstrated rapid electroclinical response to low dose initial monotherapy treatment with levetiracetam (LEV). While LEV is commonly used to treat Childhood Absence Epilepsy (CAE) and JAE, there is only moderate evidence to date regarding its efficacy. JAE is distinguished from CAE by its later peak age of onset of 10 to 12 years with a higher incidence of myoclonic seizures and generalized convulsions. Ethosuximide (ESM) is unfavorable for JAE due to its lack of efficacy in treating convulsive seizures. Valproic Acid (VPA) is highly effective but has an unfavorable side effect profile including attentional deficits, weight gain, liver toxicity and teratogenicity. Lamotrigine (LTG) is less efficacious than ESM and VPA and is associated with severe immune mediated reactions such as hypersensitivity rash. Methods: We report prospective data collected from 2 patients with a newly made clinical diagnosis of JAE following video electroencephalographic (EEG) monitoring for staring spells. Patient 1 is a 14-year-old boy admitted after an unwitnessed collapse, unresponsiveness and confusion, with a several year history of staring spells with behavioral arrest. Patient 2 is a 15-year-old girl evaluated for paroxysmal spells of staring with altered awareness that began at age 5, increasing in frequency to several times daily over the past 3 years. Both patients were started on LEV after several absences were captured with electroclinical correlation on EEG. Results: Pre-treatment EEG monitoring of Patient 1 demonstrated frequent symmetric, synchronized, high amplitude, generalized spike, polyspike, slow-wave discharges of 3.5-4 Hz that were bilateral frontally predominant, lasting between 5 to 15 seconds. There was a significant ictal burden of up to 14 ictal events per hour (Table 1). Patient 2 also demonstrated generalized spike and polyspike slow wave activity of 4-5 Hz, correlating with numerous events of altered awareness and automatisms (Table 1). Following administration of 20 mg/kg oral dose of LEV in two divided doses, within 12 hours the EEG demonstrated complete resolution of clinical ictal events, with significant and rapid reduction of spike/seizure burden during waking EEG background. (Table 1) Conclusions: We believe that levetiracetam may have a role in the treatment of a subset of late onset CAE and JAE. LEV should be considered as an initial monotherapy with guidance from video EEG monitoring in JAE. Responders to LEV are likely to require relatively low dose, reveal polyspikes in their EEGs and may show rapid reduction of spike/seizure burden.¹ Current studies have only examined LEV in both CAE and JAE including one RCT showing a 75 % response rate in 4 JAE patients,² with additional case reports and open label studies also reporting favorable response^3,4 (Table 2). A RCT comparing LEV to other anti-epileptic drugs is warranted to establish the efficacy and sustained seizure freedom rates of LEV in JAE.References1. Nolan D, Shellhaas R, Lester S, Rau S. Clinical use and efficacy of levetiracetam for childhood and juvenile absence epilepsies. American Epilepsy Society Annual Meeting Abstract 2017 1.1822. Fattore C, Boniver C, Capovilla G, et al. A multicenter, randomized, placebo-controlled trial of levetiracetam in children and adolescents with newly diagnosed absence epilepsy. Epilepsia. 2011;52(4):802–809.3. Verrotti A, Cerminara C, Domizio S, Mohn A, Franzoni E, Coppola G, et al. Levetiracetam in absence epilepsy. Dev Med Child Neurol. 2008;50:850-8534. Cavitt J, Privitera M. Levetiracetam induces a rapid and sustained reduction of generalized spike-wave and clinical absence. Arch Neurol. 2004;61(10):1604–1607  Funding: Not applicable