Oligoepilepsy and lifelong seizure susceptibility in epilepsy with generalized tonic-clonic seizures alone: experience at an adult tertiary center

Objective Epilepsy with generalized tonic-clonic seizures alone (GTCA) is the least studied syndrome within the idiopathic generalized epilepsy (IGE) spectrum. We characterize a large cohort of adult patients with GTCA to understand natural history and drug responsiveness. Methods In this retrospective single-center study using our epilepsy electronic record, we evaluated clinical characteristics, seizure outcomes, anti-seizure medication (ASM) response including seizure recurrence after ASM withdrawal, and sex differences in a cohort of GTCA patients aged ≥17 years. Results Within a cohort of 434 IGE patients, 87 patients (20%) with GTCA were included. The mean age was 34.9 years (range 17-73 years). Forty-six patients (52.8%) were females. Seventy-two patients (82.8%) were seizure-free and 15 (17.2%) had active epilepsy over the previous 12 months. Thirty-four patients (39.1%) had ≤5 lifetime seizures, aligning with a prior definition of ‘oligoepilepsy’. Sixty-five patients (74.7%) were treated with monotherapy, 19 (21.8%) were treated with polytherapy, and three were not taking any ASM. Levetiracetam (37.9%) was the most commonly prescribed ASM, followed by lamotrigine (32.1%) and valproate (31%). Seventeen patients (19.5%) attempted to withdraw their ASM. The rate of seizure recurrence after ASM withdrawal was 88.2% (15/17), including two patients who relapsed more than 20 years after ASM discontinuation. Females had more seizures in their lifetime and had trialed more ASM compared to males. Significance GTCA has a relatively good prognosis, with most patients becoming seizure-free on monotherapy. The high rate of seizure recurrence after ASM withdrawal supports lifetime seizure susceptibility. We found potential sex differences in seizure outcomes and ASM response, although further research is needed to validate this finding.