Abstract: Objective: To characterize the phenotypes and prevalence of status epilepticus (SE) in idiopathic generalized epilepsy (IGE) through a case series in an epilepsy center and a literature review.
Methods: In this study, we evaluated a cohort of IGE patients aged 18 years or older with EEG-confirmed SE at Beaumont Hospital between 2005 and 2023. A scoping review was also performed by searching PubMed/Medline and SCOPUS between January 1983 and December 2023.
Results of the case series: Eleven out of 443 adult IGE patients (2.5%) at our center had EEG-confirmed status epilepticus, all presenting as absence status epilepticus (ASE). All patients had a prior diagnosis of epilepsy; four were initially misdiagnosed with focal epilepsy. We identified two clinical presentations: ASE in isolation and associated with a GTCS. Most cases resolved with benzodiazepine therapy.
Results of the scoping review: Seventy-three studies were included, encompassing 204 adult patients who experienced SE. ASE accounted for 84.8% of cases. Common triggers included sodium channel blockers and poor antiseizure medication adherence. Benzodiazepines were the primary first-line treatment for SE, with valproate often used as a second-line option. Despite several studies not including clinical follow-up, around 40% of patients achieved seizure freedom.
Significance: ASE appears to be the most commonly reported type of SE in IGE, yet it is often not suspected clinically. The clinical presentation of ASE is wide, mainly characterized by confusion and altered awareness, and often accompanied by subtle motor and/or psychiatric symptoms. The response to benzodiazepines is generally favorable.
