Background

Status epilepticus (SE) is a common neurological emergency in people with epilepsy. However, the electroclinical phenotype of SE in idiopathic generalized epilepsy (IGE) remains unstudied. In this study, we characterized the prevalence and phenotype through a case series and a scoping review of 40 years of literature (1983-2023).

Research

Eleven patients (2.5%) out of 443 had at least one episode of SE confirmed by electroencephalogram (EEG) at our centre. All the episodes were diagnosed as absence status epilepticus (ASE), either in isolation or associated with generalized tonic-clonic seizure (GTCS). Seventy-three studies including 204 patients were included in the scoping review. 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.

Significance

Absence status epilepticus appears to be the most commonly reported type of status epilepticus in idiopathic generalized epilepsy. The clinical presentation of absence status epilepticus 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 favourable.