Generalized epilepsy with febrile seizures-plus context (GEFS+) refers to a rare heterogeneous familial condition, which has been recently described. Its prevalence is not yet established. Patients affected with GEFS+ display febrile seizures persisting sometimes beyond the age of 6 years and/or a variety of afebrile seizure types. Disease course and response to antiepileptic treatment show an important intrafamilial variation: in some patients, afebrile seizures may either occur rarely or disappear after some years of disease evolution, whereas epilepsy is severe and pharmacoresistant in other members of the same family. GEFS+ is transmitted as an autosomal dominant trait with incomplete penetrance (70-80%) and is associated with genetic heterogeneity. GEFS+ belongs to the group of channelopathies, as it is associated with mutations in genes encoding different subunits of the neuronal voltage-gated sodium channel and the gamma-2 subunit of the GABAA receptor.
Last update: March 2004
- Dr Isabelle GOURFINKEL-AN