/self-limited epilepsy with centrotemporal spikes

self-limited epilepsy with centrotemporal spikes

Rare Disease

MONDO:0007295

Also Known As

BCECTSBECRSBECTSBRERolandic epilepsybenign Rolandic epilepsybenign Rolandic epilepsy (BRE)benign Rolandic epilepsy of childhood (BREC)benign childhood epilepsy with centrotemporal spikebenign childhood epilepsy with centrotemporal spikesbenign epilepsy of childhood with centrotemporal spikesbenign epilepsy with centrotemporal spikesbenign familial epilepsy of childhood with rolandic spikescentralopathic epilepsycentrotemporal epilepsycentrotemporal epilepsy, isolated caseschildhood epilepsy with centrotemporal spikessylvan seizurestemporal-central focal epilepsybenign epilepsy of childhood with centrotemporal spikes (BECCT)benign epilepsy with centro-temporal spikes (BECTS)

Definition

A childhood-onset epilepsy syndrome that is characterized by onset of seizures between 3 and 14 years (peak 8-9 years) that usually resolve by age 13 years, but can occasionally occur up to age 18 years of age. Both sexes are affected. Antecedent, birth and neonatal history is normal. A history of febrile seizure (in 5-15%) may be seen. A history of Panayiotopoulos syndrome may be present in a very small number of cases. Neurological exam and head size is normal. Development and cognition prior to onset of seizures is normal. During the course of the active epilepsy, behavioral and neuropsychological deficits may be found, particularly in language and executive functioning. These deficits improve when seizures remit.