SELF-LIMITED EPILEPSY WITH CENTROTEMPORAL SPIKES (SeLECTS)

OVERVIEW

Self-limited epilepsy with centrotemporal spikes (previously known as benign childhood epilepsy with centrotemporal spikes (BCECTS) or rolandic epilepsy) is a self-limited epilepsy syndrome that begins in an otherwise normal child in their early school years. Seizures are brief bulbar and hemifacial sensorimotor seizures that can evolve to focal to bilateral tonic-clonic seizures. The EEG shows a normal background with characteristic high amplitude centrotemporal epileptiform abnormality, activated with drowsiness and sleep. Seizures cease by puberty.

NOTE Self-limited refers to there being a high likelihood of seizures spontaneously remitting at a predictable age.

NOTE Self-limited epilepsy with centrotemporal spikes and developmental and/or epileptic encephalopathy with spike-wave activation in sleep are considered conditions that exist on a spectrum, an individual child may transition from one syndrome to another over time.

CAUTION If cognitive or language deterioration occurs in this syndrome consider a sleep EEG (assess for developmental and/or epileptic encephalopathy with spike-wave activation in sleep)

Clinical context

This syndrome is common, accounting for 6-7% of all childhood epilepsy and is characterized by onset of seizures between 4-10 years (range 3-14 years). Both sexes are affected with a slight male predominance. Antecedent, birth, neonatal and developmental history is typically normal. Some children have prior attention-deficit/hyperactivity disorder or specific cognitive impairments. Neurological exam and head size is normal. A history of febrile seizure (in 5-15%) or rarely a history of self-limited epilepsy with autonomic seizures may be present. During the course of the active epilepsy, behavioral and neuropsychological deficits may emerge or worsen, particularly in language and executive functioning. These deficits often improve with age. Seizures typically respond to medication and usually resolve by puberty, but can occasionally occur up to 18 years of age.

CAUTION If the patient is male with intellectual impairment consider Fragile X (especially if background slowing is present on EEG)