CHILDHOOD EPILEPSY WITH CENTROTEMPORAL SPIKES

OVERVIEW

Childhood epilepsy with centrotemporal spikes (previously known as benign childhood epilepsy with centrotemporal spikes (BCECTS) or Rolandic epilepsy) is a self-limiting epilepsy seen in children in their early school years. The seizures are brief, hemifacial seizures that may evolve to a focal to bilateral tonic-clonic seizure if they occur nocturnally. This epilepsy occurs in children who are otherwise neurologically and cognitively normal and imaging studies are unremarkable. The EEG shows a normal background with high amplitude centrotemporal sharp waves, which are activated with drowsiness and sleep. Seizures cease by mid to late adolescence.

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

NOTE Childhood epilepsy with centrotemporal spikes, atypical childhood epilepsy with centrotemporal spikes, epileptic encephalopathy with continuous spike-and-wave during sleep and Landau Kleffner syndrome are syndromes that have in common certain EEG features, with variable severity of focal seizures and neurocognitive impairment. They may be considered as a spectrum, an individual child may transition from one of these syndromes to another over time.

CAUTION If cognitive or language deterioration occurs in childhood epilepsy with centrotemporal spikes consider a sleep EEG

Clinical context

This syndrome is characterized by onset of seizures between 3 and 14 years (peak 8-9 years). Seizures 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.

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