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  • Updated 11.11.2020
  • Released 10.18.1993
  • Expires For CME 11.11.2023

Self-limited epilepsy with centrotemporal spike

Introduction

Overview

Self-limited epilepsy with centrotemporal spikes (SLECTS) is the new name for the previous epilepsy syndrome of benign childhood epilepsy with centrotemporal spikes (BECTS). It was first reported in the 1950s and is now recognized as the most frequent epilepsy syndrome in children between the ages of 4 and 13 years. The term “benign” was applied to this syndrome to differentiate from other sinister causes for focal epilepsies. However, it was noted subsequently that cognitive and language problems and atypical evolutions with a not-so-benign course were seen in this syndrome. As per the recent epilepsy classification, the new term “self-limited” has replaced the term “benign”. The common idiopathic focal epilepsies of childhood are increasingly being termed “self-limited focal epilepsies of childhood” (SFEC) and at times, the word “benign” is completely omitted to name the syndrome as “childhood epilepsy with centrotemporal spikes” (CECTS). For the purpose of this review, we are using the term self-limited epilepsy with centrotemporal spikes (SLECTS) for the typical cases to maintain uniformity. Atypical varieties of the syndrome and atypical evolutions after a seemingly classical onset are also discussed separately. Emerging data about the academic performance and attention impairment in children with this syndrome are also discussed.

Key points

• Typical seizures seen in SLECTS may occur without centrotemporal spikes. Conversely, centrotemporal spikes may occur in children without seizures, in children with other epilepsy syndromes, and in children with structural lesions in the rolandic area.

• An increasing frequency of specific cognitive, language, memory, and attention-related disorders are being reported in children with SLECTS.

• Atypical features in clinical and electroencephalographic manifestations seem to correlate with transitory cognitive and language disorders.

• Very rarely SLECTS may evolve into Landau-Kleffner syndrome and continuous spike-and-waves during slow sleep syndrome with potential for persistent neuropsychological impairments.

• In cases presenting with the atypical features, especially early onset of the disease and frequent discharges in electroencephalogram (EEG) during sleep, initial treatment with benzodiazepines or sulthiame is recommended.

Historical note and terminology

Rolandic (centrotemporal) region is named after Luigi Rolando (1773-1831), an Italian anatomist known for his pioneer research into the localization of function in the human brain. A particular EEG pattern with migratory spikes originating over the rolandic region was first reported in the 1950s (79; 83). In 1958, the first clinical description associated with EEG features was published (147). The same EEG pattern was later correlated with a common form of focal childhood epilepsy, then called "midtemporal epilepsy," characterized by hemifacial and oropharyngeal ictal symptoms and a favorable prognosis (82). Because of the localization of the ictal events, Lombroso proposed the term "sylvian seizures" (135). In the same year, Loiseau and colleagues presented an electroclinical series of 122 children with what they called “a particular form of epilepsy in childhood,” stressing its benign character. Several long-term follow-up studies confirmed the good prognosis (13; 121; 134). This form of epilepsy was called “benign childhood epilepsy with centrotemporal spikes” and was placed in the group of idiopathic localization-related (focal, local, partial) epilepsies in the International Classification of Epilepsies and Epileptic Syndromes (36; 61). As new syndromes were recognized within the spectrum of benign childhood focal epilepsies (159), the term “benign” was considered initially acceptable for this syndrome but was considered inappropriate for some of the other idiopathic focal epilepsies (87).

However, the new ILAE Classification of the Epilepsies proposed the term “self-limited” for “benign” course of epilepsies to reflect a “likely spontaneous resolution of a syndrome” (187). Hence, a new term “self-limited epilepsy with centrotemporal spike” (SLECTS) has been proposed for BCECTS (187). The common idiopathic focal epilepsies of childhood are increasingly being termed “self-limited focal epilepsies of childhood” (SFEC) and at times, the word “benign” is completely omitted to name the syndrome as “childhood epilepsy with centrotemporal spikes” (CECTS) (204).

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