Sign Up for a Free Account
  • Updated 03.23.2022
  • Released 10.18.1993
  • Expires For CME 03.23.2025

Self-limited epilepsy with centrotemporal spikes

Introduction

Overview

Self-limited epilepsy with centrotemporal spikes (SLECTS) is the new name for the previous epilepsy syndrome of benign 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 it from other sinister causes for focal epilepsies. However, it was subsequently noted that dysfunctions in cognitive and language domains as well as atypical evolutions with a not-so-benign course are seen in this syndrome. As per the current 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 purposes of this review, we are using the term self-limited epilepsy with centrotemporal spikes (SLECTS) for the typical cases to maintain uniformity. Atypical evolutions after a seemingly classical onset as well as clinical variants are discussed separately. Emerging data on cognitive impairment in children with SLECTS have also been described.

Key points

• The defining feature of SLECTS is the presence of classical centrotemporal spikes on interictal EEG. However, these are not specific to this syndrome. Centrotemporal spikes may be seen in children without seizures, with other epilepsy syndromes, and with structural lesions in the rolandic area. Moreover, the typical seizures in SLECTS may be seen without the classical spikes on the interictal record.

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

• Atypical clinical and EEG features are usually seen with transitory cognitive and language impairments.

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

Historical note and terminology

Rolandic (centrotemporal) region is named after Luigi Rolando (1773-1831), an Italian anatomist known for his pioneer research in 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 (63; 68). In 1958, the first clinical description associated with EEG features was published (125). 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 (67). Because of the localization of the ictal events, Lombroso proposed the term "sylvian seizures" (114). 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 at that time confirmed the good prognosis (10; 98; 113). 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 (28; 47). As new syndromes were recognized within the spectrum of benign childhood focal epilepsies (136), the term “benign” was considered initially acceptable for this syndrome but was considered inappropriate for some of the other idiopathic focal epilepsies (71).

However, the new ILAE Classification of the Epilepsies proposed the term “self-limited” for the “benign” course of epilepsies to reflect a “likely spontaneous resolution of a syndrome” (151). Hence, a new term “self-limited epilepsy with centrotemporal spike” (SLECTS) has been proposed for BCECTS (151). 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) (167).

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125