Benign childhood epilepsy with centrotemporal spikes

Natalio Fejerman MD (Dr. Fejerman of Hospital de Pediatria JP Garrahan has no relevant financial relationships to disclose.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released October 18, 1993; last updated March 5, 2016; expires March 5, 2019

This article includes discussion of benign childhood epilepsy with centrotemporal spikes, BCECTS, benign rolandic epilepsy, sylvian epilepsy, and midtemporal epilepsy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The syndrome of benign childhood epilepsy with centrotemporal spikes 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 old. “Benign” applies well to this syndrome even though atypical evolutions with a not-so-benign course may occur in a small percentage of cases. The concept of atypical benign childhood epilepsy with centrotemporal spikes and its difference from the aforementioned cases of benign childhood epilepsy with centrotemporal spikes with atypical evolutions is clarified in this article. Recent data about academic performance and attention impairment in children with this syndrome are also discussed.

Key points

 

• Many reports have emphasized the frequency of specific cognitive disorders in children with benign childhood epilepsy with centrotemporal spikes.

 

• Atypical features in clinical and electroencephalographic manifestations seem to be correlated to the transitory cognitive disorders.

 

• Persistent neuropsychologic disorders are only seen in those children with benign childhood epilepsy with centrotemporal spikes who evolve into the encephalopathic range of the spectrum, which includes Landau-Kleffner syndrome and continuous spike-and-waves during slow sleep syndrome.

 

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

Historical note and terminology

A particular EEG pattern with migratory spikes originating over the rolandic (centrotemporal or midtemporal) region was first reported in the 1950s (Gastaut 1952; Gibbs et al 1954). In 1958 the first description of clinical features in association with the peculiarities of the EEG in those children was published (Nayrac and Beaussart 1958). 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 (Gibbs and Gibbs 1960). Because of the localization of the ictal events, Lombroso proposed the term "sylvian seizures" (Lombroso 1967). In the same year, Loiseau and colleagues presented a series of 122 children with what they called “a particular form of epilepsy in childhood,” stressing its benign character and highlighting clinical and EEG features. Several long-term follow-up studies confirmed the good prognosis (Beaussart 1972; Lerman and Kivity 1975; Beaussart and Faou 1978; Loiseau et al 1988). Atypical and not-so-benign evolutions have been reported in some patients with this form of epilepsy (Aicardi and Chevrie 1982; Fejerman and Di Blasi 1987; Fejerman 1996; Fejerman 2002; Fejerman et al 2000; Fejerman et al 2007b; Hahn et al 2001). This form of epilepsy is now called “benign childhood epilepsy with centrotemporal spikes” and is placed in the group of idiopathic localization-related (focal, local, partial) epilepsies in the International Classification of Epilepsies and Epileptic Syndromes (Commission on Classification and Terminology of the International League Against Epilepsy 1989; Engel 2001). One review focused on assessment of established and newly recognized syndromes within the spectrum of benign childhood focal epilepsies (Panayiotopoulos et al 2008). In a review on benign childhood focal epilepsies, the term “benign” was accepted for this syndrome, but was considered inappropriate for some of the other idiopathic focal epilepsies (Guerrini and Pellacani 2012).

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