Benign Epilepsy Of Childhood With Centrotemporal Spikes Research Articles

Cognitive and behavioral problems in children with centrotemporal spikes

Atypical features in benign epilepsy of childhood with centrotemporal spikes (BECTS) are not uncommon. There are children with BECTS who do not have a benign outcome in terms of neuropsychologic functioning. BECTS have been linked with Landau-Kleffner syndrome (LKS) and continuous spikes and waves during slow sleep (CSWS). At the Medical College of Georgia from January 1988 to June 1999, 78 children, ages 2-16 years, were identified to have electroencephalogram evidence of centrotemporal spikes. Their medical records were reviewed for developmental history, behavioral problems, and school performance. Children with structural lesions/other epileptic syndromes were excluded. Fifty-six demonstrated a history of clinical seizures compatible with BECTS and 22 demonstrated centrotemporal spikes without clinical seizures. Among all children with centrotemporal spikes, 9% (n = 7) were diagnosed with mild intellectual disability (intelligence quotient < 70), 10% (n = 8) with borderline functioning, 31% (n = 24) with behavioral problems, and 17% (n = 13) with specific learning disabilities. Three children with BECTS experienced language delay and regression. Seizure control for BECTS usually is achieved without much difficulty, with excellent long-term prognosis. However, the data presented indicate that a large number of BECTS patients exhibit learning or behavior problems that require intervention. A small number may demonstrate language outcome similar to children with LKS and CSWS.

Centrotemporal spikes in families with rolandic epilepsy: linkage to chromosome 15q14.

To localize a gene predisposing to benign epilepsy of childhood with centrotemporal spikes (BECTS). BECTS, or rolandic epilepsy, is the most prevalent idiopathic epilepsy syndrome in childhood. Functional relevant defects in the alpha 4 subunit of the neuronal nicotinic acetylcholine receptor (AChR) have been demonstrated in autosomal dominant nocturnal frontal lobe epilepsy, which, like BECTS, is an idiopathic partial epilepsy. A DNA linkage study was conducted screening all chromosomal regions known to harbor neuronal nicotinic AChR subunit genes. Twenty-two nuclear families with BECTS were analyzed. In an "affected-only" study, best p values and lod scores were reached between D15S165 and D15S1010 on chromosome 15q14. In multipoint nonparametric linkage analysis a nominal p value of 0.000494 was calculated by GENEHUNTER. Best parametric results were obtained under an autosomal recessive model with heterogeneity (multipoint lod score 3.56 with 70% of families linked to the locus). These markers are localized in direct vicinity to the alpha 7 subunit gene of the AChR. We found evidence for linkage of BECTS to a region on chromosome 15q14. Either the alpha 7 AChR subunit gene or a closely linked gene are implicated in pedigrees with BECTS. The disorder is genetically heterogeneous. Surprisingly, the same chromosomal area has been reported to be linked to the phenotype in families with an auditory neurophysiologic deficit as well as in families with juvenile myoclonic epilepsy, another idiopathic but generalized epilepsy syndrome.

Benign epilepsy of childhood with centrotemporal spikes and unilateral developmental opercular dysplasia

The case of a 15-year-old right-handed girl with developmental delay, mild retardation, astereognosis, and tactile discrimination impairment in the left arm, is reported. During sleep, right partial simple motor seizures with sialorrhea and saliva pooling were present, sometimes followed by secondary generalization. Absences and drop attacks were also observed. Electroencephalography revealed left centrotemporal spike waves spreading to the homologous contralateral region, generalized 3-Hz spike-and-wave complexes enhanced by hyperventilation, and generalized polyspike-and-wave discharges during drowsiness. Magnetic resonance imaging showed right perisylvian (opercular) malformation. Benign epilepsy of childhood with centrotemporal spikes (BECCTS) is a focal idiopathic epilepsy presumed to be of genetic origin. Although brain damage is not expected, structural lesions including opercular macrogyria have been reported. This coexistence has been considered mainly causal and only exceptionally causal. The Foix-Chavany-Marie syndrome or operculum syndrome is the result of bilateral opercular damage. In our child the right structural and the left functional lesions appeared mutually activated, yielding a transient opercular syndrome. The concomitance of BECCTS and developmental opercular dysplasia is suggestive of a common genetic substratum.