Summary:Purpose: To determine prevalence of active epilepsy in school children in a defined area and assess the usefulness of International League Against Epilepsy classification of seizures and epileptic syndromes, with special emphasis on frequency, additional handicaps, and therapeutic problems of severe cases.Methods: The latest International League Against Epilepsy International Classification of Epileptic Seizures (ICES, 198 1) and Epilepsies and Epileptic Syndromes (ICE, 1989) were used for determination of prevalence rates, seizure types, epilepsies and epileptic syndromes, and additional neurological deficits in all 6-to 12-year-old children with epilepsy in a Norwegian county. Children had neuropediatric and EEC examination, intelligence evaluation, and, when necessary, additional investigations.Results: Prevalence of active epilepsy on January 1, 1995, was 5.1 per 1,000. Main seizure type and epilepsy syndrome could be classified in 98% and 90% of patients, respectively. Seizure types/epileptic syndromes were more often partiall localization related than generalized. Among generalized epilepsies, idiopathic forms were more frequent in girls, and cryptogenic and symptomatic forms more frequent in boys. Epileptogenic EEG activity was most often generalized or localized to one or two areas of the brain and was never found in 14% of patients. Symptomatic etiology was found in 46% of all children and in 81% of therapy-resistant cases, respectively. Over the years, 11 ?h of children had never used antiepileptic drugs (AED), 62% had tried one or two AEDs, and 26% had tried from three to 15 AEDs. Twenty-five percent of children were without present AED treatment. Complementary/alternative medicine had been tried by 12% of children.Conclusions: Although most epilepsies could be classified, the number of cases in non-specific categories was relatively high. Symptomatic etiology was frequent, especially in therapy-resistant cases. Multidisciplinary therapeutic and habilitation approaches are often needed in childhood epilepsy.

The prevalence of OWOB and OB in Norwegian primary school children is of concern. Socio-demographic factors have pronounced effects on the current prevalence of overweight and obesity in a cohort of Norwegian children. This knowledge could help to work out strategies to reduce the burden of overweight and obesity in children.

Crisponi syndrome (CS) and cold-induced sweating syndrome type 1 (CISS1) are disorders caused by mutations in CRLF1. The two syndromes share clinical characteristics, such as dysmorphic features, muscle contractions, scoliosis and cold-induced sweating, with CS patients showing a severe clinical course in infancy involving hyperthermia, associated with death in most cases in the first years of life. To evaluate a potential genotype/phenotype correlation and whether CS and CISS1 represent two allelic diseases or manifestations at different ages of the same disorder, we carried out a detailed clinical analysis of 19 patients carrying mutations in CRLF1. We studied the functional significance of the mutations found in CRLF1, providing evidence that phenotypic severity of the two disorders mainly depends on altered kinetics of secretion of the mutated CRLF1 protein. On the basis of these findings, we believe that the two syndromes, CS and CISS1, represent manifestations of the same disorder, with different degrees of severity. We suggest renaming the two genetic entities CS and CISS1 with the broader term of Sohar-Crisponi syndrome.

In childhood epilepsy various seizure-related factors, but not SES factors, were associated with the presence or absence of SNVP. Such deficits may be especially common in children with remote symptomatic epilepsy aetiology and in complex and therapy resistant epilepsies. Low frequencies of SNVP may be found in children with idiopathic and LR cryptogenic epilepsy syndromes, simple partial or absence seizures and a late epilepsy debut. Our study contributes to an overall picture of cognitive function and its relation to central seizure characteristics in a childhood epilepsy population and can be useful for the follow-up team in developing therapy strategies that meet the individual needs of the child with epilepsy.

Our study has demonstrated a significant increase in weight-for-height in Norwegian children over the last 30 years, and that these changes are caused by an increase in fat tissue, as shown by skinfold measurements. The current prevalence of overweight and obesity is comparable to recent estimates from most Western and Northern European countries.