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Journal of Pediatric Epilepsy 2017; 06(02): 111-114
DOI: 10.1055/s-0037-1599191
Original Article
Georg Thieme Verlag KG Stuttgart · New York

SCN1A Does Not Determine Seizure Duration in Children Unaffected by Dravet's Syndrome

François Le Gal
1   Molecular Diagnostic Laboratory, Genetic Medicine Service, University Hospitals, Geneva, Switzerland
,
Sébastien Lebon
2   Pediatric Neurology Unit, Department of Paediatrics, Lausanne University Hospital, Switzerland
,
Gian Paolo Ramelli
3   Pediatric Neurology, Regional Hospital, Bellinzona, Switzerland
,
Alexandre N. Datta
4   Pediatric Neurology, Cantonal Hospital, Basel, Switzerland
,
Danielle Mercati
5   Pediatric Neurology, Cantonal Hospital, Neuchâtel, Switzerland
,
Oliver Maier
6   Pediatric Neurology, Children's Hospital, St. Gallen, Switzerland
,
Christophe Combescure
7   Clinical Research Center, Division of Clinical Epidemiology, Department of Health and Community Medicine, University of Geneva, University Hospitals, Geneva, Switzerland
,
Maria Isabel Rodriguez
8   Clinical Research Platform, Child and Adolescent Department, University Hospitals, Geneva, Switzerland
,
Margitta Seeck
9   Clinical Neurosciences Department, University Hospitals, Geneva, Switzerland
,
Eliane Roulet
2   Pediatric Neurology Unit, Department of Paediatrics, Lausanne University Hospital, Switzerland
,
Christian M. Korff
10   Pediatric Neurology Unit, Child and Adolescent Department, University Hospitals, Geneva, Switzerland
› Author Affiliations
Further Information

Publication History

08 July 2016

01 October 2016

Publication Date:
02 March 2017 (online)

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Abstract

A significant proportion of children with seizures present with status epilepticus (SE), early in their disease. Our objective was to evaluate whether SCN1A variants predispose children to SE, outside of Dravet's syndrome. SCN1A analyses were performed in children aged 1 month to 16 years with at least one episode of status epilepticus (group A), and in children with short seizures only (group B). Clinical and genetic findings were compared between both groups. Total 99 children were included: 61 in group A, 38 in group B. SCN1A variants were found in 2 (3.3%) of 61 patients with status epilepticus and in 3 (7.9%) of 38 patients with short seizures. The difference between both groups was −4.6% (95% CI −18 to 5.1), and was not statistically significant (p = 0.37, Fisher's exact test). In four of these five children, seizures were observed in a GEFS+ familial context. The last patient had a generalized genetic epilepsy not further determined. SCN1A variants were not significantly more frequent in group A than in group B. Their predicted severity was not higher. Our results indicate that SCN1A variants per se do not determine seizure duration in children not affected by Dravet's syndrome.

Keywords

seizures - childhood - duration - SCN1A