Analyzing the etiology of benign rolandic epilepsy: a multicenter twin collaboration.

Abstract:

PURPOSE:Benign rolandic epilepsy (BRE) is considered a genetically determined idiopathic partial epilepsy. We analyzed a large sample of twins from four international twin registers to probe the genetics of BRE. We also aim to synthesize the apparently conflicting family and twin data into a model of BRE etiology. METHODS:Large population-based twin registries of epilepsies from Odense (Denmark), Richmond, Virginia (United States), and Oslo (Norway) were reviewed for BRE cases and added to our Australian twin data. Diagnosis of classic BRE was based on electroclinical criteria with normal neurologic development. Cases with a compatible electroclinical picture but abnormal neurologic development were termed non-classic BRE. RESULTS:Eighteen twin pairs were identified (10 monozygous; eight dizygous) of whom at least one twin was diagnosed with classic BRE among a total sample of 1,952 twin pairs validated for seizures, and all were discordant for BRE. The estimated monozygous pairwise concordance for BRE in this sample was 0.0 [95% confidence interval (CI), 0.0-0.3). Four twin pairs (one monozygous, three dizygous) had non-classic BRE, and all co-twins had seizures. CONCLUSIONS:The twin data showing an absence of any concordant twin pairs with classic BRE suggest that noninherited factors are of major importance in BRE. Modelling the data shows that the familial occurrence of centrotemporal spikes makes only a minor contribution to the familial aggregation of BRE. Genetic factors are probably more important in non-classic BRE. The etiology and mode(s) of inheritance of BRE are much more complicated than initially conceptualized.

journal_name

Epilepsia

journal_title

Epilepsia

authors

Vadlamudi L,Kjeldsen MJ,Corey LA,Solaas MH,Friis ML,Pellock JM,Nakken KO,Milne RL,Scheffer IE,Harvey AS,Hopper JL,Berkovic SF

doi

10.1111/j.1528-1167.2006.00466.x

subject

Has Abstract

pub_date

2006-03-01 00:00:00

pages

550-5

issue

3

eissn

0013-9580

issn

1528-1167

pii

EPI466

journal_volume

47

pub_type

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