Abstract:
PURPOSE:Dravet syndrome (DS) or severe myoclonic epilepsy of infancy is an intractable epileptic encephalopathy of early childhood that is caused by a mutation in the SCN1A gene in most patients. The aim of this study was to identify a syndrome-specific epileptic network underlying interictal epileptiform discharges (IEDs) in patients with DS. METHODS:Ten patients with the diagnosis of DS associated with mutations in the SCN1A gene were investigated using simultaneous recording of electroencephalography and functional magnetic resonance imaging ((EEG-fMRI). Time series of IEDs were used as regressors for the statistical fMRI analysis. KEY FINDINGS:In nine patients with DS, individual blood oxygenation level-dependent (BOLD) signal changes were seen. In three patients the thalamus was involved. Furthermore, regions of the default mode network were activated in seven patients. However, a common activation pattern associated with IEDs could not be detected. SIGNIFICANCE:The study demonstrates that, despite a common genetic etiology in DS, different neuronal networks underlie the individual IEDs.
journal_name
Epilepsiajournal_title
Epilepsiaauthors
Moehring J,von Spiczak S,Moeller F,Helbig I,Wolff S,Jansen O,Muhle H,Boor R,Stephani U,Siniatchkin Mdoi
10.1111/epi.12119subject
Has Abstractpub_date
2013-05-01 00:00:00pages
918-26issue
5eissn
0013-9580issn
1528-1167journal_volume
54pub_type
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