Movement-activated cortical myoclonus in Dravet syndrome

Highlights • Multifocal action myoclonus occurred in all examined patients with Dravet Syndrome. • Myoclonus corresponded to brief EMG bursts which frequency peaked in beta band. • Somatosensory evoked potentials and long loop reflexes were normal in all but one. • Significant cortico-muscular coher...

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Bibliographic Details
Published in:Epilepsy research 2017-02, Vol.130, p.47-52
Main Authors: Canafoglia, Laura, Ragona, Francesca, Panzica, Ferruccio, Piazza, Elena, Freri, Elena, Binelli, Simona, Scaioli, Vidmer, Avanzini, Giuliano, Granata, Tiziana, Franceschetti, Silvana
Format: Article
Language:English
Subjects:
Adolescent
Adult
Brain - physiopathology
Child
Child, Preschool
Cortical myoclonus
Cortico-muscolar coherence
Dravet syndrome
Electroencephalography
Electromyography
Epilepsies, Myoclonic - genetics
Epilepsies, Myoclonic - physiopathology
Evoked Potentials, Somatosensory
Humans
Movement - physiology
Muscle, Skeletal - physiopathology
Myoclonus - physiopathology
NAV1.1 Voltage-Gated Sodium Channel - genetics
Neurology
SCN1A mutations
Signal Processing, Computer-Assisted
Somatosensory evoked potentials
Spectral analysis
Young Adult
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Summary:Highlights • Multifocal action myoclonus occurred in all examined patients with Dravet Syndrome. • Myoclonus corresponded to brief EMG bursts which frequency peaked in beta band. • Somatosensory evoked potentials and long loop reflexes were normal in all but one. • Significant cortico-muscular coherence fitted the cortical origin of the jerks. • Action myoclonus is intrinsic feature of Dravet syndrome and cause of disability.
ISSN:0920-1211
1872-6844
DOI:10.1016/j.eplepsyres.2017.01.007