An unexpected EEG course in Dravet syndrome.
A puzzling EEG pattern combining frontal slow bi-tri spikes followed or not by slow waves when awake and activated by sleep with 5-10s discharges of 8-9Hz spikes in a minority of adolescents with Dravet syndrome (DS) was recorded in the context of stable seizure and cognitive status, and unchanged antiepileptic medication. Tonic seizures were frequently reported in patients with this EEG pattern (3/5). This EEG pattern could suggest that of Lennox-Gastaut syndrome (LGS) but it exhibits clear differences and therefore should not be considered as a change into LGS but as a previously overlooked unusual pattern in the adolescent course of DS.
APHP, Necker-Enfants Malades Hospital, Department of Neuropediatrics, Centre de référence épilepsies rares, Paris, France. rimanabbout@yahoo.com
Rima
R
Nabbout
Isabelle
I
Desguerre
Sandra
S
Sabbagh
Christel
C
Depienne
Perrine
P
Plouin
Olivier
O
Dulac
Catherine
C
Chiron
D000293:Adolescent; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D003071:Cognition / Q000502:physiology; D002658:Developmental Disabilities / Q000150:complications; D004569:Electroencephalography; D004576:Electromyography; D004830:Epilepsy, Tonic-Clonic / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D005334:Fever / Q000150:complications; D006801:Humans; D008297:Male; D009154:Mutation; D020190:Myoclonic Epilepsy, Juvenile / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D062550:NAV1.1 Voltage-Gated Sodium Channel; D009419:Nerve Tissue Proteins / Q000235:genetics; D015222:Sodium Channels / Q000235:genetics; D013577:Syndrome
D000927:Anticonvulsants; D062550:NAV1.1 Voltage-Gated Sodium Channel; D009419:Nerve Tissue Proteins; C568242:SCN1A protein, human; D015222:Sodium Channels