Bilateral and synchronous “dents de scie” spikes: A highly specific EEG pattern of young adult Dravet syndrome

Abstract

BackgroundIn Dravet syndrome (DS), EEGs evolve over time. ObjectiveTo describe a peculiar EEG pattern in two adults with a de novo SCN1A gene mutation, in exon 5 (case 1) and 9 (case 2). MethodsTwo female patients underwent a prolonged video EEG (24 h) as part of their epilepsy assessment. ResultsIn both cases, the EEG showed a very peculiar and stereotypical pattern of bilateral synchronous spikes at about 5–6 Hz. This activity was present during wakefulness and highly activated at sleep onset and in NREM sleep, which could show nearly continuous spike activity. This activity dramatically decreased in REM sleep and after awakening. This pattern of “dents de scie” (sawtooth) spikes maintained the same morphology throughout the entire EEG recording. In both patients, the spikes were favored by passive eye closure. During wakefulness, the spikes could evolve into atypical absences while keeping the same “dents de scie” pattern. Neither patient had tonic or myoclonic seizures at the time of the EEG assessment. Both were moderately retarded, and neither one had a typical DS gait disorder. Previous EEG recordings of case 1 performed at 9.5 and 18.5 years showed spike-waves, but the morphology did not correspond to the EEG recording observed at 22 years. ConclusionsBoth patients have a similar electro-clinical phenotype. This “dents de scie” pattern appears to be very specific and could be pathognomonic in a subgroup of young adults with DS. Results of sleep EEG recording could be added to the diagnostic criteria for this syndrome.