Bilateral and synchronous "dents de scie" spikes: A highly specific EEG pattern of young adult Dravet syndrome.
Adults
Atypical absence
Dravet syndrome
EEG
Eye closure sensitivity
Spikes
Journal
Seizure
ISSN: 1532-2688
Titre abrégé: Seizure
Pays: England
ID NLM: 9306979
Informations de publication
Date de publication:
25 Nov 2023
25 Nov 2023
Historique:
received:
22
10
2023
revised:
19
11
2023
accepted:
25
11
2023
medline:
1
12
2023
pubmed:
1
12
2023
entrez:
30
11
2023
Statut:
aheadofprint
Résumé
In Dravet syndrome (DS), EEGs evolve over time. To describe a peculiar EEG pattern in two adults with a de novo SCN1A gene mutation, in exon 5 (case 1) and 9 (case 2). Two female patients underwent a prolonged video EEG (24 h) as part of their epilepsy assessment. In 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. Both 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.
Sections du résumé
BACKGROUND
BACKGROUND
In Dravet syndrome (DS), EEGs evolve over time.
OBJECTIVE
OBJECTIVE
To describe a peculiar EEG pattern in two adults with a de novo SCN1A gene mutation, in exon 5 (case 1) and 9 (case 2).
METHODS
METHODS
Two female patients underwent a prolonged video EEG (24 h) as part of their epilepsy assessment.
RESULTS
RESULTS
In 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.
CONCLUSIONS
CONCLUSIONS
Both 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.
Identifiants
pubmed: 38035489
pii: S1059-1311(23)00306-0
doi: 10.1016/j.seizure.2023.11.019
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
18-22Informations de copyright
Copyright © 2023 The Author(s). Published by Elsevier Ltd.. All rights reserved.