Mortality in tuberous sclerosis complex.
Mortality
Sudden unexpected death in epilepsy (SUDEP)
Tuberous sclerosis complex
Journal
Epilepsy & behavior : E&B
ISSN: 1525-5069
Titre abrégé: Epilepsy Behav
Pays: United States
ID NLM: 100892858
Informations de publication
Date de publication:
08 2021
08 2021
Historique:
received:
04
03
2021
revised:
20
04
2021
accepted:
24
04
2021
pubmed:
5
6
2021
medline:
1
7
2021
entrez:
4
6
2021
Statut:
ppublish
Résumé
We studied mortality in tuberous sclerosis complex (TSC) by analyzing data from the Tuberous Sclerosis Alliance Natural History Database of 2233 patients from 18 United States TSC centers. Among 31 decedents with data; mean age of death was 29 years. Cause of death could be determined in 26 cases: 11 definitely related to TSC, 14 possibly related to TSC, and 1 unrelated to TSC. Causes of death included SUDEP in 11 (35.5%; Definite (5), Probable (4), Possible (2)), respiratory conditions in 6 (23.1%; lymphangiomyelomatosis in one), tumors in 3 (11.5%), suicide in 2 (7.7%), cardiopulmonary in 2 (7.7%), shunt malfunction in one, and drowning in one. For SUDEP cases, mean age of epilepsy onset was 7 months and 10/11 were treated with multiple anti-seizure medications (ASMs) at death; 7 had intractable epilepsy and 3 were controlled with ASMs. Patients with TSC and their families should be counseled about ASM adherence and lifestyle factors, and the potential role of nocturnal supervision or seizure detection devices to prevent SUDEP.
Identifiants
pubmed: 34087679
pii: S1525-5050(21)00266-3
doi: 10.1016/j.yebeh.2021.108032
pii:
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
108032Informations de copyright
Copyright © 2021 Elsevier Inc. All rights reserved.