Comprehensive scoping review of fenfluramine's role in managing generalized tonic-clonic seizures in developmental and epileptic encephalopathies.
Details
State: Public
Version: Final published version
License: CC BY-NC 4.0
Serval ID
serval:BIB_0D05D8422600
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Comprehensive scoping review of fenfluramine's role in managing generalized tonic-clonic seizures in developmental and epileptic encephalopathies.
Journal
Epilepsia
ISSN
1528-1167 (Electronic)
ISSN-L
0013-9580
Publication state
Published
Issued date
08/2024
Peer-reviewed
Oui
Volume
65
Number
8
Pages
2186-2199
Language
english
Notes
Publication types: Journal Article ; Review ; Systematic Review
Publication Status: ppublish
Publication Status: ppublish
Abstract
Developmental and epileptic encephalopathies (DEEs) are characterized by pharmacoresistant seizures and developmental delay. Patients with DEEs experience multiple seizure types, including tonic-clonic seizures (TCS) that can be generalized tonic-clonic (GTCS) or focal evolving to bilateral tonic-clonic (FBTCS). Fenfluramine (FFA) has demonstrated efficacy in reduction of TCS in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), and other DEEs. Using the PRISMA-ScR (Preferred Reporting Items for Systematic Review and Meta-Analyses extension for Scoping Review) guidelines, we performed a scoping review to describe changes in TCS in patients treated with FFA. A comprehensive search of five literature databases was conducted up to February 14, 2023. Studies were included if they reported change in GTCS or TCS (but not FBTCS) after treatment with FFA in patients with DEEs. Duplicate patients and studies with unclear efficacy data were excluded. Fourteen of 422 studies met the eligibility criteria. Data extracted and evaluated by expert clinicians identified 421 unique patients with DS (in nine studies), CDKL5 deficiency disorder, SCN8A-related disorder, LGS, SCN1B-related disorder, and other DEEs. The median percent reduction in GTCS or TCS from baseline was available in 10 studies (n = 328) and ranged from 47.2% to 100%. Following FFA treatment, 10 studies (n = 144) reported ≥50% reduction in GTCS or TCS from baseline in 72% of patients; in nine of those (n = 112), 54% and 29% of patients achieved ≥75% and 100% reduction in GTCS or TCS from baseline, respectively. Overall, this analysis highlighted improvements in GTCS or TCS frequency when patients were treated with FFA regardless of the DEE evaluated. Future studies may confirm the impact of FFA on TCS reduction and on decreased premature mortality risk (including sudden unexpected death in epilepsy), improvement in comorbidities and everyday executive function, decreased health care costs, and improvement in quality of life.
Keywords
Humans, Fenfluramine/therapeutic use, Lennox Gastaut Syndrome/drug therapy, Epilepsies, Myoclonic/drug therapy, Epilepsies, Myoclonic/complications, Seizures/drug therapy, Spasms, Infantile/drug therapy, Anticonvulsants/therapeutic use, Dravet syndrome, Lennox–Gastaut syndrome, SUDEP, epilepsy, seizure
Pubmed
Web of science
Open Access
Yes
Create date
26/07/2024 13:56
Last modification date
13/08/2024 7:50