Real-life data comparing the efficacy of vigabatrin and oral steroids given sequentially or combined for infantile epileptic spasms syndrome.

Autor: Dozieres-Puyravel B; APHP. Service de Neurologie Pédiatrique, EpiCARE ERN Membre, Hôpital Robert Debré, Paris, France. Electronic address: blandine.dozieres@aphp.fr., Nasser H; Service de Physiologie - Explorations Fonctionnelles Pédiatriques - Centre Pédiatrique des Pathologies du Sommeil, AP-HP, Hôpital Universitaire Robert Debré, F-75019, Paris, France. Electronic address: hala.nasser@aphp.fr., Mauvais FX; Service de Physiologie - Explorations Fonctionnelles Pédiatriques - Centre Pédiatrique des Pathologies du Sommeil, AP-HP, Hôpital Universitaire Robert Debré, F-75019, Paris, France; Université Paris Cité, CNRS, Inserm, Institut Necker-Enfants Malades, F- 75015, Paris, France. Electronic address: francois-xavier.mauvais@aphp.fr., De Saint Martin A; Service de Neurologie Pédiatrique, CHU de Strasbourg, France. Electronic address: Anne.DESAINTMARTIN@chru-strasbourg.fr., Perriard C; Service de Neurologie Pédiatrique, CHU de Strasbourg, France. Electronic address: boffcaroline@gmail.com., Di Meglio C; Aix-Marseille Univ, APHM, Service de Neurologie Pédiatrique, Hopital de la Timone-Enfants. Marseille, France. Electronic address: Chloe.DI-MEGLIO@ap-hm.fr., Cances C; Service de neurologie pédiatrique, CHU de Toulouse, France. Electronic address: cances.c@chu-toulouse.fr., Hachon-LE Camus C; Service de neurologie pédiatrique, CHU de Toulouse, France. Electronic address: hachonlecamus.c@chu-toulouse.fr., Milh M; Aix-Marseille Univ, APHM, Service de Neurologie Pédiatrique, Hopital de la Timone-Enfants. Marseille, France. Electronic address: Mathieu.MILH@ap-hm.fr., Auvin S; APHP. Service de Neurologie Pédiatrique, EpiCARE ERN Membre, Hôpital Robert Debré, Paris, France; Université Paris-Cité, INSERM NeuroDiderot, Paris, France; Institut Universitaire de France (IUF), Paris, France. Electronic address: stephane.auvin@aphp.fr.
Jazyk: angličtina
Zdroj: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society [Eur J Paediatr Neurol] 2024 Jan; Vol. 48, pp. 61-66. Date of Electronic Publication: 2023 Nov 27.
DOI: 10.1016/j.ejpn.2023.11.009
Abstrakt: Aims: The prognosis of Infantile epileptic spasm syndrome (IESS), relates to the underlying etiology and delay in controlling epileptic spasms. Based on the spasm-free rate, a randomized controlled trial has demonstrated the superiority of combining oral steroids and vigabatrin over oral steroids alone but confirmation in real-life conditions is mandatory.
Methods: We compared two real-life IESS cohorts: a multicenter, retrospective cohort of 40 infants treated with vigabatrin followed by a sequential (ST) addition of steroids, and a prospective, single-center cohort of 58 infants treated with an immediate combination of vigabatrin and steroids (CT).
Results: The two cohorts were similar. When the rate of spasm-free infants in the two cohorts was compared on day 14, a significant difference was observed between the ST (27,5 %) and CT cohorts (64 %) (p < 0.0004). This difference remained significant on day 30, with 55 % spasm-free patients in the ST cohort compared to 76 % in the CT cohort (p = 0.03). After the infants had received both vigabatrin and steroids, without taking into account the time point after treatment initiation, no significant difference was observed in the spasm-free rate between the two cohorts (p = 0.38).
Interpretation: Real-life data confirm the interest of combination therapy as a first-line treatment for IESS.
(© 2023 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.)
Databáze: MEDLINE
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