Pathways to epilepsy surgery in children with tuberous sclerosis complex-associated epilepsy.

Cohort study Epilepsy surgery Long-term follow-up Tuberous sclerosis complex

Journal

Revue neurologique
ISSN: 0035-3787
Titre abrégé: Rev Neurol (Paris)
Pays: France
ID NLM: 2984779R

Informations de publication

Date de publication:
11 Jun 2024
Historique:
received: 13 02 2024
revised: 22 04 2024
accepted: 25 04 2024
medline: 13 6 2024
pubmed: 13 6 2024
entrez: 12 6 2024
Statut: aheadofprint

Résumé

Previous studies showed the efficacy of epilepsy surgery in carefully selected children with epilepsy associated with tuberous sclerosis complex. However, how this selection is conducted, and the characteristics of the patients brought to surgery are still poorly described. By conducting a multicentric retrospective cohort study covering the practice of the last twenty years, we describe the paths leading to epilepsy surgery in children with epilepsy associated with tuberous sclerosis complex. We identified 84 children diagnosed with tuberous sclerosis complex and epilepsy by matching two exhaustive registries of genetic diseases and subsequent medical records reviews within two French neuropediatric and epilepsy centers. Demographic, clinical, longitudinal, and diagnostic and surgical procedures data were collected. Forty-six percent of the children were initially drug-resistant and 19% underwent resective surgery, most often before the age of four. Stereotactic electroencephalography was performed prior to surgery in 44% of cases. Fifty-seven and 43% of patients remained seizure-free one and ten years after surgery, respectively. In addition, 52% of initially drug-resistant patients who did not undergo surgery were seizure-free at the last follow-up. The number of anti-seizure medications required decreased in 50% of cases after surgery. Infantile spasms, intellectual disability, autism spectrum disorder or severe behavioral disorders were not contraindications to surgery but were associated with a higher rate of complications and a lower rate of seizure freedom after surgery. Despite the assumption of complex multifocal epilepsy and practical difficulties in young children with tuberous sclerosis complex, successful surgery results are comparable with other populations of patients with drug-resistant epilepsy, and a spontaneous evolution to drug-sensitive epilepsy may occur in non-operated patients.

Sections du résumé

BACKGROUND BACKGROUND
Previous studies showed the efficacy of epilepsy surgery in carefully selected children with epilepsy associated with tuberous sclerosis complex. However, how this selection is conducted, and the characteristics of the patients brought to surgery are still poorly described. By conducting a multicentric retrospective cohort study covering the practice of the last twenty years, we describe the paths leading to epilepsy surgery in children with epilepsy associated with tuberous sclerosis complex.
METHODS METHODS
We identified 84 children diagnosed with tuberous sclerosis complex and epilepsy by matching two exhaustive registries of genetic diseases and subsequent medical records reviews within two French neuropediatric and epilepsy centers. Demographic, clinical, longitudinal, and diagnostic and surgical procedures data were collected.
RESULTS RESULTS
Forty-six percent of the children were initially drug-resistant and 19% underwent resective surgery, most often before the age of four. Stereotactic electroencephalography was performed prior to surgery in 44% of cases. Fifty-seven and 43% of patients remained seizure-free one and ten years after surgery, respectively. In addition, 52% of initially drug-resistant patients who did not undergo surgery were seizure-free at the last follow-up. The number of anti-seizure medications required decreased in 50% of cases after surgery. Infantile spasms, intellectual disability, autism spectrum disorder or severe behavioral disorders were not contraindications to surgery but were associated with a higher rate of complications and a lower rate of seizure freedom after surgery.
CONCLUSION CONCLUSIONS
Despite the assumption of complex multifocal epilepsy and practical difficulties in young children with tuberous sclerosis complex, successful surgery results are comparable with other populations of patients with drug-resistant epilepsy, and a spontaneous evolution to drug-sensitive epilepsy may occur in non-operated patients.

Identifiants

DOI: 10.1016/j.neurol.2024.04.009 PMID: 38866657
pubmed: 38866657
pii: S0035-3787(24)00533-2
doi: 10.1016/j.neurol.2024.04.009
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2024 Elsevier Masson SAS. All rights reserved.

Auteurs

L Gauer (L)

Hôpitaux Universitaires de Strasbourg, Neurology Department, Strasbourg, France; Hôpitaux Universitaires de Strasbourg, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Strasbourg, France. Electronic address: lucas.gauer@chru-strasbourg.fr.

S Lagarde (S)

Assistance publique-Hôpitaux de Marseille, Epileptology and Cerebral Rhythmology, Marseille, France; Assistance publique-Hôpitaux de Marseille, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Marseille, France; Aix-Marseille University, INSERM, Institut de Neurosciences des Systèmes, Marseille, France.

M-P Valenti-Hirsch (MP)

Hôpitaux Universitaires de Strasbourg, Neurology Department, Strasbourg, France; Hôpitaux Universitaires de Strasbourg, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Strasbourg, France.

J Makhalova (J)

Assistance publique-Hôpitaux de Marseille, Epileptology and Cerebral Rhythmology, Marseille, France; Assistance publique-Hôpitaux de Marseille, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Marseille, France; Aix-Marseille University, INSERM, Institut de Neurosciences des Systèmes, Marseille, France.

M Milh (M)

Assistance publique-Hôpitaux de Marseille, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Marseille, France; Assistance publique-Hôpitaux de Marseille, Pediatric Neurology Department, Marseille, France.

S Baer (S)

Hôpitaux Universitaires de Strasbourg, Pediatric Neurology Department, Strasbourg, France.

A Lepine (A)

Assistance publique-Hôpitaux de Marseille, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Marseille, France; Assistance publique-Hôpitaux de Marseille, Pediatric Neurology Department, Marseille, France.

I Ollivier (I)

Hôpitaux Universitaires de Strasbourg, Neurosurgery Department, Strasbourg, France.

D Scavarda (D)

Aix-Marseille University, INSERM, Institut de Neurosciences des Systèmes, Marseille, France; Assistance publique-Hôpitaux de Marseille, Pediatric Neurosurgery Department, Marseille, France.

E Hirsch (E)

Hôpitaux Universitaires de Strasbourg, Neurology Department, Strasbourg, France; Hôpitaux Universitaires de Strasbourg, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Strasbourg, France.

F Bartolomei (F)

Assistance publique-Hôpitaux de Marseille, Epileptology and Cerebral Rhythmology, Marseille, France; Assistance publique-Hôpitaux de Marseille, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Marseille, France; Aix-Marseille University, INSERM, Institut de Neurosciences des Systèmes, Marseille, France.

A De Saint-Martin (A)

Hôpitaux Universitaires de Strasbourg, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Strasbourg, France; Hôpitaux Universitaires de Strasbourg, Pediatric Neurology Department, Strasbourg, France.

N Villeneuve (N)

Assistance publique-Hôpitaux de Marseille, Reference Centre for Rare Epilepsies (member of the ERN network EPICARE), Marseille, France; Assistance publique-Hôpitaux de Marseille, Pediatric Neurology Department, Marseille, France.

Classifications MeSH