The relation of etiology based on the 2017 ILAE classification to the effectiveness of the ketogenic diet in drug-resistant epilepsy in childhood.


Journal

Epilepsia
ISSN: 1528-1167
Titre abrégé: Epilepsia
Pays: United States
ID NLM: 2983306R

Informations de publication

Date de publication:
11 2021
Historique:
revised: 16 08 2021
received: 03 05 2021
accepted: 16 08 2021
pubmed: 29 8 2021
medline: 19 4 2022
entrez: 28 8 2021
Statut: ppublish

Résumé

To investigate the effectiveness and safety of the ketogenic diet (KD) in drug-resistant epilepsy in childhood in relation to the new 2017 International League Against Epilepsy (ILAE) classification of etiology. A consecutive cohort of patients treated with the KD were categorized according to the ILAE classification into known (structural, genetic, metabolic, infectious, and immune-mediated) and unknown etiology. Primary outcome was the frequency of patients achieving seizure freedom with the KD at 3 months, secondary outcomes were seizure reduction >50% at 3 months, and both seizure freedom and seizure reduction >50% at 6, 12 months, and at last follow-up (LFU), and adverse effects. Outcomes were compared between etiology groups. Etiology was known in 70% (129/183). Outcomes did not differ at 3 months (known vs unknown: seizure freedom 28% vs 33%, seizure reduction 62 vs 67%), but seizure freedom was significantly less frequent in known etiology at 6 months (26% vs 43%) and beyond (22% vs 37%). Logistic regression identified duration of epilepsy, number of previous antiseizure medications (ASMs), and age-appropriate psychomotor development as positive determinants of outcome. Among individual etiology groups, the effectiveness of KD was relatively best for genetic (33% at LFU) and poorest for metabolic etiology (8% at LFU). The small number of patients with infectious and immune-mediated etiology requires larger numbers in each etiology group to corroborate our results. No differences in type and frequency of adverse effects (in 71%) between etiology groups were observed, requiring medical intervention in 21%. The KD was most effective in genetic and unknown etiology, many unknowns probably represent yet unidentified genetic causes. We recommend consequent diagnostic and genetic work-up to identify etiologies that respond best to the KD. The KD should be offered early to infants with genetic epilepsy before deterioration of epileptic symptoms and of psychomotor development.

Identifiants

pubmed: 34453316
doi: 10.1111/epi.17052
pmc: PMC9290115
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

2814-2825

Informations de copyright

© 2021 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.

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Auteurs

Markus Breu (M)

Department of Pediatrics and Adolescent Medicine, Affiliated Partner of the ERN EpiCARE, Medical University Vienna, Vienna, Austria.

Chiara Häfele (C)

Department of Pediatrics and Adolescent Medicine, Affiliated Partner of the ERN EpiCARE, Medical University Vienna, Vienna, Austria.

Petra Trimmel-Schwahofer (P)

Department of Pediatrics and Adolescent Medicine, Affiliated Partner of the ERN EpiCARE, Medical University Vienna, Vienna, Austria.

Wolfgang M Schmidt (WM)

Neuromuscular Research Department, Center for Anatomy and Cell Biology, Medical University Vienna, Vienna, Austria.

Franco Laconne (F)

Institute for Medical Genetics, Medical University Vienna, Vienna, Austria.

Julia Vodopiutz (J)

Department of Pediatrics and Adolescent Medicine, Affiliated Partner of the ERN EpiCARE, Medical University Vienna, Vienna, Austria.

Christoph Male (C)

Department of Pediatrics and Adolescent Medicine, Affiliated Partner of the ERN EpiCARE, Medical University Vienna, Vienna, Austria.

Anastasia Dressler (A)

Department of Pediatrics and Adolescent Medicine, Affiliated Partner of the ERN EpiCARE, Medical University Vienna, Vienna, Austria.

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