Severe myoclonic epilepsy in infancy: A systematic review and a meta-analysis of individual patient data

Authors

  • Behrouz Kassaï,

    1. Inserm, CIC201, EPICIM, Lyon, France; University of Lyon, UMR 5558, Lyon, France; CHU Lyon, Hop L Pradel, Service de Pharmacologie Clinique, Lyon, France
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  • Catherine Chiron,

    1. Inserm, U663, Paris, France; University of Paris Descartes, Paris, France; Assistance Publique–Hopitaux de Paris, Service de Neurologie et Metabolisme, Hôpital Necker-Enfants Malades, Paris, France
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  • Ségolène Augier,

    1. Inserm, CIC201, EPICIM, Lyon, France; University of Lyon, UMR 5558, Lyon, France; CHU Lyon, Hop L Pradel, Service de Pharmacologie Clinique, Lyon, France
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  • Michel Cucherat,

    1. Inserm, CIC201, EPICIM, Lyon, France; University of Lyon, UMR 5558, Lyon, France; CHU Lyon, Hop L Pradel, Service de Pharmacologie Clinique, Lyon, France
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  • Elisabeth Rey,

    1. Assistance Publique–Hopitaux de Paris, Service de Pharmacologie Clinique, Hopital Cochin–Saint Vincent de Paul, Paris, France; University of Paris Descartes, Paris, France
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  • François Gueyffier,

    1. Inserm, CIC201, EPICIM, Lyon, France; University of Lyon, UMR 5558, Lyon, France; CHU Lyon, Hop L Pradel, Service de Pharmacologie Clinique, Lyon, France
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  • Renzo Guerrini,

    1. Istituto di Neuropsichiatria Infantile, Università di Pisa, Pisa, Italy
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  • Julien Vincent,

    1. Istituto di Neuropsichiatria Infantile, Università di Pisa, Pisa, Italy
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  • Olivier Dulac,

    1. Inserm, U663, Paris, France; University of Paris Descartes, Paris, France; Assistance Publique–Hopitaux de Paris, Service de Neurologie et Metabolisme, Hôpital Necker-Enfants Malades, Paris, France
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  • Gérard Pons

    1. Assistance Publique–Hopitaux de Paris, Service de Pharmacologie Clinique, Hopital Cochin–Saint Vincent de Paul, Paris, France; University of Paris Descartes, Paris, France
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Address correspondence to Dr. Behrouz Kassaï, Service de Pharmacologie Clinique, Rue G Paradin, BP8071, 69376 Lyon Cedex 8. E-mail: bk@upcl.univ-lyon1.fr

Summary

Severe myoclonic epilepsy in infancy (SMEI) is a rare, but severe disorder with seizures typically resistant to conventional antiepileptic drugs. The objective of the present study was to systematically review the literature on the available treatments for SMEI.

Databases searched included Medline, Embase, and Cochrane. We used a fixed effect model to summarize the odds ratio of seizures rates and a logistic model to evaluate the influence of patient characteristics on treatment effect.

We found 23 uncontrolled studies and 2 randomized controlled trials (RCTs) that compared stiripentol with placebo. Overall, 64 children aged between 3 and 20 years were included in the two RCTs. The odds ratio of responding to stiripentol relative to placebo was 32 (CI: 6.2, 161) and stiripentol reduced seizure rate by 70% (93%; 47%). The multivariate analysis does not suggest any differences within subgroups of participants and cotherapy.

Results of uncontrolled studies in children with SMEI are potentially biased and do not provide valid information on the benefits and harms of treatments. The two RCTs identified, however, were performed with the same objectives and design and showed that seizure frequency is greatly reduced by stiripentol in children with SMEI after 2 months of treatment.

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