Bromide in Patients with SCN1A-Mutations Manifesting as Dravet Syndrome

Jan Lotte; Edda Haberlandt; Bernd Neubauer; Martin Staudt; Gerhard Josef Kluger

doi:10.1055/s-0032-1307454

Neuropediatrics, Table of Contents

Neuropediatrics 2012; 43(01): 017-021
DOI: 10.1055/s-0032-1307454

Original Article

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Bromide in Patients with SCN1A-Mutations Manifesting as Dravet Syndrome

Jan Lotte

¹Neuropediatric Clinic and Clinic for Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany

,

Edda Haberlandt

²Clinical Department of Pediatrics IV, Division of Neonatology, Neuropediatrics and Inherited Metabolic Diseases, Medical University Innsbruck, Innsbruck, Austria

,

Bernd Neubauer

³Zentrum für Kinderheilkunde und Jugendmedizin, Abteilunge für Neuropädiatrie und Sozialmedizin, Universitätsklinikum Gießen, Gießen, Germany

,

Martin Staudt

¹Neuropediatric Clinic and Clinic for Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany

⁴Department of Pediatric Neurology and Developmental Medicine, University Children's Hospital, Tübingen, Germany

,

Gerhard Josef Kluger

¹Neuropediatric Clinic and Clinic for Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany

⁵Paracelsus Medical University, Salzburg, Austria

› Author Affiliations

Abstract

We report a retrospective analysis of bromide therapy in 32 patients suffering from Dravet syndrome with SCN1A-mutations who received bromide. After 3 months of bromide treatment, 26 patients (81%) showed a relevant improvement with a reduction of seizure frequency by >50% (>75%) in 18 (12) patients (56 and 37%, respectively). After 12 months, we observed a reduction of >50% (>75%) in 15 (9) patients (47 and 28%, respectively). Long-term response was noted in 18 patients (56%). Adverse reactions were mainly mild or moderate leading to treatment termination in 5/32 patients; no aggravation was reported. We conclude that bromide holds promise in patients with SCN1A-mutations suffering from Dravet syndrome.

Keywords

SCN1A-mutation - Dravet syndrome - bromide - long-term efficacy

Full Text

References

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