Definition and new approaches to understanding the pathogenesis of Drave syndrome in children

Nowadays, Dravet syndrome is one of the most studied forms of genetic epilepsy. Despite a large number of studies and publications, Dravet syndrome remains a difficult neurological disease to treat. The relationship of epileptogenesis in Dravet syndrome with the activity of sodium channels has been studied, and data on the different effectiveness and ineffectiveness of anticonvulsant drugs with different mechanisms of action have been obtained. Drugs that modify neural transmission, enhance inhibitory transmission, and reduce excitatory transmission, as well as their combination, are more effective. Drugs that reduce the effect of inhibitory transmission (in particular, sodium channel blockers) should not be used. In our opinion, the correct choice of therapy for Dravet syndrome associated with a mutation in the SCN1A gene is possible only from the perspective of understanding the work of neural channels in the brain.

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Publication Details

Journal
Russian Journal of Child Neurology
Published
2025-04-18
DOI
https://doi.org/10.17650/2073-8803-2025-20-1-17-24
Primary Topic
Cerebral Palsy and Movement Disorders
Type
article
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article

Definition and new approaches to understanding the pathogenesis of Drave syndrome in children

M. Yu. Bobylоva, О. А. Пылаева, S. Kazakov, К. Yu. Мukhin et al.
Russian Journal of Child Neurology
Cerebral Palsy and Movement Disorders
article

Definition and new approaches to understanding the pathogenesis of Drave syndrome in children

M. Yu. Bobylоva, О. А. Пылаева, S. Kazakov, К. Yu. Мukhin, В. С. Какаулина, A. N. Ulyakov, I. S. Tishchenko
article en

Abstract

Nowadays, Dravet syndrome is one of the most studied forms of genetic epilepsy. Despite a large number of studies and publications, Dravet syndrome remains a difficult neurological disease to treat. The relationship of epileptogenesis in Dravet syndrome with the activity of sodium channels has been studied, and data on the different effectiveness and ineffectiveness of anticonvulsant drugs with different mechanisms of action have been obtained. Drugs that modify neural transmission, enhance inhibitory transmission, and reduce excitatory transmission, as well as their combination, are more effective. Drugs that reduce the effect of inhibitory transmission (in particular, sodium channel blockers) should not be used. In our opinion, the correct choice of therapy for Dravet syndrome associated with a mutation in the SCN1A gene is possible only from the perspective of understanding the work of neural channels in the brain.

Russian Journal of Child NeurologyVol. 20(1)
Pirogov Russian National Research Medical University (RU), Russian Children's Clinical Hospital (RU), Ministry of Health of the Russian Federation (RU), City Clinical Hospital (RU)
Openalex Percentile: Top 90%
Cerebral Palsy and Movement Disorders
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