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Current therapeutic procedures in Dravet syndrome
1Inserm, U663, Paris, Université Paris Descartes, Paris, France. catherine.chiron2@gmail.com
Developmental Medicine and Child Neurology
|April 21, 2011
Summary
Stiripentol is an effective adjunctive therapy for Dravet syndrome, a severe epilepsy. It enhances GABAergic neurotransmission and should be used with reduced doses of valproate and clobazam to minimize side effects.
Area of Science:
- Neurology
- Pharmacology
- Genetics
Background:
- Dravet syndrome is a severe, drug-resistant epilepsy in children.
- First-line treatments like valproate and benzodiazepines are often insufficient.
- Certain medications (lamotrigine, carbamazepine) can worsen seizures.
Purpose of the Study:
- To evaluate the efficacy and safety of stiripentol as an adjunctive therapy for Dravet syndrome.
- To understand stiripentol's mechanism of action and its role in managing pharmaco-resistant epilepsy.
Main Methods:
- Two independent double-blind, placebo-controlled trials in children with Dravet syndrome.
- Assessment of seizure frequency and severity with stiripentol combined with valproate and clobazam.
- Evaluation of side effects, particularly appetite and weight changes, related to cytochrome P450 inhibition.
Main Results:
- Stiripentol demonstrated significant efficacy in Dravet syndrome when added to valproate and clobazam.
- Stiripentol enhances GABAergic neurotransmission via GABA(A) receptors.
- Side effects like appetite and weight loss can be managed by reducing valproate and clobazam doses.
Conclusions:
- Stiripentol is an approved orphan drug for adjunctive therapy in Dravet syndrome.
- It offers a valuable treatment option for pharmaco-resistant epilepsy, with over 500 patients successfully treated.
- Topiramate and ketogenic diets are alternatives for refractory cases.
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