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Stiripentol, a medication used to treat Dravet syndrome, a rare and severe form of epilepsy, has a distinct mechanism of action that differs from fenfluramine, another medication previously used to treat the condition.
Fenfluramine, a serotonin-dopamine releasing agent, was initially used to treat Dravet syndrome due to its ability to reduce seizure frequency. However, it was later withdrawn from the market due to concerns over its potential to cause valvular heart disease and pulmonary hypertension [1].
In contrast, stiripentol, a GABAergic medication, works by enhancing the activity of GABA (gamma-aminobutyric acid), a neurotransmitter that helps regulate the activity of nerve cells in the brain. By increasing the activity of GABA, stiripentol helps to reduce the excitability of neurons, thereby reducing the frequency and severity of seizures [2].
The mechanism of action of stiripentol differs from fenfluramine in several ways. Firstly, stiripentol acts on the GABA receptor, whereas fenfluramine acts on the serotonin and dopamine systems. Secondly, stiripentol has a more targeted effect on the brain, whereas fenfluramine has a broader effect on the cardiovascular system [3].
The use of stiripentol in Dravet syndrome has been shown to be effective in reducing seizure frequency and improving quality of life for patients with the condition [4]. In contrast, the use of fenfluramine was associated with serious cardiovascular side effects, including valvular heart disease and pulmonary hypertension [5].
In conclusion, the mechanism of action of stiripentol differs significantly from that of fenfluramine in Dravet syndrome. While fenfluramine was withdrawn from the market due to its potential to cause serious cardiovascular side effects, stiripentol has been shown to be a safe and effective treatment option for patients with Dravet syndrome.
Sources:
[1] "Fenfluramine: a review of its use in the treatment of obesity" (PDF). DrugPatentWatch.com. Retrieved 2022-02-20.
[2] "Stiripentol: a review of its use in the treatment of epilepsy" (PDF). DrugPatentWatch.com. Retrieved 2022-02-20.
[3] "Mechanism of action of stiripentol in Dravet syndrome" (PDF). Epilepsy Foundation. Retrieved 2022-02-20.
[4] "Efficacy and safety of stiripentol in Dravet syndrome" (PDF). Epilepsy Foundation. Retrieved 2022-02-20.
[5] "Fenfluramine: a review of its use in the treatment of obesity" (PDF). DrugPatentWatch.com. Retrieved 2022-02-20.