司替戊醇的主治疾病及药理作用？

Stipentol is an FDA-approved anti-epileptic drug that works by reducing abnormal excitatory activity in the brain. It is specifically designed to treat epileptic seizures related to Dravet syndrome in children 6 months and older (weight 14 pounds or more) taking clobazam. It can help patients significantly reduce epileptic seizures.

Indications of stiripentol

In the EU, it is indicated for use in combination with clobazan and valproate in patients with Dravet syndrome (DS, formerly known as severe myoclonic epilepsy of infancy) in whom clobazan and valproate do not adequately control seizures.

Stivapentol, used together with clobazam, is used to control seizures in people with Dravet syndrome (a type of epilepsy that starts in early childhood and causes seizures in adults and children 6 months and older weighing at least 7 kg, and subsequent seizures that may lead to developmental delays and changes in diet, balance and walking).

Pharmacological effects of stiripentol

Stipentol is an orally active, structurally unique antiepileptic drug (AED) with multiple potential mechanisms of action, including different effects on gamma-aminobutyric acid (GABA)-A receptors and novel inhibitory effects on lactate dehydrogenase.

Stirpentol also has anticonvulsant properties and the ability of the cytochrome P450 system, and participates in the regulation of the cardiac axis and GABAergic system of other AEDs, which can have neuroprotective effects by reducing calcium-mediated neurotoxicity.

Efficacy of stiripentol

The strongest evidence for efficacy is in Dravet syndrome, where the addition of stiripentol to clobazan and valproic acid reduced the frequency and severity of seizures in most cases. Small case series have also shown benefit in malignant partial moving seizures in infancy, super-refractory status epilepticus, and refractory focal epilepsy.

Two small randomized controlled trials in which stiripentol as adjuvant therapy resulted in significantly higher remission rates after 2 months compared with placebo in patients aged 3 to about 21 years with DS not adequately controlled by clobazan and valproate.

These short-term results were subsequently supported and extended by results from long-term, open-label, observational studies, including a retrospective longitudinal cohort study that showed that the efficacy of stiripentol combined with clobazan and valproate starting in childhood was maintained in mid-adulthood, with exposure periods up to 24 years and up to 40 years of age.

Based on current evidence, stiripentol is a clearly beneficial and well-tolerated second-line treatment for patients with DS as an adjunct to clobazan and valproate.

Summary

Stipentol is available in two dosage forms: capsule preparation and oral suspension. Patients can choose the medication and treat it correctly under the guidance of a doctor. During the treatment period, strictly follow the doctor's instructions and do not adjust the dosage or stop the medication without permission to avoid withdrawal reactions and worsening of the disease.

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