From the Guidelines
The initial treatment for Dravet syndrome typically involves antiseizure medications, with stiripentol being a key consideration due to its efficacy in reducing seizures in patients with this condition. According to a recent study published in 2023 1, stiripentol, an LDHA-targeted oral commercial medication, has shown promise in treating Dravet syndrome. The study highlights the importance of considering stiripentol as part of the initial treatment options for Dravet syndrome, given its potential to reduce seizures and improve patient outcomes.
Some key points to consider when treating Dravet syndrome include:
- Antiseizure medications such as valproate and clobazam are often used as first-line options
- Stiripentol can be added as an adjunctive therapy when seizures persist
- Fenfluramine has shown significant efficacy and received FDA approval specifically for Dravet syndrome
- Medications to avoid include sodium channel blockers like carbamazepine, oxcarbazepine, phenytoin, and lamotrigine
- A ketogenic diet may be considered as an adjunctive therapy in refractory cases
It is essential to initiate treatment promptly after diagnosis to help control seizures and potentially improve developmental outcomes. Regular monitoring of medication side effects is also crucial, with valproate requiring liver function tests and complete blood counts, while fenfluramine needs cardiac monitoring due to potential heart valve effects. Treatment plans should be individualized based on seizure type, frequency, and the patient's tolerance to medications, as noted in the context of Dravet syndrome management 1.
From the FDA Drug Label
DIACOMIT is indicated for the treatment of seizures associated with Dravet syndrome in patients taking clobazam who are 6 months of age and older and weighing 7 kg or more . There are no clinical data to support the use of DIACOMIT as monotherapy in Dravet syndrome.
The initial treatment options for Dravet syndrome include the use of clobazam in combination with stiripentol (DIACOMIT) for patients 6 months of age and older and weighing 7 kg or more 2.
- Stiripentol (DIACOMIT) is not recommended as monotherapy for Dravet syndrome due to lack of clinical data supporting its use alone 2 2.
- The dosage of DIACOMIT is 50 mg/kg/day, administered by mouth in 2 or 3 divided doses, depending on age and weight 2.
From the Research
Initial Treatment Options for Dravet Syndrome
The initial treatment options for Dravet syndrome include:
- Broad-spectrum anti-seizure medications (ASMs) such as valproate (VPA) and clobazam (CLB) 3
- Adjunct ASMs like stiripentol (STP), cannabidiol (CBD), and fenfluramine (FFA) which have been approved specifically for the treatment of seizures in patients with Dravet syndrome 3
- Topiramate, which is frequently used and has evidence of efficacy in Dravet syndrome 3, 4, 5, 6
- Bromide, which is used in some regions like Germany and Japan, although its efficacy is based on anecdotal evidence 3, 6
Key Considerations for Treatment
When treating Dravet syndrome, it is essential to consider the following:
- The potential for drug-drug interactions and their consequences, which may require dosage adjustments to alleviate adverse events 3
- The need for regular cardiac monitoring, particularly with FFA, due to its association with cardiac valve disease in high doses 3
- The importance of monitoring standard laboratory and clinical parameters, including blood counts, liver function tests, and serum concentrations of ASMs 3
- The potential for certain medications, such as lamotrigine, carbamazepine, and high doses of intravenous phenobarbital, to worsen seizures and should be avoided 4, 5, 7
Additional Therapies
Other therapies that may be used in the treatment of Dravet syndrome include:
- The ketogenic diet, which has been shown to provide substantial efficacy as an adjunctive therapy 4, 5, 6
- Non-pharmacologic therapies, such as neurostimulation or surgery, although these are understudied in Dravet syndrome 7
- Psychostimulants, which can be helpful for attention-deficit hyperactivity disorder, a common comorbidity in patients with Dravet syndrome 7