Anti-Seizure Medication

Children and adults living with Dravet Syndrome normally need a combination of tailored anti-seizure medications.

These help to control or reduce their seizures. Keeping someone free from seizures is the ultimate goal of these medications, however, good seizure control in Dravet Syndrome is rare.

Anti-Seizure Medications used to treat Dravet Syndrome

The following medications may be used to treat the condition and aim to control or minimise seizures. Not all of them are suitable for everyone with Dravet Syndrome.

• Sodium valproate: Also known as ‘Epilim’, this drug can be injected directly into a vein in emergencies.
• Clobazam: This slows down the brain and body and may be used when someone hasn’t responded to other drugs.
• Stiripentol: This drug is added to sodium valproate and clobazam if someone’s seizures are not controlled with those on their own.
• Cannabidiol: Also known as ‘epidyolex’, cannibidiol is a chemical in the cannabis plant. As a medicine, it’s an oral solution which is a plant-derived cannabis-based medicine. It can be used with clobazam for people with Dravet Syndrome over two years old.
• Topiramate: This medicine is used to treat epilepsy in children and adults.
• Levetiracetam: This is approved to treat partial seizures, or as an add-on medication for partial, myoclonic and tonic clonic seizures. Find out more about these seizure types here.
• Bromides: An older anti-seizure medication, this is not prescribed much in the UK but may be by some Dravet Syndrome specialists.
• Fenfluramine: This is used as an add-on medication with two other anti-epileptic medicines, such as sodium valproate, for those aged over 24 months.

In 2022, international experts put together a ‘treatment algorithm’ based on their experience of the most effective order to trial medications in people living with Dravet Syndrome.

Graphic adapted from Wirrell et al, Epilepsia (2022)

Medications that should not be used for children

In children, some medications can increase or prolong someone’s seizures if they have Dravet Syndrome. If they are used for a long time, these drugs can affect intellectual development. These medicines are called ‘sodium channel blockers’. They include:

• carbamazepine
• lamotrigine
• phenytoin (though this may be useful when it is injected as an emergency treatment)
• oxcarbazepine
• lacosamide
• rufinamide.

Medications that should not be used for adults

In adults, sodium channel blockers should only be used to treat Dravet Syndrome when they are carefully monitored by medical professionals. They might be prescribed if a young person or adult receives a new diagnosis.

Overall, there is not enough evidence of the effect that sodium channel blockers have on adults because there haven’t been enough randomised controlled trials that have included them.

Prescriptions

In the UK, the NHS recommends that people with any type of epilepsy, including Dravet Syndrome, continue to get the same version of their epilepsy medicine, wherever possible, as seizure control is particularly sensitive to changes in medication.

If a doctor decides that a patient should stay on a specific brand name, this should be written on the prescription, and pharmacists have to provide that brand. If the prescribed product is unavailable, it may be necessary to give a product from a different manufacturer to maintain continuity of treatment but this should be discussed and agreed with both the doctor and family. For most people, it’s safer to take a different version of their medicine than to stop taking it.

When thinking about the impact of switching brands, medications are split into 3 categories:

• Category 1: Doctors are advised to ensure that their patient is maintained on a specific branded product. (Carbamazepine, phenobarbital, phenytoin, primidone).
• Category 2: Doctors can use their clinical judgement on whether someone should remain on the same version. This decision should be made in partnership with families and is made on a case-by-case basis, taking into account factors such as seizure frequency and history. (Clobazam, clonazepam, eslicarbazepine acetate, lamotrigine, oxcarbazepine, perampanel, rufinamide, topiramate, valproate, zonisamide).
• Category 3: It is usually unnecessary to ensure that patients are maintained on a specific manufacturer’s product. Difficulties for patients with autism, mental health problems, or learning disability should be considered. (Brivaracetam, ethosuximide, gabapentin, lacosamide, levetiracetam, pregabalin, tiagabine, vigabatrin).

Further reading

There are a number of guidelines, statements and publications outlining treatment for Dravet Syndrome. These are:

• NHS guidelines on Dravet Syndrome, (section 6.1).
• NHS guidelines on using fenfluramine (Fintepla) to treat Dravet Syndrome.
• The International League Against Epilepsy classification and definition of Dravet Syndrome.
• Hierarchy of Treatment for Dravet Syndrome, including medicine given in an emergency to relieve symptoms quickly.
• European consensus statement on Dravet Syndrome, a public statement by a team of experts in the field.
• North American experts in Dravet Syndrome consensus statement.