Browse Conditions Browse Medicines

NICE recommendation: Cannabidiol with clobazam for treating seizures associated with Dravet syndrome and Lennox-Gastaut syndrome

Epidyolex▼, for Severe myoclonic epilepsy of infancy and Epilepsy from GW Pharma Ltd

The National Institute for Health and Care Excellence (NICE) have published their recommendations for the use of cannabidiol with clobazam for treating seizures associated with Dravet syndrome and Lennox–Gastaut syndrome.

Dravet syndrome is a severe form of epilepsy characterized by frequent, prolonged seizures often triggered by high body temperature (hyperthermia), developmental delay, speech impairment, ataxia, hypotonia (decreased muscle tone), sleep disturbances, and other health problems.

Cannabidiol with clobazam is recommended as an option for treating seizures associated with Dravet syndrome in people aged 2 years and older, only if:
  • the frequency of convulsive seizures is checked every 6 months, and cannabidiol is stopped if the frequency has not fallen by at least 30% compared with the 6 months before starting treatment
  • the company provides cannabidiol according to the commercial arrangement.
Lennox-Gastaut syndrome is one of the most severe forms of epilepsy in children. Its symptoms include multiple types of seizure (fit), abnormal electrical activity in the brain, learning disability and behavioural problems.

Cannabidiol with clobazam is recommended as an option for treating seizures associated with Lennox–Gastaut syndrome in people aged 2 years and older, only if:
  • the frequency of drop seizures is checked every 6 months, and cannabidiol is stopped if the frequency has not fallen by at least 30% compared with the 6 months before starting treatment
  • the company provides cannabidiol according to the commercial arrangement
This means that patients suffering from Dravet syndrome and Lennox-Gastaut syndrome can be treated on the NHS in England and Wales with this combination of medicines.

Cannabidiol is available under the brand name Epidyolex.

For more information see the full article.

Why NICE made these recommendations for Dravet syndrome
Current treatment for Dravet syndrome includes antiepileptic drugs. People with Dravet syndrome would have cannabidiol with clobazam if their convulsive seizures are not controlled well enough after trying 2 or more antiepileptic drugs.

Clinical trials show that cannabidiol reduces the number of convulsive and non-convulsive seizures when compared with usual care.

The cost-effectiveness estimates are uncertain for cannabidiol because of some of the assumptions in the company's model. The cost-effectiveness estimates do not include the benefits of:
  • reducing the number of non-convulsive seizures
  • reducing the duration of convulsive seizures
  • improving the quality of life of the siblings of people with Dravet syndrome
When taking both the uncertainties and the uncaptured benefits into account, cannabidiol is considered an appropriate use of NHS resources and is recommended as an option for treating Dravet syndrome in the NHS.

Why the committee made these recommendations for Lennox–Gastaut syndrome
Current treatment for Lennox–Gastaut syndrome includes antiepileptic drugs. People with Lennox–Gastaut syndrome would have cannabidiol with clobazam if their drop seizures are not controlled well enough after trying 2 or more antiepileptic drugs.

Clinical trials show that cannabidiol reduces the number of drop and non-drop seizures when compared with usual care.

The cost-effectiveness estimates are uncertain for cannabidiol because of some of the assumptions in the company's model. The cost-effectiveness estimates do not include the benefits of:
  • reducing the number of non-drop seizures
  • improving the quality of life of the siblings of people with Lennox–Gastaut syndrome
When taking both the uncertainties and the uncaptured benefits into account, cannabidiol is considered an appropriate use of NHS resources and is recommended as an option for treating Lennox–Gastaut syndrome in the NHS.

About Epidyolex (source EMA)
Epidyolex is a medicine used in addition to clobazam, another epilepsy medicine, to treat patients from the age of two years who have Lennox-Gastaut syndrome or Dravet syndrome, rare types of epilepsy that begin in childhood and can continue into adulthood.

Symptoms include multiple types of seizure (fits), abnormal electrical activity in the brain, learning disability and behavioural problems.

These conditions are rare, and Epidyolex was designated an ‘orphan medicine’ (a medicine used in rare diseases). Further information on the orphan designations can be found on the European Medicines Agency’s website: (Dravet syndrome: 15 October 2014; Lennox-Gastaut syndrome: 20 March 2017).

Although the way it works is not clearly understood, the active substance in Epidyolex, cannabidiol, is thought to act on targets that play a role in the movement of calcium in the cells. As this is important for the transmission of electrical signals in some nerve cells, and seizures are caused by excessive electrical activity in the brain, altering the movement of calcium is expected to reduce or prevent the seizures in patients with Lennox-Gastaut syndrome or Dravet syndrome. Cannabidiol is also thought to act on adenosine, a chemical messenger in the brain that plays an important role in suppressing seizures.

Epidyolex was first made available in the EU in 2019. It is manufactured by GW Pharma.

Sources
European Medicines Agency website page for Epidyolex
Accessed 06/01/20
Links available in External Resources

© NICE [2019]

Cannabidiol with clobazam for treating seizures associated with Dravet syndrome. Technology appraisal guidance [TA614]. Published date: 18 December 2019
Available from: See Link below. All rights reserved. Subject to Notice of rights
NICE guidance is prepared for the National Health Service in England. All NICE guidance is subject to regular review and may be updated or withdrawn. NICE accepts no responsibility for the use of its content in this product/publication.

The information provided by NICE was accurate at the time this article was issued.


NICE information: Cannabidiol with clobazam for treating seizures associated with Dravet syndrome

New medicines and vaccines that are under additional monitoring have an inverted black triangle symbol (▼) displayed in their package leaflet and summary of product characteristics, together with a short sentence explaining what the triangle means – it does not mean the medicine is unsafe. You should report all suspected adverse drug reactions (ADRs) for these products. ADRs can be reported by your doctor, pharmacist or online via the Yellow Card system.

Reporting of suspected adverse reactions

Reporting suspected adverse reactions (side effects) after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product. Healthcare professionals or patients are asked to report any suspected adverse reactions via the Yellow Card Scheme at yellowcard.mhra.gov.uk or search for MHRA Yellow Card in the Google Play or Apple App Store.


Disclaimer: This site is designed to offer information for general educational purposes only. The health information furnished on this site and the interactive responses are not intended to be professional advice and are not intended to replace personal consultation with a qualified physician, pharmacist, or other healthcare professional. We cannot provide individual medical advice. You must always seek the advice of a professional for questions related to a disease, disease symptoms, and appropriate therapeutic treatments.


About Severe myoclonic epilepsy of infancy About Epilepsy About medicine