Childhood epilepsy: every parent's worst nightmare and the triumph of cannabis

Epilepsy is a chronic disease characterized by frequent, unpredictable seizures. Around 30% of children with epilepsy become refractory or resistant to standard anti-epileptic drugs, and those suffering from epileptic encephalopathy (brain damage) can be particularly complex to treat. The human endocannabinoid system can modify the physiological processes underlying epilepsy.

The anti-epileptic properties of several cannabinoids, namely Δ9-tetrahydrocannabinol and cannabidiol (CBD), have been demonstrated in in vitro and in vivo studies. Cannabis-based therapies have long been used to treat many illnesses, including epilepsy.

Several large randomized controlled studies designed to measure the efficacy and safety of pharmaceutical-grade CBD in children with Dravet and Lennox-Gastaut syndromes have shown similar efficacy to other antiepileptic drugs. The lack of information concerning the appropriate dosage to prescribe to children with epilepsy, as well as paediatric pharmacokinetics, still make it difficult to authorize cannabis-based therapies for children with epilepsy who are resistant to the treatment they are given(1).

What is Dravet syndrome?

Dravet syndrome is a rare, severe epilepsy that manifests itself in the first year of life as recurrent, fever-triggered, long-lasting hemiclonic seizures(unilateral convulsive seizures). Some 70-80% of patients carry abnormalities in the SCN1A gene. This type of epilepsy is highly drug-resistant and difficult to treat. Although children manage to continue growing normally at first, over time most patients develop moderate to severe intellectual impairment, behavioral problems and a typical crouching gait. Many children suffer sudden and unexpected death due to epilepsy. Seizures can rarely be completely controlled. Some new drugs to treat Dravet syndrome, such as stiripentol, cannabidiol and fenfluramine, are very promising and seem to have optimistic results(2).

What is Lennox-Gastaut syndrome?

This is an early-onset epileptogenic encephalopathy that results in several types of generalized seizures, including tonic seizures, atypical absence seizures and frequent états de mal épileptiques. What's more, this form of epilepsy, which begins in infancy, is probably described as every parent's worst nightmare. Children develop several types of seizures, which can lead to a progressive and rapid decline in cognitive, behavioral and intellectual abilities(3).