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Dravet Syndrome – Medical Marijuana Research Overview

The following information is presented for educational purposes only. Medical Marijuana Inc. provides this information to provide an understanding of the potential applications of cannabidiol. Links to third party websites do not constitute an endorsement of these organizations by Medical Marijuana Inc. and none should be inferred.

Dravet syndrome is a type of severe epilepsy that develops during the first year of childhood and is characterized by frequent febrile seizures. Studies have shown cannabis has the capability of reducing, and in some cases eliminating, seizures.

Overview of Dravet Syndrome

Dravet syndrome, which is also referred to as severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy that typically develops during the first year of life. The high frequency of seizures caused by the syndrome commonly causes children to experience difficulty developing language and motor skills. In addition, they often experience hyperactivity and have problems relating to others.

Dravet syndrome typically causes febrile seizures, which are fever-related (associated with a high body temperature but without any attributing health issue). Seizures can be triggered by slight body temperature changes, flashing lights, emotional stress or excitement. In addition, myoclonic seizures, characterized by involuntary muscle spasms, and status epilepticus, a state of continuous seizure that lasts longer than 30 minutes and requires emergency care, can arise.

According to the National Institute of Neurological Disorders and Stroke, 30 to 80% of Dravet syndrome cases are caused by defects in the SCN1A gene, which are essential for the brain cells to properly function.

There is no cure for Dravet syndrome, but in some cases medications can help control seizures. In addition, adjusting the diet so that it’s high in fats and low in carbohydrates may be beneficial. While it’s a lifelong condition, it’s not uncommon for children with Dravet syndrome to experience improvements and better cognitive function with age.

Findings: Effects of Cannabis on Dravet Syndrome

One of the major cannabinoids found in cannabis, cannabidiol (CBD), has been determined to be a well-tolerated therapeutic treatment that can reduce or even eliminate seizures in a variety of childhood epilepsy disorders3,4,5,7,14,16,17,22,24. Tetrahydrocannabinol (THC) has also demonstrated efficacy at reducing seizures in children with epilepsy14. Preclinical trials have found that cannabis causes significant anticonvulsant effects in mice and rats9,10.

CBD is able to reduce or eliminate seizures because it interacts with cannabinoid receptors (CB1 and CB2) of the body’s endocannabinoid system28. The CB1 receptor can inhibit the release of a particular neurotransmitter and reduces overall neuronal excitability, thus silencing the triggers of seizures2,10,19,24,28. CBD’s influence on the CB2 receptor may indirectly support the dysregulation of the endocannabinoid system in the brain that causes seizures caused by Dravet syndrome25.

One case report analyzing a young girl with Dravet syndrome found that medical marijuana brought the child’s seizure frequency from nearly 50 convulsive seizures per day to 2-3 nocturnal convulsions per month. In addition, the child was able to wean from the additional anti-epileptic drugs she had been taking21.

Double-blind randomized, placebo-controlled studies are currently lacking, but early research suggests that cannabis may be effective in treating a variety of severe pediatric epilepsy disorders like Dravet syndrome. In one questionnaire study, 84% of parents reported a reduction in their child’s seizure frequency following cannabis treatment. Out of those parents, 11% of them responded that their child has reached complete seizure freedom, while 42% reported a greater than 80% reduction in seizure frequency. The parents also reported additional beneficial effects, such as increased alertness, better mood and improved sleep23. Another similar survey found that CBD-enriched cannabis brought about a reduction in seizure frequency in 85% of epileptic children, while 14% experienced complete seizure freedom. The children also reported an improvement in sleep (53%), alertness (71%), and mood (63%) while being treated with CBD16. Another study examining the effect of CBD-enriched medical cannabis on children with epilepsy found that 89 percent of children reported a reduction in seizure frequency with CBD treatment. The children also reportedly saw improvements in behavior and alertness, language, communication,motor skills and sleep25.

Another cannabinoid found in cannabis, cannabidivarin (CBDV) has also shown to have non-psychoactive anticonvulsant effects1,11.

Traditional medicines used to treat epilepsy are not just ineffective for most; they also often come with a number of adverse side effects. Cannabinoids found in cannabis, however, have shown to produce anticonvulsant effects in preclinical and preliminary human studies while producing fewer adverse effects that other antiepileptic drugs8. A questionnaire study found that parents tried an average of 12 different antiepileptic drugs, due to ineffectiveness or unacceptable side effects, before finding gentle effectiveness with cannabis23.

States That Have Approved Medical Marijuana for Dravet Syndrome

Currently, just South Carolina has approved medical marijuana specifically for the treatment of Dravet syndrome. However, several states have approved medical marijuana specifically to treat epilepsy and other seizure disorders. These states include: Alabama (debilitating epileptic conditions), Connecticut, Delaware (intractable epilepsy), Georgia (seizure disorder), Iowa (intractable epilepsy), Louisiana, Maine, Mississippi (intractable epilepsy), Missouri (intractable epilepsy), New Hampshire, New Jersey (seizure disorders), New Mexico, New York, North Carolina (intractable epilepsy), North Dakota, Ohio, Oklahoma (pediatric epilepsy), Pennsylvania, South Carolina (Dravet syndrome, Lennox-Gastaut syndrome, Refractory epilepsy), Texas (intractable epilepsy), Utah (intractable epilepsy), Virginia (intractable epilepsy), West Virginia, Wisconsin (seizure disorders), and Wyoming (intractable epilepsy).

In addition, several states approve medical marijuana to specifically treat seizures. These states include: Alaska, Arizona, Arkansas, California, Colorado, Delaware, Florida, Hawaii, Louisiana, Maryland, Michigan, Minnesota, Montana, Nevada, New Hampshire, North Dakota, Ohio, Oregon, Pennsylvania (intractable seizures), Rhode Island, Tennessee (intractable seizures), Vermont, Washington, and West Virginia.

The state of Massachusetts will consider allowing medical marijuana to be used for the treatment of Dravet syndrome if it’s determined in writing by a qualifying patient’s physician.

In Washington D.C., any condition can be approved for medical marijuana as long as a DC-licensed physician recommends the treatment.

Recent Studies on Cannabis’ Effect on Dravet Syndrome

  • CBD-enriched cannabis reduced seizure frequency in 85% of children and 14% reported complete seizure freedom. Children also saw improvements in sleep (53%), alertness (71%), and mood (63%).
    Perceived efficacy of cannabidiol-enriched cannabis extracts for treatment of pediatric epilepsy: A potential role for infantile spasms and Lennox-Gastaut syndrome.
    (
    http://www.epilepsybehavior.com/article/S1525-5050(15)00157-2/fulltext)
  • Cannabis reduced seizure frequency in 84% of children, while 11% reported complete seizure freedom. Additional beneficial effects included increased alertness, better mood, and improved sleep.
    Report of a parent survey of cannabidiol-enriched cannabis use in pediatric treatment-resistant epilepsy.
    (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4157067/)

References:

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  • October 6, 2015