- Dravet Syndrome
THE FOLLOWING INFORMATION IS PRESENTED FOR EDUCATIONAL PURPOSES ONLY. MEDICAL MARIJUANA INC. PROVIDES THIS INFORMATION TO PROVIDE AN UNDERSTANDING OF THE POTENTIAL APPLICATIONS OF CANNABINOIDS. 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 cannabinoids and their influence on the endocannabinoid system may have the capability of reducing, and in some cases eliminating, seizures.
OVERVIEW OF DRAVET SYNDROMEDravet 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 SYNDROMEStudies have found that the body’s endocannabinoid system can be modulated to produce anti-seizure effects4,5. Cannabinoids found in cannabis interact with the endocannabinoid system’s cannabinoid receptors (CB1 and CB2), suggesting that they may hold therapeutic potential for managing seizure activity.
The endocannabinoid system’s CB1 receptor in particular has shown that it can be used to inhibit the release of a particular neurotransmitter and reduce overall neuronal excitability, thus silencing the triggers of seizures1,2. Body-synthesized cannabinoids, called endocannabinoids, have shown to play a role in the regulation of seizure threshold and intensity, suggesting that cannabis-derived cannabinoids and their influence on the receptors may also support the endocannabinoid system and its efforts to control seizures caused by Dravet syndrome4.
STATES THAT HAVE APPROVED MEDICAL MARIJUANA FOR DRAVET SYNDROMECurrently, 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
OVERVIEW OF DRAVET SYNDROMEDravet 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 SYNDROMEStudies have found that the body’s endocannabinoid system can be modulated to produce anti-seizure effects4,5. Cannabinoids found in cannabis interact with the endocannabinoid system’s cannabinoid receptors (CB1 and CB2), suggesting that they may hold therapeutic potential for managing seizure activity.
The endocannabinoid system’s CB1 receptor in particular has shown that it can be used to inhibit the release of a particular neurotransmitter and reduce overall neuronal excitability, thus silencing the triggers of seizures1,2. Body-synthesized cannabinoids, called endocannabinoids, have shown to play a role in the regulation of seizure threshold and intensity, suggesting that cannabis-derived cannabinoids and their influence on the receptors may also support the endocannabinoid system and its efforts to control seizures caused by Dravet syndrome4.
STATES THAT HAVE APPROVED MEDICAL MARIJUANA FOR DRAVET SYNDROMECurrently, 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
- The cannabinoid receptor 1 (CB1) of the endocannabinoid system can be used to produce anti-seizure effects.
Activation of the cannabinoid type-1 receptor mediates the anticonvulsant properties of cannabinoids in the hippocampal neuronal culture models of acquired epilepsy and status epilepticus.
http://jpet.aspetjournals.org/content/317/3/1072.long
- Blair, R.E., Deshpande, L.S., Sombati, S., Falenski, K.W., Martin, B.R., and DeLorenzo, R.J. (2006, June). Activation of the cannabinoid type-1 receptor mediates the anticonvulsant properties of cannabinoids in the hippocampal neuronal culture models of acquired epilepsy and status epilepticus. The Journal of Pharmacology and Experimental Therapeutics, 317(3), 1072-1078. Retrieved from http://jpet.aspetjournals.org/content/317/3/1072.long.
- Karlócai, M. R., Tóth, K., Watanabe, M., Ledent, C., Juhász, G., Freund, T. F., & Maglóczky, Z. (2011). Redistribution of CB1 Cannabinoid Receptors in the Acute and Chronic Phases of Pilocarpine-Induced Epilepsy. PLoS ONE, 6(11), e27196. Retrieved fromhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208595/.
- NINDS Dravet Syndrome Information Page. (2001, September 29). National Institute of Neurological Disorders and Stroke. Retrieved fromhttp://www.ninds.nih.gov/disorders/dravet_syndrome/dravet_syndrome.htm.
- Wallace, M.J., Martin, B.R., and DeLorenzo, R.J. (2002). Evidence for a physiological role of endocannabinoids in the modulation of seizure threshold and severity. European Journal of Pharmacology, 452(3), 295-301. Retrieved from http://www.sciencedirect.com/science/article/pii/S0014299902023312.
- Wallace, M.J., Blair, R.E., Falenski, K.W., Martin, B.R., and DeLorenzo, R.J. (2003, October). The endogenous cannabinoid system regulates seizure frequency and duration in a model of temporal lobe epilepsy. The Journal of Pharmacology and Experimental Therapeutics, 307(1), 129-37. Retrieved from http://jpet.aspetjournals.org/content/307/1/129.long.
- What is Dravet syndrome? (2014, March). Epilepsy Foundation. Retrieved fromhttp://www.epilepsy.com/learn/types-epilepsy-syndromes/dravet-syndrome.