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West syndrome is a type of severe pediatric epilepsy that affects one baby out of a few thousand in the US2. Studies have shown cannabinoids may be effective at significantly reducing seizure frequency, even potentially offering complete seizure freedom, in children with West’s syndrome and other types of epilepsy.
Overview of West’s Syndrome (Infantile Spasms)
West syndrome, or infantile spasms, is a pediatric epilepsy syndrome that is characterized by the baby’s experiencing involuntary stiffening. Named after Dr. William James West who first described the syndrome in the 1840s, West’s syndrome is considered an age specific epilepsy, typically developing between three and eight months of age. Nearly all cases begin before an infant turns one and will stop between the ages of two to four years2.
Commonly, the baby’s arms will fling out and the knees will pull up to the chest as the torso bends forward, in what appears to be a “jackknife.” In some cases, however, the baby’s head can throw back as the body and legs stiffen straight out.
Seizures last a second or two, but typically occur shortly after one another in a series. With each attack, the spasms can repeat from 10 to 20 or more times3. The spasms most commonly occur upon waking. Infants may cry during or immediately after experiencing a series of spasms. When the syndrome first develops, attacks typically are infrequent and do not occur in a series, which often causes an incorrect initial diagnosis of colic5.
According to the Epilepsy Foundation, about two thirds of babies with West syndrome have some known cause for the seizures2. Conditions, like malformations in the brain, a lack of oxygen to the brain, problems with a gene, issues with body metabolism, brain infections, or brain injuries may occur before the seizures first develop. In the other third of cases, there is no known condition or injury that causes the syndrome2.
West syndrome can limit the normal development of baby’s and cause them to lose the ability of rolling over, sitting, or babbling. Most children with infantile spasms experience intellectual disabilities later in life and some develop autism3. Most children with West syndrome, even if the spasms cease, will eventually develop other kinds of epilepsy like Lennox-Gastaut syndrome2.
Treating the seizures associated with West syndrome early and appropriately is important for limiting their damage. Traditional treatments are steroids or anti-seizure medications. In some cases, epilepsy surgery to address brain malformations may be necessary2.
Findings: Effects of Cannabis on West Syndrome
Evidence suggests that cannabinoids found in cannabis could potentially be therapeutically beneficial for managing seizures. This is because cannabinoids interact with the body’s endocannabinoid system, a regulatory network that researchers have determined plays a role in the reduction of seizure activity7.
The body synthesizes its own cannabinoids, called endocannabinoids, which have shown in studies to play a role in the regulation of seizure threshold and intensity6.
Studies suggest that the ability of cannabinoids to reduce or eliminate seizures is due to their interaction with the endocannabinoid system’s cannabinoid receptor 1 (CB1). When stimulated, the CB1 receptor dampens neurotransmission and produces an overall reduction in neuronal excitability1,4.
States That Have Approved Medical Marijuana for West Syndrome
While no states have approved medical marijuana specifically for the treatment of West syndrome, 20 states have approved medical marijuana specifically to treat epilepsy. These states include: Alabama (debilitating epileptic conditions), Connecticut, Delaware (intractable epilepsy), Georgia (seizure disorder), Iowa (intractable epilepsy), Maine, Mississippi (intractable epilepsy), Missouri (intractable epilepsy), New Hampshire, New Jersey (seizure disorders), New Mexico, New York, North Carolina (intractable epilepsy), Oklahoma (pediatric epilepsy), 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 have approved medical marijuana to treat seizures. These states include: Alaska, Arizona, Arkansas, California, Colorado, Delaware, 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 (intractable seizures).
Recent Studies on Cannabis’ Effect on West Syndrome
- 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.
- Infantile Spasms / West’s Syndrome. (2015, December). Epilepsy Foundation. Retrieved from http://www.epilepsy.com/learn/types-epilepsy-syndromes/infantile-spasms-wests-syndrome.
- Infantile Spasm (West Syndrome). (2014, October 16). Medscape. Retrieved from http://emedicine.medscape.com/article/1176431-overview.
- 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 from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208595/.
- West syndrome (infantile spasms). (n.d.). Epilepsy Action. Retrieved from https://www.epilepsy.org.uk/info/syndromes/west-syndrome-infantile-spasms.
- 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.