Feature Image

West 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.

West syndrome is a type of severe pediatric epilepsy that affects one baby out of a few thousand in the US1. Studies have shown CBD-enriched cannabis is 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 years[1].

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 times[2]. 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 colic[3].

According to the Epilepsy Foundation, about two thirds of babies with West syndrome have some known cause for the seizures[1]. 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 syndrome[1].

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 autism[2]. Most children with West syndrome, even if the spasms cease, will eventually develop other kinds of epilepsy like Lennox-Gastaut syndrome[1].

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 necessary[1].

Findings: Effects of Cannabis on West Syndrome

Several studies have found that cannabidiol (CBD), a major cannabinoid found in cannabis, can reduce or even eliminate seizures[4,5,11,12].

These results are also found in severe pediatric epilepsies like West syndrome, Dravet syndrome, Doose syndrome, and Lennox-Gastaut syndrome. In one questionnaire study, CBD-enriched cannabis reduced seizure frequency in children with West syndrome and Lennox-Gastaut syndrome by 85 percent, while 14 percent experienced complete seizure freedom. They also reported an improvement in sleep (53 percent), alertness (71 percent), and mood (63 percent)[8].

Another survey found that 85 percent of parents reported that cannabis treatment caused a reduction in their child’s seizure frequency. Out of those parents, 11 percent of them responded that their child has reached complete seizure freedom, while 42 percent reported a greater than 80 percent reduction in seizure frequency. The parents also reported an increase in alertness, better mood, and improved sleep[10].

One case report inspecting a young epileptic girl found that cannabis treatments reduced 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 antiepileptic drugs she had been taking[9].

CBD’s capability to reduce or eliminate seizures is due to its activation of the cannabinoid receptor 1 (CB1). When stimulated, the CB1 receptor dampens neurotransmission and produces an overall reduction in neuronal excitability[7,13].

Traditional medicines used to treat pediatric epilepsy often come with a number of adverse side effects. Preclinical and preliminary human studies have reported that cannabis treatments produce fewer adverse effects that other antiepileptic drugs[6]. 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 cannabis[10].

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

  • CBD-enriched cannabis reduced seizure frequency in 84% of children with West syndrome (infantile spasms) and Lennox-Gastaut syndrome, 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/)
  • CBD-enriched cannabis reduced frequency of seizures in 85% of children, while 14% reported complete seizure freedom. Children also experienced 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)

References:

1. Infantile Spasms / West’s Syndrome. (2015, December). Epilepsy Foundation. Retrieved from http://www.epilepsy.com/learn/types-epilepsy-syndromes/infantile-spasms-wests-syndrome.

2. Infantile Spasm (West Syndrome). (2014, October 16). Medscape. Retrieved from http://emedicine.medscape.com/article/1176431-overview.

3. West syndrome (infantile spasms). (n.d.). Epilepsy Action. Retrieved from https://www.epilepsy.org.uk/info/syndromes/west-syndrome-infantile-spasms.

4. Blair, R.E., Deshpande, L.S., and DeLorenzo, R.J. (2015, September). Cannabinoids: is there a potential treatment role in epilepsy? Expert Opinion on Pharmacology, 16(13), 1911-4. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4845642/.

5. Devinsky, O., Cilio, M.R., Cross, H., Fernandez-Ruiz, J., French, J., Hill, C., Katz, R., Di Marzo, V., Jutras-Aswad, D., Notcutt, W.G., Martinez-Orgado, J., Robson, P.J., Rohrback, B.G., Thiele, E., Whalley, B., and Friedman, D. (2014, June). Cannabidiol: pharmacology and potential therapeutic role in epilepsy and other neuropsychiatric disorders. Epilepsia, 55(6), 791-802. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4707667/.

6. dos Santos, R.G., Hallak, J.E., Leite, J.P., Zuardi, A.W., and Crippa, J.A. (2015, April). Phytocannabinoids and epilepsy. Journal of Clinical Pharmacology and Therapeutics, 40(2), 135-43. Retrieved from http://onlinelibrary.wiley.com/doi/10.1111/jcpt.12235/full.

7. Hoffman, M.E., and Frazier, C.J. (2013, June). Marijuana, endocannabinoids, and epilepsy: potential and challenges for improved therapeutic intervention. Experimental Neurology, 244, 43-50. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3332149/.

8. Hussain, S. A., Zhou, R., Jacobson, C., Weng, J., Cheng, E., Lay, J., Hung, P., Lerner, J.T., and Sankar, R. (2015, June). Perceived efficacy of cannabidiol-enriched cannabis extracts for treatment of pediatric epilepsy: A potential role for infantile spasms and Lennox-Gastaut syndrome. Epilepsy & Behavior, 47, 138-41. Retrieved from http://www.epilepsybehavior.com/article/S1525-5050(15)00157-2/fulltext.

9. Maa, E. and Figi, P. (2014, June). The case for medical marijuana in epilepsy. Epilepsia, 55(6), 783-6. Retrieved from http://onlinelibrary.wiley.com/doi/10.1111/epi.12610/full.

10. Porter, B.E., and Jacobson, C. (2013, December). Report of a parent survey of cannabidiol-enriched cannabis use in pediatric treatment-resistant epilepsy. Epilepsy & Behavior, 29(3), 574-7. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4157067/.

11. Rosenberg, E.C., Tsien, R.W., Whalley, B.J., and Devinsky, O. (2015, August 18). Cannabinoids and Epilepsy. Neurotherapeutics, Epub ahead of print. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4604191/.

12. Szaflarski, J.P., and Bebin, E.M. (2014, December). Cannabis, cannabidiol, and epilepsy–from receptors to clinical response. Epilepsy & Behavior, 41, 277-82. Retrieved from http://www.epilepsybehavior.com/article/S1525-5050(14)00413-2/fulltext.

13. Wallace, M.J., Wiley, J.L., Martin, B.R., and DeLorenzo, R.J. (2001, September 28). Assessment of the role of CB1 receptors in cannabinoid anticonvulsant effects. European Journal of Pharmacology, 428(1), 51-7. Retrieved from http://www.sciencedirect.com/science/article/pii/S0014299901012432.

  • February 5, 2013
  • Jeffrey Stamberger