Medical Marijuana Benefits for Dravet Syndrome
Dravet Syndrome has many theorized causes for this medication-resistant seizure disorder. From a genetic-based disorder to a nerve transmission problem, the theories are as varied as cannabis has helpful qualities.
Dravet is often associated with febrile seizures – triggers which include elevated body temperatures; emotional stressors; flashing lights and excitement. Myoclonic seizures may occur. This involves involuntary muscle spasms. Some may experience seizures that last as long or longer than 30 minutes.
There are many more conditions associated with Dravet syndrome. Delayed growth; chronic infections, delayed language and speech, behavioral, cognitive and emotional development; balance and mobility stability issues; dysfunctional internal regulation of body temperature and sweating; bone growth and formation slowed and sensory integration dysfunction.
The National Institute of Neurological Disorders and Strokes estimates that up to 80 percent of cases are caused by a genetic defect essential to proper brain function. Research has shown that Dravet syndrome patients have a mutation in a specific gene (SCN1A) which occurs in the egg or sperm before fertilization. Around two years of age, this mutation becomes apparent in the child’s development. It is thought that these children don’t produce enough of a certain protein. That action holds back inhibitory compounds that would decrease the brain’s excitability. This increases seizure frequency and mortality.
Think of this whole system as a teeter-totter. The brain is in constant mediation in this tug-of-war, the up and down balance between the excitatory and inhibitory impulses is crucial. With epilepsy, one side of the teeter-totter is heavier, the excitatory pulses override the lighter inhibitory impulse side. So as the inhibitory impulses are ignored, epilepsy may occur. Frequent seizures typically indicate a permanent shift toward excitatory impulses, resulting in a diagnosis of epilepsy.
An epilepsy-related illness is FIRES or Febrile Infection-Related Epilepsy. Average onset is in children about six years of age, following a fever. Severe bouts of epilepsy and life-threatening seizures can result from this illness. One trial indicated that cannabidiols (CBDs) reduced seizure frequency of FIRES.
At a molecular level, it is possible that CBD may modulate intracellular calcium channels of brain receptors. Calcium is part of the messaging system within and between each nerve cell. The exchange of calcium into and then out of a particular cell helps a nerve signal to cross from one cell to another. By slowing down this process, it is thought that nerve signal transmission is interrupted, calming down high excitatory levels.Possible Cures?
CBDs have been found to be anticonvulsant in mice. The conjecture of how that is possible, is this: CBDs activate a cannabinoid receptor naturally found in the body, CB1. This receptor accepts cannabis- or body- supplied cannabinoids, decreasing the excitability of neurons through decreasing the release of a neurotransmitter that ups excitability.
Early research shows that medical marijuana (MMJ) may be treatment for Dravet syndrome and severe epilepsy. In a self-reported survey, 84 percent of parents agreed that MMJ dramatically reduced the number of seizures in their child. Of those parents, 11 percent said child had a complete remission of epileptic seizures and 42 percent said they saw an 80 percent reduction of seizure frequency.
Another study confirmed the above, and also noted an improvement in alertness; sleep and mood with CBD use. One other research study revealed an alarming statistic. Patients tried 12 different traditional anti-convulsant drugs before turning to MMJ as an effective remedy of various symptoms. Overmedicated America?
Currently, there are no U.S. Food and Drug Administration sanctioned drug for Dravet syndrome. GW Pharma, an U.K.-based company, has developed a high CBD medicine, Epidiolex, which has been shown to ease seizures in patients with this illness. It is a plant-based derivative that has also been shown to be possibly decrease neural transmission and excitability.
In all fairness, CBD-based compounds are in stage 2 and 3 of research trials for use in the U.S. Depending on the outcome of these trials, these extraordinary compounds are a few years away from approval..