Cannabidiol for the therapy of epilepsy in children with Angelman syndrome

In recent years, interest in CBD (cannabidiol) for medical purposes has increased significantly. One important indication is forms of epilepsy in children, who often respond very poorly to the usual antiepileptic medications. Today I got an enthusiastic email from the mother of a 5-year-old patient I treat with CBD, saying, "I hope you don't get too overrun with Angelman patients in the near future, but CBD is really the craze."

From the U.S. media and IACM Information, the free Internet newsletter of the Consortium for Cannabinoid Medication, CBD treatment for epilepsy in so-called Dravet syndrome has become most well known. Dravet syndrome is a genetic disorder associated with epileptic seizures, among other conditions. Another rare neuro-genetic disorder is Angelman syndrome. Today's email inspired me to introduce this second, even lesser-known syndrome and the benefits of CBD for this serious condition.

Angelman syndrome is characterized by severe intellectual impairment, sleep disturbances, epileptic seizures, thrashing movements, frequent laughter and smiling, and generally very cheerful behavior. The disorder is based on a mutation of the 15th chromosome. It seems that the neurons of children with this syndrome are formed normally, but do not function optimally. They speak little because their non verbal abilities to communicate are better than verbal abilities. They have difficulty maintaining physical balance, and walking is hindered by trembling of the extremities. The epileptic seizures usually begin before the age of three, but the developmental delays become apparent much earlier.

There is no cure for these children yet. The epileptic seizures can be treated with the usual antiepileptic drugs. Often, several medications must be given together. Often, children with Angelman syndrome suffer from multiple forms of epilepsy, so it is often difficult to find an effective optimal combination of medications. As children get older, the hyperactivity, poor sleep, and frequency of epileptic seizures often decrease or disappear completely. Most adults who have Angelman syndrome are able to eat steadily by themselves and perform simple household tasks. Their general health is good and their life expectancy is not much below average.

Since the usual antiepileptic drugs are often associated with severe side effects, especially when several have to be given at the same time, there is a great need for alternatives with few side effects. Some parents of children with Angelman syndrome in the U.S. have tried this non-psychotropic cannabinoid in their children as well, based on their experience with CBD in children with Dravet syndrome. The epileptic seizures of the children of both syndromes are similar. And indeed, CBD works very well against the seizures in at least some of the Angelman syndrome patients.

Today, parents of children with rare disorders are often well connected and informed on the Internet, so that word is now spreading quickly about the possibility of cannabidiol treatment in Germany as well. CBD extracts are both available on the Internet, but many parents prefer to give their children drug-grade CBD. Doctors in Germany can prescribe cannabidiol from the Frankfurt-based company THC-Pharm on a standard private prescription. Often, doses as low as 2-3 milligrams per kilogram of body weight are effective for these children.

During the 68th Annual Congress of the American Epilepsy Society, a clinical trial was presented that evaluated the efficacy of a cannabis extract (Epidiolex) from the British company GW Pharmaceuticals that is rich in CBD (cannabidiol). The study enrolled 23 patients with treatment-resistant epilepsy, specifically Dravet syndrome, with an average age of ten years. It was conducted at two epilepsy centers in New York and in San Francisco. Patients received CBD at a constant dose of 5 mg/kg body weight in addition to their current epilepsy medications. The daily dose was slowly increased until intolerance occurred or a maximum dose of 25 mg/kg body weight was reached. After three months of treatment, 39% of patients showed a reduction in seizure frequency of more than 50%. Seizure freedom occurred in three of nine Dravet patients and one of 14 patients with other forms of epilepsy. Cannabidiol is thus significantly more effective in certain forms of epilepsy than in others.