Over the years, there has been considerable discussion of whether the seizure disorder associated with MEP2 Duplication Syndrome should be categorized as Lennox-Gastaut syndrome. Up until very recently, this has been mostly an academic discussion, but a recent development means that this argument may have important practical implication for individuals with MECP2 Duplication Syndrome and their families. On June 25, 2018, the FDA approved Cannabidiol treatment for individuals with Lennox-Gastaut, (and Dravet syndromes) and having a diagnosis of Lennox-Gastaut may affect availability and cost to individuals MECP2 duplication syndrome. So, here are some facts to consider. Continue reading
THANK YOU TO THE GOVERNMENT OF CANADA FOR LISTENING TO THIS POST AND ALL OF THE OTHER VOICES ASKING FOR EXEMPTION OF CBD! This year the government agreed and exempted CBD.
As currently proposed, the Canadian Cannabis Excise Tax will create an excessive economic burden on many sick and disabled Canadians. In many cases, it will add an additional $1000 or year or more to the existing federal taxes. The stated rationale for this tax is that recreational users may claim to be medical users in order to avoid the excise tax on recreational marijuana. This rationale, however, cannot be appropriately applied to Cannabidiol (CBD) oil, because CBD is not psychotropic, and CBD oil has no potential for recreational use. Therefore, adding an excise tax to CBD oils that contain only small amounts of THC, serves no purpose other than to create an additional financial burden of sick and disabled Canadians.
If you agree, please e-mail the Department of Finance Canada at firstname.lastname@example.org and tell them:
Exempt CBD oils that contain less than 3mg/ml of THC from the Canadian Cannabis Excise Tax. Taxing Low-THC CBD oils is unfair and unnecessary.
The Department of Finance will accept comments on the proposed tax until 7 December 2017 and you can view the entire proposal here: http://www.fin.gc.ca/n17/data/17-114_1-eng.asp
Let the Canadian Department of Finance know what you think about the proposed excise tax. Feel free to quote or draw on any part of this blog post, if that is helpful. Also feel free to share this post with anyone who may be interested.
For more details on why CBD should be exempted, see below:
About me I want to be clear about the fact that I have a personal stake in this matter. Our son’s doctor has prescribed CBD oil to help manage intractable epilepsy Continue reading
This week the New England Journal of Medicine published a double-blind study on the effectiveness of Cannabidiol (CBD) on seizures demonstrating that it was safe and effective for kids whose seizures could not be effectively controlled with typical anticonvulsants. The publication of the study set off a media storm with the results reported in hundreds of newspapers and on electronic media. The actual study focused on individuals with a Dravet syndrome diagnosis and so it is difficult to know how well it can be applied to individuals with MECP2 duplication. Nevertheless, there are some interesting findings and likely impacts with potential implications. Continue reading
Drop seizures are extremely common among individuals with MECP2 duplication syndrome. There is a sudden loss of muscle tone, and if the individual is not supported, he or she drops suddenly to floor, sometimes resulting in injury. Usually these are brief and it is over in a second, but sometimes they come in clusters.
While these drops are certainly one of the most, if not the absolute most, common seizures in MECP2 duplication syndrome, there are actually two kinds of “drops” that look pretty much the same, but are fundamentally very different. It is hard to tell them apart, and at least some individuals with MECP2 duplication syndrome, have both kinds. It may be helpful to figure out which kind is occurring, but it can be really hard to figure out, and it appears that at least some individuals experience both atonic and myoclonic drops.
So what’s the difference? ATONIC VS MYOCLONIC-ATONIC Continue reading
Research published in December 2016 provides a bit more information about seizure disorders in MECP2 duplication syndrome, particularly as they compare to seizure disorders in Rett syndrome. In general, this is a great piece of research as it describes the course of seizure disorders in Rett syndrome over a period of years. It emerged from the earlier natural history study. Unfortunately from the the perspective of MECP2 duplication syndrome, this publication is a bit disappointing, not because of any weakness in the sturdy or the reporting, but merely because the number of participants with MECP2 duplication and the number of observations in the study was relatively small, which only permitted a very limited picture of this group. If there is a lesson for the MECP2 duplication syndrome study to be learned from this study, it may be the importance of participation in the current natural history study in order for researchers to be able to glean useful information.
Nevertheless this study did briefly report some general observations relevant to MECP2 duplication syndrome:
This post expresses my personal opinion. It is merely a theory for discussion and possibly a hypothesis that will or will not be supported by evidence over time. Simply stated, my theory is:
A: There are different kinds of seizure disorders.
B. It is possible for an individual to have more than one kind of seizure disorder.
C. Individuals with MECP2 Duplication Syndrome often have multiple seizure disorders. Continue reading
Several new studies provide encouraging results about the use of cannabidiol (CBD) oil to treat seizure disorders that are not controlled by other medications. Research also, however, points toward important cautions.
Already in 2016,at least nine studies have been published on CBD and seizures. Two 2016 studies, one from Israel (Tzadok et al.,, 2016) and one the United States (Devinsky et al., 2016) are generally reporting particularly encouraging results. This is not to say the others are negative, just less relevant. The two discussed here are important because they include some results Continue reading
A few days ago, I posted a video (Drop Seizure Video) on this blog. It showed a girl having “drops” or what the video caption called “infantile spasms.” A large number of MECP2 Duplication Syndrome family members agreed that although their affected family members had many kinds of seizures, this looked a lot like what some of their drops look like. I definitely found this very interesting, since infantile spasms are generally described as starting before one year of age, and are very rare in older children or adults.
Thanks to the internet, I was able to find out more. I got some great leads from two colleagues in Kyoto Japan and Doha Qatar Continue reading
This YouTube video looks a lot like what has been described as a drop in individuals with individuals with MECP2 Duplication Syndrome. The individual in this video does not have MECP2 Duplication Syndrome, but the video provides a good example of what some drops look like.
The text below is the original text accompanying the video on YouTube. Continue reading
Seizures present a major problem for individuals with MECP2 Duplication Syndrome. Most, if not all, will develop seizures at some point in their lives, and their seizures are typically extremely difficult to treat with conventional anticonvulsants. Some families have attempted to treat seizures with Cannabidiol (CBD) after widespread publicity has touted its apparent effectiveness in treating seizures in children and adults with uncontrolled epilepsy. Officially the verdict is still out on its effectiveness, but the good news is that their is now a flood of new scientific reviews and studies emerging and generally it is quite supportive of CBD. Listed here are a few recent publications and a brief summary of relevant findings.
Hussain, S. A., Zhou, R., Jacobson, C., Weng, J., Cheng, E., Lay, J., et al. (2015). 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, 138-141.
This was a survey of 117 parents Continue reading