http://www.raredr.com/news/cannabis-lennox-gastaut-syndrome
Managing Seizures in Children With Lennox-Gastaut Syndrome with Cannabis Preparation

Christina Loguidice


Lennox-Gastaut syndrome (LGS) is a severe childhood epileptic encephalopathy that is estimated to affect 15 per 100,000 persons. The syndrome is associated with multiple seizure types, including tonic, atonic, tonic clonic, myoclonic, and absence seizures. Some patients may also experience focal or partial seizures. Treatment with antiepileptic medications is often not sufficient for controlling the seizures; thus, there is a great need for more effective therapies. Cannabidiol has been viewed as one potential candidate based on favorable anecdotal reports for other pediatric epilepsies, such as Dravet syndrome. During the American Epilepsy Society’s 2014 Annual Meeting, researchers from UCLA reported the results of a survey-based study that showed that the agent might have a role in treating this patient population, but that considerably more research is needed to establish its safety and efficacy before it can be recommended.
 
The study surveyed 53 parents of children (median age, 3.6 years) with LGS or infantile spasms who had administered cannabidiol-enriched cannabis preparations to their children. The survey focused on perceived efficacy, dosage, and tolerability. Of the parents surveyed, 92% reported a reduction in their children’s seizure frequency and 13% reported complete freedom from seizures. The majority of parents (89%) reported that changes in seizures (improvement or worsening) occurred within 2 weeks of administering the cannabis preparation, which most often had a cannabidiol to tetrahydrocannabinol (the main psychoactive component of marijuana) ratio of 15 to 1.
 
Before starting treatment with the cannabis preparations, the parents reported that their children, on average, had previously tried 8 other medications without success, including hormonal therapies (62%) and vigabatrin (73%). Another 57% reported no benefits with a ketogenic diet. The parents also reported that their children had fewer side effects with the cannabis preparations than with the other medications (median of 5 before treatment versus a median of 1 during treatment), and they noted improvements in alertness (77%), mood  (64%), and sleep (53%) during cannabis treatment. The median length of therapy was 6.9 months. Based on the perceived benefits, 94% of parents continued to administer cannabis preparations to their children at the time of survey completion.
 
Although these reports appear favorable, the investigators note that caution is warranted in interpreting them. “From a methodological standpoint, this study is extraordinarily vulnerable to participation bias and placebo effect as our data is self-reported by parents and did not use objective measures such as [electroencephalography],” said lead researcher Raymond Zhou, research associate, UCLA Infantile Spasms Project, in an American Epilepsy Society press release. “Our hope in presenting this data is to emphasize the need for controlled clinical trials to establish safety and efficacy,” he added.

Reference

Zhou R, Jacobson C, Weng J, et al. Potential Efficacy of Cannabidiol for Treatment of Refractory Infantile Spasms And Lennox Gastaut Syndrome. Presented at: American Epilepsy Society 68th Annual Meeting; December 5-9, 2014; Seattle, WA. Abstract # 2.372.

Rare Disease Quick Facts – Lennox-Gastaut Syndrome

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