Although the label Lennox-Gastaut is often broadly attached to severe epilepsies with compatible seizure types and intellectual disabilities, it is important that there is a clear syndromic diagnosis.
People always have the option of seeking a second opinion.
Guidance from the Association of British Neurologists (ABN)
On 1 November 2018, the Government's landmark decision to reschedule some cannabis based products for medicinal use, came into force. The change in law means that specialist doctors in the UK can now prescribe medicinal cannabis to people with a limited number of conditions, including epilepsy. Here we explain what the change in law means for people with epilepsy.
“Clinicians should not feel under pressure to prescribe cannabis-based medicinal products until they have undergone proper clinical trials,” says the BPNA.
THC is the psychoactive compound in cannabis. It is responsible for the “high” people feel. The legal limit of THC content in a product, as stipulated by the Home Office, is 0.2%.
A list of targets and actions reported for CBD based on results of studies in different experimental models and systems24–36
In the second trial, 225 patients with Lennox-Gastaut syndrome (mean age 16 years, median number of drop seizures per month at baseline 85) were randomised to three groups and allocated to two doses of CBD (10 or 20 mg/kg/day) or placebo.124 Enrolled patients were receiving a median of 3 concomitant AEDs. Duration of the trial was 14 weeks (2-week titration and 12-week maintenance). The reduction in monthly frequency of drop seizures was significantly greater in the CBD 20 mg/kg group (42%) and 10 mg/kg group (37%) than in the placebo group (17%; p = 0.0047 and 0.0016, respectively, Fig. 4 ). The proportion of patients with a ≥ 50% decrease in drop seizure frequency was also significantly greater in the 20 and 10 mg/kg groups (40% and 36%, respectively) than in the placebo group (15%; p = 0.0006 and p = 0.0030, respectively). Total seizures were also significantly reduced in both CBD groups compared with placebo. Adverse events were reported in 94% of patients allocated to 20 mg/kg, 84% of those allocated to 10 mg/kg, and 72% of placebo patients, the most common being somnolence and decreased appetite. Serious treatment-related adverse events occurred in five patients in the 20 mg/kg group, two patients in the 10 mg/kg group, and no patients on placebo patients. Some elevations in transaminases were seen. Of 212 completers, 99% entered an open-label extension study.
In the cannabis plant, most cannabinoids are synthesized in their acidic form. These acidic cannabinoids undergo decarboxylation to their neutral counterparts (e.g., CBD and THC) under the influence of auto-oxidation, light and heat. In most common extraction and delivery methods, plant materials are exposed to heat, resulting in the conversion of the acidic forms to the neutral constituents.60 However, some cannabis products may retain some content in acidic cannabinoids, particularly cannabidiolic acid (CBDA) and 9-Δ-tetrahydrocannabinolinic acid (THCA). THCA has been found to possess anticonvulsant activity in preliminary preclinical investigations, and be devoid of adverse psychoactive effects.61 Interest in THCA been rekindled recently by the fact that it is sometimes used for anti-seizure purposes in the USA, where it can be more readily available and/or affordable than CBD. One specific concern is that accidental exposure to heat of artisanal THCA preparations may result in partial conversion to THC.61
The genus Cannabis refers to a flowering plant of which there are three main species, Cannabis sativa, Cannabis indica and Cannabis ruderalis. These plants contain over 100 biologically active chemicals called cannabinoids, with the most abundant and best characterized among those being THC and CBD ( Fig. 2 ).7 Crude preparations of cannabis include dried leaves, stems and flower pods (marijuana), resins (hashish), and oily extracts (hashish oil), all of which have been used through the centuries mostly for their psychoactive properties. In general, cannabis products derived from Cannabis sativa exhibit a higher CBD/THC ratio than products derived from Cannabis indica. Different Cannabis strains have been bred either to maximize THC content or, conversely, to reduce THC content and increase the concentration of CBD and other non-psychoactive ingredients.8
The first studies on the medical use of cannabis date back to the Chinese Emperor Shen Nung (about 2,700 B.C.).2 As evidence of the important role of the plant in ancient Chinese culture, archeological excavations in the Xinjiang-Uighur Autonomous Region of China have recently unearthed a 2,700-year-old grave of a shaman which contained a large cache of cannabis, perfectly preserved by climatic and burial conditions, presumably employed as a medicinal or psychoactive agent, or as an aid to divination.3 Early written records of medical applications can be traced to Sumerian and Akkadian tablets, around 1,800 B.C., which mention the use of a medicinal plant, most likely cannabis, to treat a variety of ailments, including nocturnal convulsions.2,4 In less ancient times, there are records in the Arabic and Islamic literature which mention explicitly cannabis as a treatment for seizures and epilepsy.4