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Overview of Lennox- Gastaut Syndrome

Lennox-Gastaut syndrome is a severe form of epilepsy that typically develops before the age of four. The syndrome typically causes impaired intellectual development and limits information processing, causing behavioral problems. While the cause of Lennox-Gastaut syndrome can’t always be determined, it many cases, it be attributed to either brain malformations, perinatal asphyxia, severe head injury, central nervous system infection and inherited degenerative or metabolic conditions.

The type of seizures caused by Lennox-Gastaut syndrome can include tonic (body stiffens, eyes deviate upward, pupils dilate, respiratory patterns become altered), atonic (brief loss of consciousness and muscle tone, abrupt falling), atypical absence (spell of staring), and myoclonic (sudden muscle jerks). The Epilepsy Foundation, however, notes that tonic and atonic seizures are most common in Lennox-Gastaut syndrome.

There is no cure for the disorder and complete recovery and freedom from seizures is unusual. It’s rare for anti-seizure medications to be effective in individuals with Lennox-Gastaut syndrome. In addition, the National Institute of Neurological Disorders and Stroke note that children that respond positively to a drug can later show tolerance and resume their uncontrollable seizures.

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Findings: Effects of Cannabis on Lennox- Gastaut Syndrome

A number of scientific reviews have concluded that a major cannabinoid found in cannabis, cannabidiol (CBD) is a well-tolerated and promising therapeutic treatment that has demonstrated the ability to reduce or even eliminate seizures (Blair, Deshpande & DeLorenzo, 2015) (Cunha, et al., 1980) (Filloux, 2015) (Hosseinzadeh, et al., 2016) (Rosenberg, Tsien, Whalley & Devinsky, 2015) (Szaflarski & Bebin, 2014) (Devinsky, et al., 2014) (Jones, et al., 2010) (Jones, et al., 2012) (Detyniecki & Hirsch, 2015) (Cilio, Thiele & Devinsky, 2014). Tetrahydrocannabinol (THC) has also demonstrated efficacy at reducing seizures in children with epilepsy (Lorenz, 2004). Another cannabinoid found in cannabis, cannabidivarin (CBDV) has also shown to have non-psychoactive anticonvulsant effects (Amada, Yamasaki, Williams & Whalley, 2013) (Hill, et al., 2012). In a preclinical trial, the administration of cannabis provided significant anticonvulsant effects in mice and rats (Hill, et al., 2013).

The capability of CBD to reduce or eliminate seizures is due to its effects on the body’s endocannabinoid system (Wallace, et al., 2003). CBD activates cannabinoid receptor 1 (CB1); The CB1 receptor then dampens the release of neurotransmitter and produces an overall reduction in neuronal excitability (Blair, et al., 2006) (Wallace, Wiley, Martin & DeLorenzo, 2001) (Hoffman & Frazier, 2013) (Wallace, Martin & DeLorenzo, 2002) (Karlócai, 2011).

Although double-blind randomized, placebo-controlled studies are currently lacking, early research suggests that cannabis is effective in the treatment of severe pediatric epilepsy disorders like Lennox-Gastaut syndrome. In one questionnaire study, 84% of parents reported a reduction in their child’s seizure frequency following cannabis treatment. Out of those parents, 11% of them responded that their child has reached complete seizure freedom, while 42% reported a greater than 80% reduction in seizure frequency. The parents also reported additional beneficial effects, such as increased alertness, better mood and improved sleep (Porter & Jacobson, 2013). Another similar survey found that CBD-enriched cannabis brought about a reduction in seizure frequency in 85% of children with Lennox-Gastaut syndrome, while 14% experienced complete seizure freedom. The children also reported an improvement in sleep (53%), alertness (71%), and mood (63%) while being treated with CBD (Hussain, et al., 2015).

Another study examining the effect of CBD-enriched medical cannabis on children with epilepsy found that 89 percent of children reported a reduction in seizure frequency with CBD treatment. The children also reportedly saw improvements in behavior and alertness, language, communication,motor skills and sleep (Tzadok, et al., 2016).

One case report analyzing a young girl with Dravet syndrome, another severe childhood epilepsy disorder, found that medical marijuana brought the child’s seizure frequency from nearly 50 convulsive seizures per day to 2-3 nocturnal convulsions per month. In addition, the child was able to wean from the additional antiepileptic drugs she had been taking (Maa & Figi, 2014).

Traditional medicines used to treat epilepsy are not just ineffective for most; they often come with a number of adverse side effects. Cannabinoids found in cannabis, however, have shown to produce anticonvulsant effects in preclinical and preliminary human studies while producing fewer adverse effects that other antiepileptic drugs (dos Santos, et al., 2015). A questionnaire study found that parents tried an average of 12 different antiepileptic drugs, due to ineffectiveness or unacceptable side effects, before finding gentle effectiveness with cannabis (Porter & Jacobson, 2013).

 

References:

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