Cannabidiol (CBD) is a cannabinoid derived from the plant, Cannabis sativa.
CBD extract is diluted with a carrier oil like hemp seed oil to produce CBD oil. Being a cannabinoid, CBD mediates a wide variety of bodily responses via the receptors of the endocannabinoid system (ECS) which regulates the activity of neurotransmitters.
CBD is not psychoactive and the oil provides several health benefits by relieving pain and stress.
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Overview of Prion Disease
Prion diseases also called transmissible spongiform encephalopathies (TSEs), are a class of progressive neurodegenerative disorders affecting both humans and animals.
TSEs are characterized by long incubation periods, spongiform changes leading to neuronal loss, and immune evasion.
The causative agents, prions, induce the abnormal folding of normal cellular proteins found in the brain which leads to neuronal damage. Common examples include the mad cow disease and kuru.
Benefits of CBD in Prion Disease
The benefits of using CBD to combat prion disease is linked with its ability to confer neuroprotection. CBD helps in promoting synaptic transmission and enhances the activities of neurotransmitters. It also prevents the entry and accumulation of the misfolded prior proteins into the brain thereby increasing the lifetime of the patient.
Being a muscle relaxant, CBD oil helps in reducing muscle stiffness that is usually an outcome of the disease. It also helps in treating ataxia and movement disorders by preventing the uncontrolled contractions of several muscle groups. CBD also helps in combating fatigue by modulating serotonergic activity.
1. CBD Exerts a Neuroprotective Effect
Prion diseases involve a spectrum of fatal neurodegenerative disorders brought about by the misfolded form of a normal protein found abundantly in the brain.
The misfolded protein is responsible for the progression of the disease and accumulation of such aggregates results in large-scale neuronal loss across the brain.
A collaborative study between the Boston University School of Medicine and the University of California, Santa Cruz revealed that the pathogenic prion proteins act as a molecular on-off switch.
One part of the protein usually sends toxic signals that damage nerve cells, whereas the other end counteracts this mechanism by breaking the signal i.e. acts as a ‘brake’.
In the pathogenic state, the ‘brake’ is ineffective and nerve damage occurs. The abnormally folded proteins aggregate together forming clumps in the brain.
When this process is allowed to continue unregulated, it leads to personality changes, memory loss, and even muscular disturbances.
Extensive research has been undertaken to have been conducted to stop the loss of nerve cells by applying compounds that accelerate the healing process and prevent the progression of the disease.
Furthermore, a 2016 review conducted by the University of São Paulo studied the role of CBD in neuroprotection.
It was established that CBD reduces the damage caused by neurodegenerative conditions of prion diseases by amplifying synaptic (nerve ending) plasticity and transmission as well by stimulating neurogenesis.
Phytocannabinoids like CBD diminishes neurodegenerative damage caused by prion diseases simply by augmenting the synaptic plasticity and preventing neuronal loss.
2. CBD Prevents the Entry of Prions into the Brain
Prion diseases can occur in a number of ways.
Sometimes proteins naturally occurring in the body can spontaneously undergo misfolding to form the pathogenic aggregates but, at other times, the disease can be transmitted by the consumption of beef infected with the prion protein.
However, the incubation period can be quite long and depends upon the time when the prions enter the brain from the body cavities.
More often than not, the initial infection might be followed by a long gap wherein the prions strive to reach the nervous tissue.
The manifestation of symptoms only happens when the disease has reached an advanced stage. The neurodegenerative damage, at this point, is so severe that it is difficult to combat the disease.
A study about the progression of prion diseases at the University of Zurich tried to explain the reason behind such long incubation periods.
It was established that misfolded prions often reach the immune cells of the spleen via the gut lining.
However, the follicular dendritic cells (immune cells) of the spleen that bind the prions were nowhere near the nervous tissue.
This might provide an insight into the delay in disease progression. The incubation stage of prion disease holds the key to treatment as the misfolded proteins are yet to reach the central nervous system and cause neurodegenerative damage.
It has been found that cannabinoids like CBD prevent the entry of prions into the brain.
A 2007 animal study at the Centre National de la Recherche Scientifique showed that CBD prevented the accumulation of the prion protein in the nervous tissue by affecting their migration into the microglia.
It also prevented the neurotoxic effects of the misfolded prions and also helped in increasing the survival time of the prion-infected mice.
CBD helps in preventing accumulation of prion protein in the nervous tissue and also combats the neurotoxic effects of these aggregates.
3. CBD Oil Is A Muscle Relaxant
One of the major complications experienced by patients suffering from prion disease is muscle stiffness.
Rigidity in the muscles often cause unwarranted contractions and make everyday life difficult.
The stiffness is mostly brought about due to the death of nervous tissue which is responsible for coordinating the cycle of contraction and relaxation.
A severe outcome of muscle stiffness is difficulty in swallowing. This leads to malnutrition as patients are unable to ingest any kind of food.
In fact, in the advanced stages of the disease, tube feeding may be necessary. Speech is also impaired as muscle groups in and around the face and throat get severely affected.
While nerve tissue degeneration is one of the main reasons behind muscle rigidity, a study by the University of California, San Francisco has revealed that prions tend to aggregate in large numbers in the muscles.
The 2002 animal study showed that these misfolded proteins exhibited a preference for muscle tissue, particularly the hind limb of the test animals.
In order to relieve the rigidity, therapies should focus on approaches that allow muscle relaxation.
As cannabinoids are involved in modulating a wide range of physiological responses, alternative medical therapies with CBD have gained eminence as a muscle relaxant.
A study reviewed by the National Academy of Sciences showed that the regular use of the plant-derived CBD oil helps in relaxing the muscle groups and combating the muscle stiffness that is usually an outcome of neurodegenerative damage as well as accumulation of prions in skeletal muscle.
As CBD oil helps in relaxing the muscles and preventing neurodegenerative damage, it can help in preventing muscle rigidity and aid in swallowing and speech which are usually impaired in patients with prion disease.
4. CBD Combats Movement Disturbances
As a direct consequence of muscle stiffness, prion diseases also result in movement disturbances.
This is due to the uncontrolled muscle contractions caused due to the neuronal loss and lack of muscular coordination via nervous control. Initially, the problem is limited to a disturbance in overall balance and gait.
As the disease progresses and nerve cells degenerate at a rapid rate, ataxia or unsteadiness leading to loss of bodily control is developed.
Walking becomes quite difficult and special care must be taken to prevent the patient from suffering sudden falls.
Prion aggregation and neurodegeneration also leads to the increased involuntary rhythmic muscle contraction or myoclonus.
The spasmodic twitching of certain muscle groups can increase the difficulty in coordinating movements, particularly that of the hands. Such uncontrolled movements increase clumsiness and cause difficulties in regular lives.
Shakiness or muscle tremors are often experienced by patients which further aggravate the problems of ataxia.
Movement disturbances severely disrupt normal lives and hence, finding therapies that help in regaining control and coordination are active areas of medical research. CBD, as mentioned earlier, has shown potential in treating such movement disorders.
A 2015 review by the University of Colorado School of Medicine evaluated the role of CBD in treating movement disorders.
It was established that CBD oil helps in combating problems like ataxia due to its nature as a cannabinoid. Such compounds activate the endocannabinoid system (ECS) that mediates neurotransmitter activity and enhances motor function.
As CBD oil helps in mediating motor function via the ECS, it helps in treating movement disturbances brought about by prion disease.
5. CBD Oil Treats Fatigue
One of the earliest symptoms of prion disease is chronic fatigue.
Usually, the major symptoms take much longer to manifest themselves and indicate late-stage nerve damage.
However, fatigue is often associated in the initial phase and can be both physical and mental. Muscle fatigue is caused due to the damage to skeletal and involuntary muscle groups.
Fatigue is usually a constant feeling of drowsiness characterized by severely low energy levels. While mild tiredness is usually normal, chronic fatigue is an indicator of a deeper underlying cause.
Dietary imbalances, excessive physical exhaustion, and inadequate sleep are usually the precursors but, on rare occasions, fatigue is also a symptom of nerve damage.
Aggregation of misfolded prion proteins in the central nervous system often affect neurotransmitter activity which is responsible for mediating a wide range of physiological responses.
Consistent neuronal loss often inhibit s the activity of significant neurotransmitters like serotonin and dopamine resulting in low energy levels.
Excessive muscle stiffness and uncontrolled twitching (myoclonus) result in muscular fatigue.
Prion diseases cause ataxia and related movement disorders which are often the result of severe muscle contraction. Prolonged jerks and contractions are also responsible for fatigue.
Several studies have been conducted to develop medical therapies to relax the body and stimulate neurotransmitter activity to combat the fatigue caused by such neurodegenerative diseases.
CBD has come to the forefront on account of its nature as a muscle relaxant and cannabinoid.
A 2010 review by the Innsbruck Medical University has shown that phytocannabinoids like CBD help in stimulating serotonergic activity.
This helps in combating fatigue and low energy levels. As CBD oil also helps in relaxing muscle contractions, it prevents the development of muscular fatigue.
As CBD oil helps in stimulating serotonergic activity and helps in treating muscle rigidity, it can be inferred that CBD combats fatigue felt by patients suffering from prion diseases.
A healthcare provider should be consulted before beginning any medication containing THC.
The dosage depends on the mode of delivery as the CBD products can be orally ingested or inhaled by a vaporizer.
There is no prescribed dosage for using CBD oil to treat prion diseases as neurodegenerative diseases are yet to be cured completely.
To relieve symptoms like fatigue and muscle stiffness as well as to delay disease progression, small doses of 90-100mg should be initiated and the optimum dose should be decided according to the individual’s tolerance level.
CBD is known to have very few side-effects and can be consumed regularly. Oral doses of 500mg can be tolerated daily for as long as 6 months without any unwanted complications.
CBD products also contain small amounts of the psychotropic agent, tetrahydrocannabinol (THC) which is mostly responsible for the side-effects.
While the concentration of THC is quite harmless, sensitive individuals might be susceptible to its hallucinatory effects and hence, it is always recommended to start with small doses.
CBD inhibits the activity of cytochrome p450, a class of liver enzymes responsible for metabolizing drugs and hence, CBD usage might lower drug efficacies.
Products which contain indiscriminate amounts of THC might cause dizziness, confusion, disorientation, nausea, fatigue, impaired balance, hallucinations, and paranoia.
THC-containing medications might also cause tachycardia which is a condition wherein the heart rate is substantially raised for a short duration.
Cannabis products containing excessive amounts of THC should also not be accompanied by alcohol.
Pregnant and lactating women should consult a gynaecologist before consuming CBD products. Caution should also be maintained while administering THC-containing products to children and adolescents as it might affect their CNS differently.
To summarize, it can be said that CBD confers several neurological benefits in combating prion disease.
It helps in protecting the nervous system from neurodegeneration and enhances synaptic plasticity.
CBD also prevents the entry and accumulation of misfolded aggregates of prion proteins in the brain and increases the survival time of the individual.
As a muscle relaxant, CBD oil helps in preventing muscle stiffness and rigidity which, in turn, helps in swallowing and speech.
As an extension of this benefit, it also helps in treating movement disorders like ataxia by reducing the uncontrolled contractions of muscle groups brought about by prion aggregates. Finally, it helps combat fatigue by stimulating neurotransmitter activity.