In anticipation of Global Motor Neuron Disease Day on 21 June 2023, communities worldwide affected by Motor Neuron Disease (MND) and Amyotrophic Lateral Sclerosis (ALS) are coming together to amplify the understanding of this debilitating and incurable condition. This collective effort aims to generate greater awareness and offer support to patients, caregivers, and families affected by MND/ALS.
Many people might remember the Ice Bucket Challenge but not exactly the message behind it. It remains the world’s most significant social media phenomenon, urgently moving millions of people to action. Three young men with ALS conceived the challenge in August 2014 to inspire their communities, celebrities, and the world to dump buckets of ice water on their heads to fight the disease. Over $220 million was donated to ALS charities worldwide to fight the disease.
As no cure has yet been found, recent clinical studies have shifted their attention towards slowing the progression of the disease and improving the quality of life of patients.
In line with these advances in the management of neurodegenerative diseases, Azela Health, a South African company specializing in clinical nutritional products for specific medical purposes, has partnered with Rigi Care, a Swiss medical research organization, to introduce Glutamed®ND. A novel, cost-effective, and accessible medical and nutritional product, it has specifically been formulated for patients with neurodegenerative diseases such as MND and ALS, Multiple Sclerosis, and Parkinson’s Disease.
Glutamed®ND contains a blend of pharmaceutical-grade Ursodoxicoltaurine (TUDCA), Butyrates, Trehalose, and L-Glutamine, all of which have shown promising results in recent and current clinical research.
ALS (Amyotrophic Lateral Sclerosis), or Motor Neuron Disease, is a rare neurological disease affecting motor neurons. These nerve cells in the brain and spinal cord control voluntary muscle movement. Voluntary muscles are those we choose to produce activities like chewing, walking, and talking. It is estimated that more than 200 000 patients with MND worldwide. Late Springbok rugby legend Joost van der Westhuizen, world-renowned physicist Stephen Hawking, and more recently, former Scotland rugby player Doddie Weir succumbed to MND.
Regarding Multiple Sclerosis (MS), there are today over 2.5 million people around the world that have MS.
With its focus on beneficial nutrients that might make a difference, Glutamed®ND’s unique formulation consists primarily of the following:
- L-Glutamine
Glutamine is an amino acid that plays an important role in numerous physiological processes, including maintaining gut health, immune system function, and muscle protein synthesis. Muscle wasting is a condition characterised by the loss of muscle mass, strength, and function, often observed in diseases such as cancer, sepsis, and MND. This especially occurs in diseases such as cancer, sepsis, and MND. Glutamine has also been studied for its potential effects on the gut-brain axis.
A major energy source for the cells lining the gastrointestinal tract, glutamine is therefore vital for maintaining the integrity of the gut barrier, which helps prevent harmful substances from entering the body.
Additionally, research suggests that glutamine has anti-inflammatory and immune-modulatory properties. It can help reduce inflammation in the body and protect tissues and organs against inflammation-related damage. Glutamine can act as a precursor for glutathione, an essential antioxidant that protects cells from oxidative stress and damage.
- TUDCA
Tauroursodeoxycholic acid (TUDCA) is a hydrophilic bile acid that is normally produced endogenously in humans in the liver, by conjugation of taurine to ursodeoxycholic acid (UDCA). It is commonly used for treating chronic cholestatic liver diseases and gallstones.
TUDCA is orally bioavailable and blood-brain barrier permeable. Upon oral administration, TUDCA is absorbed in the intestines and subsequently taken up in the liver before being excreted directly into the bile.
Bile acids, including TUDCA, play a role in intestinal homeostasis by regulating the size and composition of the intestinal microbiota. Clinical studies have demonstrated that the chronic administration of bile acids is safe and well-tolerated.
Regarding MND/ALS, TUDCA is thought to exert an effect by protecting nerve cells from dying, modifying the body’s immune system, and neutralising the effects of harmful oxidative substances. Reducing damage to nerve cells may slow down the worsening of symptoms.
The large amount of available research showing the beneficial effects of this bile acid in neurodegeneration models indicates that TUDCA is a candidate with great potential in the treatment of these neurodegenerative illnesses.
- Trehalose
Trehalose is a naturally occurring sugar that serves as an energy source and has potential benefits for maintaining stable blood glucose levels, improving insulin sensitivity, and reducing the risk of insulin resistance and type 2 diabetes. Additionally, Trehalose possesses natural antioxidant properties, which can help in reducing oxidative stress and inflammation.
Trehalose is of current interest in the field of neurodegenerative conditions, as it shows the ability to stabilise proteins, thereby alleviating the accumulation of misfolded proteins that can be toxic. Moreover, Trehalose facilitates autophagy, a natural ‘cleaning’ process in the body that facilitates the orderly breakdown and recycling of unnecessary, dysfunctional, or harmful cell components.
- Butyrates
There are three types of “Butyrates” or short-chain fatty acids (SCFA’s) in Glutamed®ND, namely propionate, butyrate, and hydroxy-methyl-butyrate (HMB). Where the first two are naturally produced in the colon by certain types of bacteria when they break down dietary fibre, the latter is produced from the breakdown of the amino acid, leucine.
Butyrates have been shown to have numerous health benefits, including promoting gut health, reducing systemic inflammation and preserving the integrity of the blood-brain barrier.
Studies conducted on animal models and cell cultures have suggested that butyrates may have neuroprotective effects on MND by promoting the survival of motor neurons and improving mitochondrial function. In a recent UK clinical study in Multiple Sclerosis patients, propionate was seen to significantly improve gait function. Hydroxy-methyl-butyrate (HMB). has been shown to reduce loss of muscle mass, which is of particular concern to MND patients.
Additionally, Glutamed®ND contains essential minerals and trace elements that are beneficial to patients with chronic neurological conditions. These are all combined into a convenient single-serving 25g sachet for ease of use and compliance. It can be administered enterally or orally.
While access to new interventions for neurodegenerative diseases can be costly and unaffordable for many patients, especially in lower socio-economic regions, local manufacturing in South Africa in accordance with internationally prescribed GMP manufacturing guidelines, will ensure that the product remains accessible and affordable worldwide for those who need it most.
MND/ALS patients are looking for the best combination of interventions for managing their devastating condition, and cost should not be a hindrance. The new products on the horizon are very promising but clearly unaffordable for many patients. “In developing Glutamed®ND, we have succeeded in combining several high-potential compounds into a single-dose product that is accessible and affordable,” says Dr Jørgen Thorball MD and Managing Director of Rigi Care.
Glutamed®ND is available to purchase online at www.glutamednd.net, where comprehensive additional information is provided. Alternatively, it can be ordered directly via e-mail info@azelahealth.co.za.
Creating awareness may help identify undiagnosed patients
The collective effort to disseminate information about MND aims to generate greater awareness. It is thought that MND may be underdiagnosed in South Africa. According to statistics, the prevalence in our country is estimated at 1 per 100 000 people, while the prevalence in Europe and North America has been shown to be 3.37 per 100 000 people. Any number of reasons could explain this discrepancy. However, it may also suggest underdiagnosis.
In addition, an analysis of the available information about MND in 204 countries and territories clearly shows a rise in the incidence of MND from 1990 to 2019. Between 2000-3000 people have MND. The true figure could be two to three times higher. This indicates that many South Africans are not diagnosed. They may miss out on appropriate medical help and support.
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