Have you or someone you love been diagnosed with a motor neuron disease? If so, that can bring in a lot of scary feelings—I know from personal experience.
What is Motor Neuron Disease?
Motor Neuron Disease: a progressive degenerative disorder that leads to muscle weakness and the deterioration of motor neurons. Symptoms can vary from person to person, ranging from slurred speech and difficulty swallowing to muscle spasms and paralysis.
Diagnosis is usually based on symptoms since most cases of ALS (one type of MND) are not caused by genetic mutations.(5)
Diseases Included
- Amyotrophic Lateral Sclerosis (ALS)
- Progressive Bulbar Palsy (PBP)
- Pseudobulbar Palsy
- Progressive Muscular Atrophy (PMA)
- Primary Lateral Sclerosis (PLS)
- Spinal Muscular Atrophy (SMA)
- Monomelic Amyotrophy (MMA)
What Causes Motor Neuron Disease?
The causes of motor neuron disease are not fully understood, but it is an autoimmune disorder. This is what happens when the immune system starts attacking the body—in essence, an insurrection. The process involves neuroinflammation, which causes breakdowns. (4)
Remember the breach of the capitol on January 6, 2021? What would have happened if the military got involved in a coup to overthrow the government? That’s precisely what is happening in the body.
When lymphocytes and macrophages (the branch of the immune system for the body) infiltrate the blood-brain barrier, this can activate microglia and reactive astrocytes (the branch of the brain), triggering neuroinflammation. (4)
Famous People with Motor Neuron Disease
Wilhelmina Terpstra
(1940-2006)
She is also known by her nickname, ‘Willie’. This woman had outstanding courage. She was from Rotura in New Zealand.
She actually traveled to China for a controversial fetal cell (a.k.a stem cell) operation, hoping it would restore her speech and give her life back.
"She never once complained about her fate and fought an unbelievable fight.” — Nz Herald 11/21/2006 “Laughter, tears at funeral of inspirational battler”
Lou Gehrig
(1903–1941)
American baseball player (AKA the Iron Horse)
Lou Gehrig was one of the most durable players in American professional baseball and one of its great hitters.
Babe Ruth was much more colorful and well-known for his baseball career; Lou was more quiet and gentle. The disease he was diagnosed in 1939 with amyotrophic lateral sclerosis, and the disease is well-known by his name. (1)
Emotional Impact
The heartbreaking feeling of losing hope when you feel your life slipping through your fingers like sand—this is the cold reality of a disease that steals your very identity. What’s worse, not everyone has access to treatment options. According to Wendy Smith, New Zealanders could not afford the medication in years past. (2)
MND Linked to Glutathione Deficiency
- Chronic decrease in glutathione (including the reduced form) can lead to overt motor neuron degeneration. (3,7)
- Oxidative stress causes mitochondria (power plants for your cells) to not work correctly in motor neurons, which can cause them to die. (5)
- Glutathione is the master antioxidant and is essential for mitochondria to function. (6)
Additionally, targeting the survivor gene (Nrf2) has been the subject of research in neurodegeneration. (8)
You need to consider additional ways to make your immune system work for you, not against you.
Opportunity
Treatment usually focuses on controlling symptoms and can include medications, physical therapy, and speech therapy.
But think—wouldn’t it be great if there were additional ways to bridge the gap? You don’t have to wonder— I can’t wait to see the look on your face when you find out what is possible!
Citations
- Britannica, T. Editors of Encyclopaedia (2022, November 15). Lou Gehrig. Encyclopedia Britannica. https://www.britannica.com/biography/Lou-Gehrig
- Smith, W. (2005, October). Rilutek should be subsidised. Kai Tiaki: Nursing New Zealand, 11 (9), 4 https://link.gale.com/apps/doc/A138396437/HWRC?u=spl_main&sid=bookmark-HWRC&xid=b09c6272
- Killoy, K. M., Harlan, B. A., Pehar, M., Helke, K. L., Johnson, J. A., & Vargas, M. R. (2018). Decreased glutathione levels cause overt motor neuron degeneration in hSOD1WT over-expressing mice. Experimental neurology, 302, 129–135. https://doi.org/10.1016/j.expneurol.2018.01.004
- Liu, J., & Wang, F. (2017). Role of Neuroinflammation in Amyotrophic Lateral Sclerosis: Cellular Mechanisms and Therapeutic Implications. Frontiers in Immunology, 8. https://doi.org/10.3389/fimmu.2017.01005
- Obrador E, Salvador-Palmer R, López-Blanch R, Jihad-Jebbar A, Vallés SL, Estrela JM. The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS. International Journal of Molecular Sciences. 2021; 22(12):6352. https://doi.org/10.3390/ijms22126352
- Marí, M., Morales, A., Colell, A., García-Ruiz, C., & Fernández-Checa, J. C. (2009). Mitochondrial glutathione, a key survival antioxidant. Antioxidants & redox signaling, 11 (11), 2685–2700. https://doi.org/10.1089/ARS.2009.2695
- Chi, L., Ke, Y., Luo, C., Gozal, D., & Liu, R. (2007). Depletion of reduced glutathione enhances motor neuron degeneration in vitro and in vivo. Neuroscience, 144 (3), 991–1003. https://doi.org/10.1016/j.neuroscience.2006.09.064
- Abdalkader, M., Lampinen, R., Kanninen, K. M., Malm, T. M., & Liddell, J. R. (2018). Targeting Nrf2 to Suppress Ferroptosis and Mitochondrial Dysfunction in Neurodegeneration. Frontiers in neuroscience, 12, 466. https://doi.org/10.3389/fnins.2018.00466
- Winter AN, Ross EK, Daliparthi V, et al. A Cystine-Rich Whey Supplement (Immunocal®) Provides Neuroprotection from Diverse Oxidative Stress-Inducing Agents In Vitro by Preserving Cellular Glutathione. Oxid Med Cell Longev. 2017;2017:3103272. doi:10.1155/2017/3103272