In the US, Global Motor Neuron Diseases Awareness Day is celebrated every year on June 21st to create awareness about MND (Motor Neuron Diseases) and ALS (Amyotrophic lateral sclerosis). 

What is a motor neuron and what are motor neuron diseases? 

  • Motor neurons are the nerve cells that control your movements, speaking, swallowing, and breathing by sending signals from the brain to the muscles with the help of the spinal cord. They are of two types: upper motor neurons and lower motor neurons. 
  • Upper motor neurons send messages to your spinal cord from your brain. 
  • Your spinal cord contains lower motor neurons. They carry messages from your brain to your muscles. Their nerve fibers are the longest in your body (from the base of the spinal cord to all the way to the tips of the toes).  
  • Motor Neuron Disease (MND) is a disease that affects the connection of motor nerves from the brain to the spinal cord so they start losing their functionality over time. The electrical signals are affected by this disease and muscles start losing their functions. It can happen to anyone at any age.  

Types  

There are several types of disease which include: 

  1. Amyotrophic Lateral Sclerosis (ALS): This is the most common type of motor neuron disease. It affects both upper (from your brain) and lower (from your spinal cord) motor neurons. It makes you lose control over the muscles of the arms, legs, mouth, and respiratory system. It is not hereditary, however, in the US, 5 to 10% of cases are found in families. On average, once the symptoms are discovered, people only have a life expectancy of 3 to 5 years; but with proper supportive care, this might reach up to 10 years.  
  2. Primary Lateral Sclerosis: This type of disease is like ALS, but it only affects the upper motor neurons. It progresses more slowly than ALS. It causes stiffness and weakness in arms and legs that leads to poor coordination, slowed walking pace, or balance problems. It usually occurs in people between 40 to 60 years of age. In exceedingly rare cases, it is also found in children. It affects the quality of life, but it isn’t fatal.   
  3. Progressive Bulbar Palsy (PBP): This disease is a form of ALS and people with BPB might develop ALS as time goes on. It damages the motor neurons in the brain stem (the base of the brain). This affects your eating, chewing, swallowing, and speaking abilities. You might also experience slurring of your speech and have difficulty chewing and swallowing if you have PBP. It also makes controlling emotions difficult. You may unintentionally laugh or cry.  
  4. Progressive muscular atrophy: This type of MND is very rare. It affects the lower motor neurons of the spinal cord causing slow and progressive muscle wasting and leading to weakness or cramps in the arms, legs, and mouth muscles. This disease can later turn into ALS.  
  5. Spinal Muscular Atrophy (SMA): SMA affects your lower motor neurons. This is a type of hereditary MND and mostly develops in children. There are 3 types of SMA caused by the genetic change in the SMA1 gene. This gene makes protein and protects the motor neurons and without it, they die. This causes weakness in the arms, legs, and trunk.  
    • Type 1 (Werdnig-Hoffmann disease)– It begins around the age of six months. The child is unable to sit or hold its head up. They have low muscle tone, poor reflexes, and difficulty swallowing and breathing. 
    • Type 2– It usually begins between the ages of 6 and 12 months. Children with this form can sit but cannot stand or walk on their own. They may also have difficulty breathing. 
    • Type 3 (Kugelberg-Welander disease)– It begins between the ages of 2 and 17. It affects a child’s ability to walk, run, stand up, and climb stairs. This type of child may also have a curved spine and shortened muscles or tendons around their joints. 
    • Type 4– It usually begins after the age of 30. This type of person may experience muscle weakness, shaking, twitching, or breathing difficulties. It primarily affects the arms and legs.
       

Causes of MND 

Although, what exactly causes MND is still unknown. Experts say that 10% are hereditary and 90% develop ‘randomly’ or ‘sporadically’. According to the National Institute of Neurological Diseases and Stroke- genetics, toxic chemicals, and viral, and environmental factors might play a role in the development of MND.  

Symptoms 

The early symptoms start showing in your arms, legs, mouth, and respiratory system which include: 

  • Weakening grip, hard to hold and to pick up things 
  • Fatigue 
  • Muscle pains, cramps, and twitches 
  • Slurred speech 
  • Weakness in arms and legs 
  • Clumsiness and stumbling while walking 
  • Difficulty in swallowing 
  • Trouble in breathing or shortness of breath 
  • Unsuitable emotional responses, such as laughing or crying 
  • Weight loss (as muscles lose their mass) 

As the condition gets worse, symptoms start to get severe: 

  • muscle shrinkage 
  • Difficulty in moving 
  • joint pain 
  • drooling, due to problems with swallowing 
  • uncontrollable yawning, which can lead to jaw pain 
  • changes in personality and emotional responses 
  • difficulty breathing 

Eventually, when this disease is in advanced stages, a person starts to depend on others for eating, moving, breathing, or a combination of these. This disease can be life-threatening because breathing problems can lead to asphyxiation and death.  

Risk factors 

  • This disease can develop in anyone, at any age.  
  • It is more commonly found in males than females.  
  • The inherited form is usually present at birth. Most of the time, it appears after the 50s.  
  • Different types have different risk factors SMA is hereditary, but it’s not true with other types of MND. In the US, 10% of ALS cases are hereditary.  
  • According to the National Institute of Neurological Disease and Stroke observation, Veterans have a two times higher chance of developing ALS. This indicates that exposure to certain toxins increases the risk of ALS. 

Treatment 

There is no cure for MND, you can slow the progression of the disease with medication and some physical therapy. Supporting devices also help in the advanced stage. The treatment depends on certain factors like MND type, symptoms, personal needs, and availability and affordability of drugs and medicine. Medication helps in slowing down progress and helps to avoid pain. Botox injections help relieve symptoms of muscle stiffness and cramps. Physical therapy also helps the crucial functions like breathing, eating, moving, and communicating. 

MND is rare but once you have it there is no cure. You can take antioxidants or vitamin E to help prevent MND to some extent.  

Want any help and need to know more about neurological diseases? At Doral Health and Wellness, we have doctors that can help you manage your condition. For more information, you can visit us at 1797 Pitkin Avenue, Brooklyn, New York, 11212, or call us at 1-347-384-5690. You can also visit our website at https://doralhw.org or contact us at info@mainwebsite.dialysisny.com if you have any queries.