Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis
Amyotrophic Lateral Sclerosis

A number of progressive neurological disorders can affect individuals. Two prominent examples are Alzheimer’s disease and Huntington’s disease. Both are markedly different from one another, but they both affect the function of the brain. Another disease that does this is amyotrophic lateral sclerosis, or ALS. You might be more familiar with it as Lou Gehrig disease. Discovered in 1969, ALS becomes worse over time, eventually robbing patients of their ability to use their muscles and move the body.

What Is Amyotrophic Lateral Sclerosis?

According to the National Institute of Neurological Disorders and Stroke, ALS is not one disease, but many. The NINDS states,

"ALS is a group of rare neurological diseases that mainly involve the nerve cells (neurons) responsible for controlling voluntary muscle movements. Voluntary muscles produce movements like chewing, walking, and talking. The disease is progressive, meaning the symptoms get worse over time."

Another definition can be found from the ALS Association, which states that,

"ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord."

The name originates from the Greek meaning literally "no muscle nourishment."

ALS affects the motor nerves necessary for most of the voluntary actions we make every day. Raising your arm requires these nerves. Opening your mouth does as well. So does uttering speech or eating food.

There are two types of ALS. The most common is called sporadic ALS. Up to 95% of all cases fall under this heading. What “sporadic” means here is that there is no discernable cause for the condition. It can affect anyone at all. The second type, and the rarer of the two, is called familial ALS, and is inherited. It accounts for between 5 and 10% of all cases. For a parent with familial ALS, there is a 50% chance of passing the condition to a child.

According to the ALS Association, there are currently 20,000 Americans suffering with ALS, with an unknown percentage at risk for inheriting the genetic mutation responsible. Most patients are between the ages of 40 and 70. However, women are more likely to develop ALS at a younger age than men, and risk does increase with age.

What Are the Symptoms of ALS?

Ultimately, ALS leads to a total loss of voluntary muscle movement and control, including the ability to breathe. It is invariably fatal. However, catching early signs and symptoms can help to increase a patient’s quality of life. Some of the most common early symptoms include:

  • Twitching in the tongue
  • Twitching in the shoulders or arms
  • Difficulty walking
  • Leg weakness
  • Weakness in the hands
  • Loss of coordination in the fingers
  • Slurred speech
  • Difficulty swallowing
  • Difficulty holding up your head
  • Tripping

ALS often begins innocuously – a minor hand tremor, for instance. It then progresses over time, spreading to other body parts. Note that ALS does not affect an individual’s cognitive abilities – only muscle control and movement.

What Is the Cause of ALS?

There is no single cause that can be attributed to all cases of ALS. In some cases, a genetic mutation is responsible (familial ALS). However, in sporadic cases, there is no clear reason for the condition. With that being said, researchers strongly suspect several potential causes.

One of those is a chemical imbalance in the brain. ALS sufferers often have higher than usual levels of glutamate, which can be toxic to specific types of nerve cells. Another potential cause is mishandling of proteins within nerve cells, leading to an accumulation of abnormal protein forms, thereby destroying the nerve cells. In some instances, ALS may actually be a form of autoimmune disease, with the body attacking normal, healthy nerve cells.

When it comes to increased risk factors, those who have a family history of ALS are definitely at an increased risk of developing the disease. Other risk factors include age and exposure to toxins within your environment. Interestingly, military service also makes an individual more likely to develop ALS, although there is no clear reason for this.

What Treatments Are Available for ALS Sufferers?

There is no cure for ALS. The disease has bad prognosis, even using what treatment methods are available. It is also currently impossible to reverse the damage done by ALS, or even to permanently halt the damage to nerves. However, it is possible to slow the damage down using medications and a number of different therapy types. Riluzole and edaravone are two medications used to treat ALS. These can be used in conjunction with physical therapy, occupational therapy and breathing care. Speech therapy, nutritional support, and social support also play important roles here.

What Role Can Stem Cell Therapy Play?

Stem cells are being researched for their potential use in treating an incredibly wide range of conditions and diseases, including ALS. This is understandable given their role as the progenitors of all cell types, and their ability to transform themselves into any other cell or tissue type for healing.

There is a significant amount of ongoing research surrounding stem cell therapy for ALS patients, including laboratory work, animal testing, and even limited human testing. For instance, a trial with 18 patients began in 2017, as reported by the CRIM. Another study, this one published in the journal Stem Cells, noted the types and effectiveness of stem cells used to treat ALS. The ALS Association itself details many efforts to treat the disease using stem cells.

However, most of the clinical trials and studies use autologous stem cells – cells harvested from the patient’s own body, or adult stem cells. These cells are inferior to other options in many ways, including their limited ability to heal damage. They may also be attacked by the body’s immune system.

Allogeneic stem cells harvested from umbilical cord blood and tissue provide the greatest healing factor and the longest lifespan. They are also immune system naïve, and replicate the benefits of using embryonic stem cells without any ethical issues.

Moving Forward

Currently, there are no FDA-approved stem cell treatments for humans, and any such treatments are experimental in nature. However, with the ongoing research, it is only a matter of time before a way to halt, reverse, and even prevent ALS is found.

Source:

https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/diagnosis-treatment/drc-20354027
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet
http://www.alsa.org/about-als/what-is-als.html
https://hsci.harvard.edu/als-0
http://www.alsa.org/research/focus-areas/stem-cells/
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4225704/
https://blog.cirm.ca.gov/2017/10/09/can-stem-cell-therapies-help-als-patients/

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