Lou Gehrig Disease – A Century of Research and Discoveries

ALS also known as amyotrophic lateral sclerosis, it is a neurodegenerative disease that affects the nerve cells in the brain and the spine.

The disease was discovered in 1869 by a French neurologist Jean-Martin Charcot as one of the many conditions affecting the lateral horn of the spinal cord. Further research isolated a disease that caused the death of motor neurons-ALS.

In England, it was known as the motor neuron disease (MND) while in the US it was referred to as amyotrophic lateral sclerosis. The disorder was later called the Lou Gehrig disease after it caused the death of the legendary Baseball player Lou Gehrig in 1941.

 

Meaning of ALS

 A-myo-trophic is derived from the Greek language where “A” denotes no, “Myo” represents the muscle, while trophic refers to nourishment. So, the entire phrase means no muscle nourishment. When a muscle does not get nourishment, it wastes away.

Lateral refers to the areas in an individual’s spine where the portion of the nerve cells that control the muscles is located. As the area degenerates, it leads to hardening (sclerosis) of the region.

 

ALS and Motor Neurons

Motor neurons are neurotransmitters that send signals from the brain to the spinal cord and then to the muscles. There are two types of motor neurons:

  • Upper motor neurons (nerve cells located in the brain)
  • Lower motor neurons (nerve cells found in the spinal cord).

The motor neurons control the muscles that cause voluntary movements in the arms, legs, and face. As such, they signal your muscles to contract when you want to grab a chair, walk, run, or chew.

Lou Gehrig is a rare disease that affects the motor neurons. Other diseases that affect motor neurons include:

  • The primary lateral sclerosis that affects the central motor neurons and is characterized by painless and progressive weakness of the leg muscles
  • Progressive Bulbar Palsy that causes difficulty in talking swallowing and chewing. Some patients also experience weak palatal movements, gag reflexes, and reduced movement of the facial and tongue muscles
  • Pseudobulbar palsy, which is also characterized by one’s inability to control facial movements. The patient may also manifest uncontrolled emotional outbursts

 

How ALS Affects the Body

When the motor neurons die, the brain lacks control of the muscle, leading to paralysis. As such, muscles that are under the voluntary control of nerve cells become affected and patients gradually lose their ability to talk, swallow walk, and breathe.

During this process, the mind remains alert, observing the loss of every function. The diaphragm and the chest muscles eventually fail, and the patient stops breathing, causing death. Most patients diagnosed with ALS die of respiratory failure within 3-5 years.

 

Types of ALS

Medical research indicates that there are two forms of amyotrophic lateral sclerosis.

  • Sporadic ALS

It is the most common form of the Lou Gehrig Disease and affects 90-95% of the people diagnosed with the disease. This type happens without an apparent cause.

  • Familial ALS

This kind affects 5-10% of the people who have ALS. It is hereditary, hence parents tend to pass a faulty gene to their kids. If one of the parents carries an ALS gene, his children have a 50% chance of contracting the disease.

 

Causes of ALS

Apart from carrying a hereditary gene, researchers have still not established the primary cause of the disease. Speculations as to the causes of ALS include:

  • Glutamate Imbalance– the chemical acts as a neurotransmitter in the nerves and the brain. People who have ALS have higher levels of glutamate in the nerve cells in the spinal fluid. High levels of glutamate are toxic to the nerve cells. In fact, FDA-approved medications to treat ALS work by reducing glutamate levels.
  • Immune system problems– the immune system protects the body from foreign substances like viruses and bacteria. The brain has microglial immune cells, which sometimes destroys healthy motor neurons, causing ALS.
  • Oxidative Stress– cells use oxygen to produce energy. Some of this energy may form harmful substances known as free radicals that damage cells

 

Symptoms of ALS

  • Weakness of the hands, legs, and feet.
  • Muscle cramps and twitching of the shoulders, arms, and tongue.
  • Tripping and falling.
  • Slow speech.
  • Difficulty walking or holding the head up.

 

An Example in Real Life

Apart from the legendary Luo Gehrig, other notable individuals who have been diagnosed with ALS include Stephen Hawking an author, cosmologist, and theoretical physicist, NBA Hall of Fame basketball player George Yardley, US Army General Maxwell Taylor, and jazz musician Charles Mingus, among others also suffer from the ailment.

 

Treating ALS

Recent years have shown successive research as to the physiology of ALS disease. Currently, the only FDA-approved drug that is believed to reduce the progression of ALS is known as Riluzole. Other viable treatments of ALS include:

Ø  Patient Education

It is enhanced by referring patients to multi-disciplinary clinics that have a staff specialized in ALS. It also involves providing educational materials for families and patients from national organizations that deal with ALS. A doctor may also recommend outpatient care with the guidance of a neurologist or other care giver with a particular interest in the disease.

Ø  Edaravone

The pyrazolone free radical drug was approved in May 2017 to treat ALS by reducing oxidative stress, which causes the disease.

Ø  Botulinum Toxin

The treatment is ideal for patients who for ALS patients with sialorrhea (excessive production of saliva). However, the use of this treatment should be controlled due to its adverse effects (exacerbation of weakness of the bulbar muscles).

Conclusion

Despite the advances in treatments and etiology, Lou Gehrig’s disease still remains somewhat of an enigma for the medical world. However, awareness campaigns, focused on educating patients and physicians alike, on the diverse aspects of this condition has sparked a new hope that there may be a way to reverse and perhaps cure this condition.

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