What is ALS?

Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neurodegenerative disease that affects the nerve cells in the brain and spinal cord and the motor neurons, responsible for controlling breathing and voluntary muscle movement, such as speaking, walking and swallowing.

Etiology and Prevalence

The exact cause of ALS remains undefined, however, several different theories on the etiology of ALS have been proposed, including genetic, environmental and molecular causes of the disease. 

The worldwide prevalence of ALS is estimated at about 4.42 per 100,000 individuals. This disease affects men slightly more often than women, and symptoms usually begin between the ages of 40 and 70.

Symptoms and Progression

The symptoms of ALS and progression can vary significantly among individuals. There are some common symptoms signifying possible ALS:

• Muscle twitching or spasming, often presented in the upper limbs
• Progressive muscle weakness.
• Difficulty swallowing or speaking.
• Limited use of the arms and legs.

Since ALS is a progressive disorder, early-developed symptoms tend to worsen, while new symptoms develop. The progression of physiological symptoms can slowly lead to:

• Paralysis
• Respiratory failure (most common cause of death in ALS patients)

While ALS does not typically affect cognitive functions, such as memory, and rational thinking, some people may develop a form of dementia known as frontotemporal dementia.

Diagnosis

There is no singular specific test just for ALS, therefore the diagnosis includes a series of different tests and examinations. A diagnosis is typically based on clinical observations, detailed patient history and diagnostic tests such as electromyography (EMG) and nerve conduction studies. You can read more about the als diagnostic process here.

Treatment and Management

While there's currently no cure for ALS, there are treatments available to decrease the effects of symptoms and improve quality of life. Riluzole and edaravone are FDA-approved drugs known to slow disease progression. Involving multidisciplinary care in your caregiving is also greatly beneficial. It is recommended to hire a team of physical therapists, occupational therapists, respiratory therapists, and others, to ensure higher well-being and symptom management.

ALS is one of the most researched conditions. Scientists are trying to understand the genetic, environmental, and molecular mechanisms powering the disease. Medical discoveries are powering novel therapeutic approaches, including gene therapy and stem cell treatments in clinical trials, offering hope for future breakthroughs and hopefully a cure. 

Credits:
What is FTD and how is it connected to ALS?
Johns Hopkins: ALS
ALS Life Expectancy: Understanding the Prognosis
What causes ALS?
How Is ALS Diagnosed?