ALS

Amyotrophic lateral sclerosis (ALS) is a neurological disease that causes a progressive degeneration of nerve cells in the brain and spinal cord that are essential for initiating muscle movement. Although ALS can affect anyone, the disease is not contagious.

ALS is more commonly known as Lou Gehrig’s disease, named for the beloved baseball legend who died from ALS in 1941 — less than two years after he revealed his condition to the world. The ALS Association estimates that approximately 30,000 people in the U.S. are living with ALS at any given time — and there is currently no cure. 

As ALS progresses, the affected motor neurons increasingly deteriorate and die, resulting in a permanent loss of voluntary muscle control and function. Over time, ALS triggers muscular atrophy and total paralysis, which ultimately inhibits the ability to breathe. Since ALS targets motor neurons only, the disease does not usually impact cognitive function, nor does it affect the senses of touch, taste, smell, sight and hearing. 

The progression rate of ALS varies from person to person, but the average life expectancy from the time of diagnosis is two to five years. According to statistics from The ALS Association, half of all people diagnosed with ALS live three years or longer, while approximately 20 percent live five years or longer and 10 percent are expected to survive past 10 years. 

In some instances, ALS advances at an abnormally slow rate and even stops progressing entirely; however, how and why this anomaly occurs remains a medical mystery — as does the disease itself. 

A small percentage of ALS diagnoses have been linked to an inherited genetic mutation, but what causes the vast majority of cases is not known. ALS is difficult to diagnose since there is no single definitive test to confirm the suspicion. Before an official conclusion is made, a series of diagnostic tests and procedures are typically needed so doctors can rule out other neurological disorders that mimic ALS.

Risk Factors
ALS most commonly develops in people between the ages of 40 and 70. The disease sometimes will strike people in their 20s and 30s, but symptoms usually present as men and women approach their senior years. More men are affected by ALS earlier in life than women; however, according to the Mayo Clinic, this gender disparity ceases to exist after age 70.
 

People diagnosed with a familial form of ALS, which accounts for five to 10 percent of documented cases, have a 50/50 chance of passing the gene on to their children. 

Other suspected risk factors under investigation include cigarette smoking and exposure to certain environmental toxins, such as lead and pesticides. Additionally, for reasons unknown, recent studies show that people who served in the military are nearly twice as likely to develop ALS. 

Early Signs and Symptoms
The onset of ALS symptoms can be subtle and often overlooked. While not every person with ALS develops the same symptoms in the same order, the two main symptoms experienced by all people with ALS are muscle weakness and subsequent paralysis.
 

The disease tends to affect motor control in the hands, feet and limbs before spreading to other muscle groups in the body. Initial signs and symptoms of ALS include:
• Muscle weakness and a diminished ability to move the arms and/or legs.
• Spastic twitching and cramping of muscles.
• Frequent tripping and/or complications with walking and coordination.
• Trouble gripping or lifting objects.
• Slurred speech and difficulty with vocal projection.
• Problems maintaining proper posture and/or uncontrollable drooping of the head.
• Strained swallowing and labored breathing.

As ALS migrates to the muscles in the neck and torso, the abilities to speak, chew, swallow and breathe become impaired and eventually eliminated. Feeding tubes and ventilators are often required for patients with advanced-stage ALS to prolong survival.

Treatment Options
Although no cure for ALS exists yet, the rate at which the disease spreads throughout the body can be delayed to a degree. The FDA-approved drug Riluzole has been shown to help slow the progression of ALS and increase the lifespan of some people by a few months. Physical therapy is also used to help maintain muscle strength and functionality for as long as possible.

Other various therapies and devices for managing ALS focus on improving comfortability, self-sufficiency and overall quality of life. Research on ALS and clinical trials for new drugs and therapies continue to be conducted in hopes of finding a cure.

   

Related:
The ALS Ice Bucket Challenge
Living with ALS


 
Sources: The ALS Association, Mayo Clinic

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