Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease that results in the death of nerve cells in the brain and spinal cord called motor neurons, which control voluntary muscles.

Progression of the disease

The early symptoms of amyotrophic lateral sclerosis (ALS) often involve muscle weakness or stiffness. This is generally followed by progressive weakness, wasting, and paralysis of the muscles of the limbs and trunk as well as those that control such vital functions as speech, swallowing, and, eventually, breathing.

ALS progression strips a person of the ability to walk, dress, write, speak, swallow, and — in advanced stages — to breathe.

The speed and order in which different abilities are lost vary from person to person. Even in the same individual, the disease does not always progress in a straight line, and it is common to have periods of little or no loss of function that last for weeks or months. In very rare cases, there may even be an improvement and some recovery of lost function. But these periods of ALS “arrest” and “reversal” are often transient.

Types of ALS

The most common form of ALS is called sporadic ALS, because it occurs spontaneously without a known cause. In the United States, sporadic ALS represents up to 90 to 95 percent of all disease cases.

Another form of ALS is called familial ALS (FALS), which is inherited and comprises about 10 percent of all ALS cases. FALS is inherited in an “autosomal dominant” manner, meaning that only one faulty gene responsible for the disease can cause ALS in a person, and this person has a 50% chance of his or her children also inheriting it.

Incidence

ALS can affect anyone; worldwide, it occurs in people regardless of their racial, ethnic, or socioeconomic differences. However, military veterans, particularly those deployed during the Gulf War, are about twice as likely to develop ALS compared to non-veterans. According to the ALS CARE database, about 93 percent of patients are Caucasian, but this could be because cases among whites are recorded more often than among minority groups.

According to the ALS Association, approximately 6,000 people in the U.S. are diagnosed with ALS each year, which averages to about 15 new cases each day. It is estimated that as many as 20,000 Americans have the disease at any given time.

Mortality

Estimates suggest that ALS is responsible for as many as five in every 100,000 deaths in people ages 20 or older.

Age 

Most people develop ALS between the ages of 40 and 70, and the average age at diagnosis is 55. The disease is most common among people age 60 or older, but ALS is known to also strike people in their 20s and 30s.

Gender

ALS is 20 percent more common in men than in women. However, with increasing age, this gender difference in ALS cases narrows. Generally, ALS occurs in greater percentages as both men and women grow older.

Life expectancy

Although the average life expectancy of a person with ALS is about two-to-five years from the time of diagnosis, people can live with the disease for more than five years. More than half of all people with ALS live more than three years after diagnosis; 20 percent live five years or more; up to 10 percent live more than 10 years, and about 5 percent live 20 years or more.

Costs 

There are significant costs for medical care, equipment, and home healthcare during later stages of the disease. It is important to learn about health plan coverage and other programs for which you may be eligible, including Social Security Disability, Medicare, Medicaid, and Veteran Affairs benefits.

Treatment

Currently, there is no cure for ALS. Rilutek (riluzole) is an anti-glutamate oral drug approved by the U.S. Food and Drug Administration (FDA) for the treatment of ALS in 1995. The drug was shown to prolong patients’ life expectancy by at least a few months. More recent studies suggest that Rilutek slows the progression of ALS, allowing the patient more time in the higher functioning states with greater control of muscle movement.

Radicava (edaravone) is an infusion therapy approved by the FDA in 2017 to treat people with ALS. The drug has been shown to slow the decline in physical functioning in some ALS patients.

The future

Many ALS patients are living longer and more productive lives as research  advances into the causes, ways of preventing, and of treating the disease. Patient survival has also increased with improvements in medical management, including support with nutrition and breathing. But more research is needed to better understand the disease and determine possible risk factors that may contribute to its development. Such understanding is crucial in the development of new and improved treatments.

 

ALS News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.