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

Progression of ALS

The early symptoms of ALS include muscle weakness or stiffness in the hands, feet, legs, and ankles; difficulty walking, swallowing, or holding the head up; muscle cramps; twitching in the arms, shoulders, and tongue; and slurred speech. This is generally followed by progressive weakness, wasting, and paralysis of the muscles of the limbs and trunk, as well as loss of control of vital functions such as speech, swallowing, and eventually breathing.

The speed of ALS progression varies between patients, as does the order in which different abilities are lost. Even in the same individual, progression of the disease may not be linear. It is common to have periods where there is little or no loss of function lasting for weeks or months. In rare cases, patients may even experience an improvement in symptoms and some recovery of lost function. But these periods of ALS “arrest” and “reversal” are often transient.

Forms of ALS

The most common form of ALS is called sporadic ALS because it occurs spontaneously without any known family history or associated genetic or environmental risk factors. In the U.S., sporadic ALS represents 90 percent or more of all ALS cases.

Another form of ALS is called familial ALS (FALS), which is inherited from the parents and comprises 5–10 percent of ALS cases. FALS is inherited in an autosomal dominant manner, meaning that inheritance of a single copy of the faulty gene is sufficient to cause ALS in a person.

Incidence of ALS

ALS can affect anyone worldwide regardless of racial, ethnic, or socioeconomic status. In general, symptoms commonly develop between the ages of 40 and 70, with the average being 55 years at diagnosis. The disease is most common among people 60 years or older, but nearly 10 percent of ALS patients are 45 or younger.

Although ALS is 20 percent more likely to develop in men than in women, with advancing age, the gender difference disappears.

Moreover, Caucasians and non-Hispanics are more likely to develop ALS compared with other ethnic groups. According to the ALS Patient Care Database, 93 percent of ALS patients were Caucasian, but this may be due to more cases being recorded for Caucasians than other ethnic groups.

Studies suggest that military veterans are about 1.5–2 times more likely to develop ALS. This could be because of their exposure to lead, pesticides, and other environmental toxins. Military veterans deployed during the Gulf War were twice as likely to develop ALS compared with non-veterans.

According to the ALS Association, approximately 5,000 people in the U.S. are diagnosed with ALS each year, which averages to about 15 new cases each day. It is estimated that up to 20,000 Americans have the disease at any given time.

Mortality and life expectancy

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

Although the average life expectancy of a person with ALS is about two to five years from the time of diagnosis, some people can live with the disease for more than five years. More than half of all ALS patients 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 five percent live 20 years or more.

Cost of living for patients

ALS patients incur substantial costs for medical care, equipment, and home healthcare. The medical costs are substantial and increase rapidly and substantially as the disease progresses, so patients should learn about their health plan coverage, and other programs for which they may be eligible, including Social Security Disability, Medicare, Medicaid, and Veterans Affairs.

Treatment for ALS

Currently, there is no cure for ALS but there are some treatments available.

For example, Rilutek (riluzole) is an anti-glutamate oral treatment approved by the U.S. Food and Drug Administration (FDA) in 1995 for the treatment of ALS. Analysis of results from four clinical trials demonstrated a nine percent increase in the probability of survival by one year and increased median survival from 11.8 to 14.8 months in patients treated with Rilutek. The treatment has also been shown to slow the progression of ALS, allowing the patient more time to be in a higher functioning state with greater control of muscle movement.

Radicava (edaravone) is another treatment in the form of infusion therapy approved by the FDA in 2017 to treat ALS. It protects nerve cells and slows down disease progression by removing free radicals. The treatment has been shown to slow the decline in physical functioning in some ALS patients.

The future of ALS

Many ALS patients are living longer and more productive lives thanks to advances in research that increased understanding of the causes of ALS. Patient survival has also increased with improvements in medical management, including support for nutrition and breathing. Further research is needed to determine the possible risk factors that contribute to ALS and to develop new and improved treatments that can improve survival and the quality of life for ALS patients.

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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.