ALS / Amyotrophic lateral sclerosis / Lou Gehrig’s Disease

Overview

Amyotrophic lateral sclerosis (ALS) is a disease of the brain and nerves which send signals to the spinal cord, and nerves from the spinal cord to muscles throughout the body. It is also called Lou Gehrig’s disease, after the famous baseball player who was diagnosed with it in 1939. The nerve cells waste away and die. They cannot send signals to voluntary muscles (muscles over which a person has control). The unused muscles get weak and waste away. When the chest muscles stop working, it becomes impossible to breathe. The disease is fatal, usually 3-5 years after diagnosis. A few people will live more than 10 years. A very few people seem to experience remission or the progression stops; why is unknown. ALS does not affect the senses of sight, hearing, taste, smell and touch.

Symptoms and Diagnosis

At the onset of ALS, the symptoms may be so slight that they are often overlooked. It can start with weakness in the hands and arms, legs and feet, or the muscles of speech and swallowing. Tripping, dropping things, slurred, or “thick” speech, muscle cramps and twitching, or uncontrollable outbursts of laughing or crying. A person may notice difficulty with activities of daily living, such as dressing or washing. Some cases start with difficulty breathing, others with difficulty swallowing, choking, gagging or drooling. The head may drop as muscles weaken. The rate and sequence of progression are quite variable, but in all cases, muscle weakness progresses to paralysis. Ultimately, a ventilator must take over breathing for survival.

ALS is difficult to diagnose early because the symptoms are overlooked or are the same as several other diseases of the nervous system. The average age at diagnosis is 55. Blood tests may be done to rule out other diseases. Special tests are done with electrodes measuring muscle response and how fast nerves can send signals to the muscle. Breathing and swallowing tests and an MRI of the brain are performed. Sometimes a muscle biopsy is required. It is not clear whether or not ALs affects thinking, memory or judgment.

Causes

Worldwide, up to 5 people per 100,000 are affected. In up to 95 percent of cases the cause is unknown; about 10 percent are inherited. Veterans, especially those deployed to the Gulf War, have twice the incidence of the disease in the general population. There are no other proven risk factors. The disease affects all races, ethnicities, and socioeconomic groups. Environmental factors that may affect an individual’s risk for developing ALS are under investigation. Smoking, exposure to lead in the workplace, and military service are some of the environmental factors of interest. Continue reading for Prevention and Treatment Information . . .

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