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Amyotrophic lateral sclerosis (ALS) is a serious neurological disease that causes muscle weakness, disability and eventually death. ALS is often called Lou Gehrig's disease, after the famous baseball player who died of it in 1941.
Worldwide, ALS occurs in one to three people per 100,000. An inherited form of the disease occurs in 5 to 10 percent of the cases. But in the vast majority of cases, doctors don't yet know why ALS occurs in some people and not in others.
ALS often begins with muscle twitching and weakness in an arm or leg, or with slurring of speech. Eventually, ALS affects your ability to control the muscles needed to move, speak, eat and breathe.
Symptoms
At the onset of ALS the symptoms may be so slight that they are frequently overlooked. With regard to the appearance of symptoms and the progression of the illness, the course of the disease may include the following:
- muscle weakness in one or more of the following: hands, arms, legs or the muscles of speech, swallowing or breathing
- twitching (fasciculation) and cramping of muscles, especially those in the hands and feet
- impairment of the use of the arms and legs
- "thick speech" and difficulty in projecting the voice in more advanced stages, shortness of breath, difficulty in breathing and swallowing
The initial symptoms of ALS can be quite varied in different people. One person may experience tripping over carpet edges, another person may have trouble lifting and a third person's early symptom may be slurred speech. The rate at which ALS progresses can be quite variable from one person to another. Although the mean survival time with ALS is three to five years, many people live five, ten or more years. In a small number of people, ALS is known to remit or halt its progression, though there is no scientific understanding as to how and why this happens. Symptoms can begin in the muscles of speech, swallowing or in the hands, arms, legs or feet. Not all people with ALS experience the same symptoms or the same sequences or patterns of progression. But, progressive muscle weakness and paralysis are universally experienced.
Muscle weakness is a hallmark initial sign in ALS, occurring in approximately 60% of patients. Early symptoms vary with each individual, but usually include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and/or uncontrollable periods of laughing or crying.
The hands and feet may be affected first, causing difficulty in lifting, walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes.
As the weakening and paralysis continue to spread to the muscles of the trunk of the body the disease, eventually affects speech, swallowing, chewing and breathing. When the breathing muscles become affected, ultimately, the patient will need permanent ventilatory support in order to survive.
Since ALS attacks only motor neurons, the sense of sight, touch, hearing, taste and smell are not affected. For many people, muscles of the eyes and bladder are generally not affected.