Lou

Lou Gehrig's disease (also known as Amyotrophic lateral sclerosis or ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. The disease is named after Lou Gehrig, a Hall of Fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s.

Symptoms

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 a cup of coffee or a book, or a third person’s problem 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. Symptoms can begin in the muscles that control speech and 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.

Causes

The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. An important step toward answering that question came in 1993 when scientists supported by the National Institute of Neurological Disorders and Stroke (NINDS) discovered that mutations in the gene that produces the SOD1 enzyme were associated with some cases of familial ALS. Although it is still not clear how mutations in the SOD1 gene lead to motor neuron degeneration, there is increasing evidence that the gene playing a role in producing mutant SOD1 protein can become toxic.

Treatment

There is no cure for ALS, but the drug riluzole has been approved for treatment and may slow progression of the disease. It is not a cure, however, and can prolong life by only a few months. Other treatments are directed toward relieving symptoms, such as spasticity, and can be handled by a multidisciplinary team of healthcare professionals.

See also

• Motor neuron disease
• Neurodegeneration
• Neurological disorder

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