Amyotrophic lateral sclerosis

Alternative Names

Lou Gehrig’s disease; ALS; Upper and lower motor neuron disease; Motor neuron disease

Definition of Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain and spinal cord that control voluntary muscle movement.

Causes, incidence, and risk factors

In about 10% of cases, ALS is caused by a genetic defect. In the remaining cases, the cause is unknown.

Symptoms

Symptoms usually do not develop until after age 50, but they can start in younger people. Persons with ALS have a loss of muscle strength and coordination that eventually gets worse and makes it impossible to do routine tasks such as going up steps, getting out of a chair, or swallowing.

Signs and tests

The health care provider will take a medical history, which includes strength and endurance.

Treatment

There is no known cure for ALS. The first drug treatment for the disease is a medicine called riluzole. Riluzole slows the disease progression and prolongs life.

Expectations (prognosis)

Over time, people with ALS progressively lose the ability to function and care for themselves. Death often occurs within 3 – 5 years of diagnosis. About 25% of patients survive for more than 5 years after diagnosis.

Review

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 8/27/2010

Central nervous system

ADAM Medical Encyclopedia

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