amyotrophic lateral sclerosis (ALS) (ā'mīətrōf`ik, sklĭrō`sĭs) or motor neuron disease, sometimes called Lou Gehrig's disease, degenerative disease that affects motor neurons in the brain brain, the supervisory center of the nervous system in all vertebrates. It also serves as the site of emotions, memory, self-awareness, and thought.

Anatomy and Function

..... Click the link for more information. and spinal cord spinal cord, the part of the nervous system occupying the hollow interior (vertebral canal) of the series of vertebrae that form the spinal column , technically known as the vertebral column.
..... Click the link for more information. , preventing them from sending impulses to the muscles. The muscles atrophy quickly, causing weakness, paralysis, and eventual death, usually when the muscles that control respiration fail. The intellect, eye motion, and bladder control are not affected. ALS sometimes originates in the brain, causing initial symptoms such as difficulty in swallowing or talking; in other cases it originates in the spinal cord, causing initial symptoms such as weakness in the extremities. About 10% of ALS cases are hereditary. ALS usually develops after age 40; more men are affected than women.

There appear to be several causes of ALS. In 1991 a research team led by Teepu Siddique and Robert H. Brown, Jr. located the gene for familial ALS on chromosome 21. A later discovery pinpointed a mutation in the gene that codes for an enzyme, superoxide dismutase (SOD), as responsible for a percentage of familial cases. These defects do not appear to be present in the more common nonfamilial, or "sporadic," form of the disease. In addition to genetic factors, scientists have studied the buildup of the chemical glutamate that occurs in ALS patients. Glutamate normally acts as a neurotransmitter in the brain, with excess amounts being absorbed by the cells. In ALS patients the reabsorption process fails, and the buildup of glutamate selectively destroys motor neurons. Other possible causes of ALS include defects in the gene that makes the neurofilament proteins that support nerve cell axons, and antibodies that interfere with calcium channels in the cells and cause a toxic buildup of calcium in the neurons.

There is no cure for ALS. Devices such as wheelchairs and speech synthesizers can help patients maintain independence. Research into treatment has concentrated on neurotrophic factors (proteins that assist nerve growth and health) and glutamate blockers. Rilutek (formerly Riluzole), the first drug approved by the Food and Drug Administration for treatment of ALS (1995), adds a few months to the life expectancy of most patients but does not relieve symptoms. Another drug, myotrophin, seemed somewhat promising in early studies (1996), but its effectiveness was not confirmed and it has not been approved. Baseball star Lou Gehrig Gehrig, Lou (Louis Gehrig) (gâr`ĭg), 1903–41, American baseball player, b. New York City.
..... Click the link for more information. died of ALS in 1941, bringing it national attention.

amyotrophic lateral sclerosis (ALS)

or Lou Gehrig disease

Degenerative nervous-system disorder causing muscle wasting and paralysis. The disease usually occurs after age 40, more often in men. Most victims die within two to five years from respiratory muscle atrophy. ALS affects motor neurons; the muscles they control become weak and atrophied, with debility usually beginning in the hands and creeping slowly up to the shoulders. The lower limbs become weak and spastic. Variants include progressive muscular atrophy and progressive bulbar palsy. In 1993 the defective gene that accounts for 5–10% of cases was discovered; it produces an ineffective version of an enzyme that neutralizes free radicals, which destroy motor neurons.