Aging Begins at 30

Lou Gehrig's Disease

Not only Lou Gehrig had this disease but also Vice President Henry Wallace, Dimitri Shostakovich and David Niven. Its name is amyotrophic (wasting of nerve cells controlling muscles) lateral (at the side of the spinal cord ) sclerosis (scarring) and is due to death of nerve cells that supply muscles usually for the limbs but sometimes in the mouth and throat. Its a progressive fatal nerve disease of unknown cause usually lasting less than 3 years. Twenty percent live over 5 years.

An African American patient of mine, aged 66, first noted small local spontaneous muscle contractions easily visible through the skin of his forearm, a sign that the muscle was dying because its nerve supply had withered. Another man in his 50's (it's more common in men than women) had tiny tremors of his tongue. He was one of the 10% that have the disease run in their family. Often its weakness of one hand that is noted, then the other is affected. Later the toe catches on carpets. All four limbs are soon affected but there is no loss of sensation, mental ability, or bladder or bowel control. Weakness, incoordination, cramps and spasm develop with difficulty in using the hands for skilled movements, also with walking, speaking and swallowing.

The final stages are often distressing for the patient and the family. With awareness and intellect unaffected, the victim is unable to speak, to swallow, or move purposefully. Then the patient has trouble breathing. Realistic choices have to be made about the use of "heroics" in an emergency and whether a respirator should be used.

Needles placed in the muscles (EMG) show decreased and abnormal contractions. For diagnosis, this should be found to be abnormal in 3 limbs or the mouth and throat muscles and 2 limbs. If there are sensory abnormalities, the diagnosis of ALS is unlikely. It is a difficult and two competent neurologists should agree on the diagnosis. The physician, specialist nurse, physical therapist, occupational therapist, speech therapist, and dietitian, as a team, all have important roles in helping the patient and the family deal with anger, despair, and final resignation. False hopes and newspaper anecdotes of "cures" will all disturb the inevitable progression.

Glimmers of hope are showing. In animal models, a treatment with naturally occurring nerve growth factors has arrested the disease. Human tests have started. A disease in Guam resembles ALS and may be a slow poisoning from an amino acid in the false Sago plant seeds. Another similar disease lathyrism is due to toxicity from chickling peas. Electric shock can cause a similar disease or working in the leather industry. In the familial form of ALS, a deficiency of an enzyme (superoxide dysmutase) needed to deal with external poisons, has been found. Different insults to the nervous system may singly or together lead to ALS, suggesting that your environment can kill you. The disease is no longer neglected. There is hope.

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See related Patient Topics Amyotrophic Lateral Sclerosis or Brain and Nervous System.

See related Provider Topics or Brain and Nervous System.

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