Ripken streak brings a look at illness that felled Gehrig


During their coverage of Cal Ripken's record-breaking consecutive-game streak, the media constantly referred to Lou Gehrig's disease. I would like to know more about that disease.

Amyotrophic lateral sclerosis, or ALS, the medical term for Lou Gehrig's disease, is a relentlessly progressive disorder caused by deterioration of the motor nerves (nerves that control the action of muscles) in both the brain and spinal cord. The first manifestations are typically weakness and clumsiness of one or both hands; but the symptoms may begin in almost any muscle, and most muscles are affected as the disease worsens. The muscle weakness is accompanied by a decrease in their size, stiffness and muscle twitches. There is no loss of sensation or pain except for possible muscle cramps. The disease eventually involves the tongue and muscles controlling swallowing and respiration. Death often results from respiratory failure. ALS is invariably fatal; the average lifespan after the onset of symptoms is two to five years.

ALS most often begins between ages 50 and 60, and the disorder becomes more common as people grow older. Lou Gehrig developed ALS at an unusually young age -- less than 3 percent of people with ALS have symptoms before the age 35. ALS affects men about 50 percent more often than women, and it is not exceedingly rare. It is estimated that about one in 800 men in this country dies of ALS.

An inherited genetic disorder is responsible for about 5 percent to 50 percent of the cases of ALS, and the defective gene responsible for this inherited form of ALS was identified in the past few years. In the great majority of patients with ALS, however, no other family members are affected and the underlying cause is completely unknown. Many different medications have been tried in an unsuccessful effort to find a treatment that would cure ALS or at least slow its progression. Some modest success has been achieved recently, and the Food and Drug Administration will soon consider approval of a drug (Riluzole) that has prolonged survival of ALS patients by 5 to 6 months in clinical trials.

Dr. Margolis is professor of medicine and biological chemistry at the Johns Hopkins School of Medicine.

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