By Richard Saltus, The Boston Globe
BOSTON -- It was 60 years ago last May that New York Yankees
first baseman Lou Gehrig benched himself after playing 2,130
consecutive games, and a few weeks later was diagnosed with the
fatal disease that now bears his name.
Gehrig's progressive troubles in playing baseball -- stumbling,
loss of coordination, fatigue, weakness -- were the first signs of
what the medical world calls amyotrophic lateral sclerosis, or ALS.
The cause is unknown, but the devastating effects result from the
death of motor neurons in the brain and spinal cord -- nerve cells
that control muscles throughout the body.
Because there's no cure, Lou Gehrig's disease eventually
incapacitates the person, who is otherwise entirely normal
mentally, and erodes the ability to chew, swallow, and breathe.
Death is unavoidable. On average, people with ALS live about
three years after diagnosis, ``but I always emphasize that there's
huge variability and some people live considerably longer,'' said
Dr. Gilmore O'Neill, a neurologist at Massachusetts General
Hospital. ``There are people living very many years.'' Best-known
is physicist Stephen Hawking, 57, who was diagnosed with ALS when
he was 21 and has carried on a successful career and family life
although he can speak only when using a computerized voice
synthesizer.
Hall of Fame pitcher Jim ``Catfish'' Hunter was one of an
estimated 5,000 people diagnosed last year with ALS, which
typically attacks people between the ages of 40 and 70. About
30,000 people in the United States have the disease at any given
time. It's more common than Huntington's disease, the
neurodegenerative disease that killed folk singer Woody Guthrie,
and about as common as multiple sclerosis, a disease that attacks
the central nervous system.
Hunter, who first noticed weakness in his arms while hunting in
May of last year, is battling ALS at a time of unprecedented hope,
though no dramatic way to combat the disease is on the horizon. But
in contrast to the past, when there was no treatment at all (ALS
was first identified in 1869 by French neurologist Jean-Martin
Charcot), there is now one drug that modestly slows the disease and
several others undergoing testing. In 1995, the Food and Drug
Administration approved the drug Rilutek, which can extend survival
by about 20 percent, said O'Neill.
Another drug, myotrophin, may have a similar effect and is also
in testing. Researchers believe that substances called growth
factors, which nourish nerve cells and combat their degeneration,
could play an important future role in ALS treatment.
A key advance came in 1993 when scientists found a gene mutation
involved in the small minority of ALS cases that are inherited.
That faulty gene causes the body to make an abnormal enzyme -- a
substance that normally helps protect motor nerve cells against
damage by so-called ``free radicals,'' or destructive forms of
oxygen. This may be a clue to treatment, and many patients now take
high doses of antioxidant vitamins in hopes of slowing the damage
to nerve cells.
One reason that O'Neill is optimistic about future advances
against ALS is that ``there's a huge amount of cooperation (among
scientists) in this disease, and a large number of people are
working on it.''
