Married ... With Lou Gehrig's Disease
May 5, 2000 -- First comes love, then comes marriage, then comes ... Lou
Gehrig's disease? This seems to be true for nine married couples in France. In
each case, both husband and wife have been diagnosed -- after years of marriage
-- with the rare nerve disorder amyotrophic lateral sclerosis (ALS, more
commonly known in this country as Lou Gehrig's disease.) Researchers studying
these couples -- and their children -- hope to learn more about what part
environment and genes play in this devastating disease. They presented their
findings here at the 52nd annual meeting of the American Academy of
ALS results in a gradual and ongoing destruction of the nerves that control
the body's muscles. Symptoms include muscle weakness and cramping,
uncontrollable twitching of the hands and feet, and slurred speech. As the
disease progresses, it attacks nerves that connect to the muscles that control
breathing - usually leading to death within 5 years of being diagnosed.
According to the ALS Association, about 5,000 new cases are diagnosed in the
U.S. each year, and there are 30,000 Americans with ALS at any given time.
The cause of ALS is unknown but is believed to be a combination of genes and
environment. For example, a toxin sometimes used as a folk medicine by Pacific
islanders has been associated with symptoms of ALS. Exposure to the virus that
causes polio has also been suspected as a cause.
The French researchers, led by Philippe Corcia, MD, studied nine couples in
which husband and wife both developed ALS. The couples ranged in age from 40 to
80 and lived together for an average of 20 years before coming down with the
disease. When one spouse developed ALS, there was a lag time of 1-30 years
before their partner began showing symptoms, but the average delay was 8
Although husband-wife cases of Parkinson's and Huntington's diseases have
been seen, this is the first report of marital ALS, says senior author William
Camu, MD, a neurologist at the University of Montpellier, France. He explains
that the occurrence is unlikely to be a coincidence.
"The risk of ALS is one in one million, and there are 20 million
[married] couples in France," he says. "However, we have found nine
cases in nine years." The researchers believe this happens because of the
environment the couples share, rather than because of genetic factors. Just to
be safe, however, Camu and his co-investigators are now studying the children
of these nine couples to determine if there is also a genetic factor they can
"It is always interesting to speculate what [results like these] might
mean," says Robin L. Brey, MD, associate professor of neurology at the
University of Texas Health Science Center in San Antonio.