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Contact: Internet: hokef@mail.med.upenn.edu |
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June 1997 Clues to ALS, Alzheimer's,
and Parkinson's Diseases Sought in Guamanian
Disorder In 1945, just after World War II's end and only a few
years after 37-year-old baseball player Lou Gehrig died in
1941 of amytrophic lateral sclerosis, or ALS, military
physicians stationed on the Pacific island of Guam
identified a new neurodegenerative disease. The disease,
later named Lytico-Bodig, appeared to combine some of the
most fearsome symptoms of ALS, Alzheimer's, and Parkinson's
diseases, including movement difficulties, memory loss, and
dementia. Further study revealed that the disease struck the
island's minority Chamorro population at rates about 100
times higher than the incidence rates in the United States
for the better known neurological diseases. In the decades since then, scientists have been drawn to
the remote island and to the Chamorro to try to learn the
genetic or environmental -- or combined -- causes of the
debilitating disorder. So far, answers have proven elusive.
This year, 35 investigators from six research institutions
combined resources under a cooperative grant from the
National Institute on Aging to again attack the problem of
the mysterious Guamanian disease and, at the same time they
hope, provide a critical boost to our still-limited
understanding of ALS, Alzheimer's, and Parkinson's
diseases. "From a societal and humane point of view, this is a
devastating illness, and we would like to be able to help
these patients," says Virginia M.-Y. Lee, PhD, professor of
pathology and laboratory medicine at the University of
Pennsylvania Medical Center and one of eight principal
investigators on the project. "From a scientific point of
view, the disease presents a unique research setting in
which we may begin to understand the causes and mechanisms
of other neurodegenerative diseases." Lee notes that neuronal tissue from Lytico-Bodig patients
is characterized by neurofibrillary tangles in the absence
of the amyloid plaques that -- with the tangles -- have long
defined Alzheimer's disease. As a result, research into the
disease on Guam may help resolve a long-standing debate
among scientists as to which pathological feature might be
the primary cause of Alzheimer's disease. "Also
interesting," Lee adds, "is the possibility that the
Chamorro may harbor a risk-factor gene -- not a gene that
leads irrevocably to disease but one that, in interaction
with environmental factors, may trigger disease."
Alzheimer's disease is suspected to result from one or more
such genes, she says. Also a principal investigator on the project is John Q.
Trojanowski, MD, PhD, a professor of pathology and
laboratory medicine and director of the Alzheimer's Disease
Center at Penn. The overall project is being led by W.C.
Wiederholt, MD, at the University of California, San Diego,
and Ulla-Katrina Craig, DrPH, at the University of Guam. The University of Pennsylvania Medical Center's sponsored research ranks fifth in the United States, based on grant support from the National Institutes of Health, the primary funder of biomedical research in the nation -- $149 million in federal fiscal year 1996. In addition, for the second consecutive year, the institution posted the highest growth rate in its research activity -- 9.1 percent -- of the top 10 U.S. academic medical centers during the same period. News releases from the University of Pennsylvania Medical Center are available to reporters by direct e-mail, fax, or U.S. mail, upon request. They are also posted electronically to the medical center's home page (http://www.uphs.upenn.edu) and to EurekAlert! (http://www.eurekalert.org), an Internet resource sponsored by the American Association for the Advancement of Science. |
