Amyotrophic lateral sclerosis and parkinsonism-dementia of Gaum: Clinical, epidemiological, and genetic patterns

American Journal of Human Biology : the Official Journal of the Human Biology Council
Ralph M Garruto

Abstract

The strikingly high incidence of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD), two uniformly fatal neurodegenerative disorders which often occur in the same families and occasionally in the same individual, was recognized on Guam more than three decades ago. Since the first systematic observations began, nearly 800 Guamanian Chamorro patients have been clinically diagnosed as having either disease. The original incidence rates for ALS and PD accounted for one in five deaths among Chamorros over ago 25. During the past 30 years, however, the incidence and mortality rates have dramatically declined and today the risk to Guamanian chamorros is only several-fold higher than that for non-Chamorro residents of the continental United States. The accumulating epidemiological and genetic data strongly suggest that environmental factors are primarily involved in the etiology and pathogenesis of these disorders. The high incidence focus of ALS and PD on Guam and similar, but less well-studied, foci in West New Guinea and the Kii Peninsula of Japan represent natural paradigms of chronic degenerative disease that have provided new information and insights for understanding not only ALS and PD, but other neurological di...Continue Reading

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Citations

Jan 1, 1992·American Journal of Human Biology : the Official Journal of the Human Biology Council·J H Underwood
Jan 1, 1989·American Journal of Human Biology : the Official Journal of the Human Biology Council·Ralph M Garruto
Aug 1, 1991·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·M J Strong, R M Garruto
Jul 1, 1996·Acta Neurologica Scandinavica·Z X ZhangG C Román
Jul 2, 2003·Annals of Neurology·Michael J StrongWencheng Yang
Jan 5, 2002·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·M J Strong
Dec 18, 2020·Frontiers in Neurology·Michael J StrongKathryn Volkening
Jun 5, 2003·Pharmacology & Therapeutics·Michael J Strong

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