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July 1966

Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex on Guam: Further Pathologic Studies

Author Affiliations

From Henry and Lucy Moses Research Laboratories of the Laboratory Division, Montefiore Hospital and Medical Center, Bronx, NY. Visiting Scientist, Epidemiology Branch, National Institute of Neurological Diseases and Blindness (NINDB), National Institutes of Health (NIH), and now neuropathologist, Laboratory Division, Montefiore Hospital and Medical Center, New York (Dr. Hirano); Professor of Psychiatry and Neuropathology, University of California, San Francisco (Dr. Malamud); formerly Visiting Scientist (Neurologist), NINDB Research Center, Guam, and now neurologist, NINDB, NIH, Bethesda, Md (Dr. Elizan); and formerly Chief, Epidemiology Branch, NINDB, NIH, and now Chief, Section of Medical Statistics, Epidemiology and Population Genetics, Mayo Clinic, Rochester, Minn (Dr. Kurland).

Arch Neurol. 1966;15(1):35-51. doi:10.1001/archneur.1966.00470130039004

THE EXTRAORDINARY prevalence of fatal neurological diseases on Guam has evoked the interest of a number of investigators for the last 15 years. The best known diseases affecting the indigenous Chamorro people are a type of amyotrophic lateral sclerosis (ALS), apparently unique to Guam,1 where, in addition to the classical histological changes of ALS, Alzheimer's neurofibrillary changes are present in specific parts of the central nervous system (CNS); and parkinsonism dementia complex (PD) in which the same neurofibrillary changes are consistently observed.2

At this point one may obviously ask whether these diseases are two distinct entities or are in some way related. This relationship has recently been discussed from the clinical3 and epidemiological4 stand-points. In this paper we would like to provide neuropathological evidence for such a relationship. For this purpose we have examined tissues associated with both diseases regardless of the principal clinical

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