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

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