A high incidence of neurodegenerative disease is found within the Chamorro population of the Western Pacific island of Guam, and includes parkinsonism, dementia, and amyotrophic lateral sclerosis (ALS), each of which may occur in isolation but are more commonly combined (121). The cause is unknown but a toxic or viral etiology has been postulated (122-124). The histopathology is dominated by the presence of numerous NFTs (125,126), with similar immunohistochemical, biochemical, and ultrastructural features as those seen in AD (127,128), but usually in the absence of SP (Fig. 6A,B). The anatomical distribution of NFTs is different from AD (129) and more similar to that seen in PSP and postencephalitic parkinsonism (PEP) (130,131). Chronic degenerative changes including neuronal loss and gliosis are found in regions where NFTs are numerous, including the frontotem-poral neocortex, hippocampus, entorhinal cortex, nucleus basalis, basal ganglia, thalamus, subthalamus, substantia nigra, locus ceruleus, and periaqueductal gray (125,126,129). Glial pathology has recently been described and includes argyrophilic, tau-positive coiled bodies in oligodendro-glia and granular inclusions in astrocytes (132). There tends to be large numbers of Hirano bodies and abundant granulovacuolar degeneration in the hippocampus (133). Cases with clinical features of ALS have pathologic changes in the pyramidal motor system, similar to sporadic ALS (134).
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