Abstract
A number of discrete foci of neurological disease have been discovered in the Pacific region. They include amyotrophic lateral sclerosis and parkinsonism-dementia in the Mariana Islands, amyotrophic lateral sclerosis in the Kii peninsula of Japan and in certain parts of New Guinea, spastic diplegia associated with leprosy in New Caledonia, familial periodic paralysis on Tongariki, deafness and spasticity in children associated with goitre in New Guinea, and kuru in the Eastern Highlands of New Guinea. Genetic mechanisms may in part explain these isolated foci of disease. However, the genes responsible have not always arisen in isolation. In addition, exogenous factors are involved, particularly in kuru where a slow virus infection and the practice of cannibalism contribute to the aetiology. The search for a similar virus in the amyotrophic lateral sclerosis of Guam has so far proved negative. The finding of such disease foci is of importance to the study of the human biology of the Pacific region. Furthermore, aetiological discoveries in these diseases, especially kuru, have wide implications in human disease.
Published Version
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