Motor neurone disease of the western Pacific: do the foci extend to Australia?

Abstract

Hyperendemic foci of motor neurone disease, and other neurodegenerative disorders, are located in the western Pacific area, in the Japanese of the Kii Peninsula of Honshu Island, the Chamorros of the Mariana Islands, the Auyu and Jakai of West New Guinea. It is suspected that there is a common aetiologic pathway from toxic metal and essential minerals in these three foci. A fourth focus of motor neurone disease occurs in an isolated tribal group living on the same Pacific longitude, at Angurugu on Groote Eylandt in the Gulf of Carpentaria, Northern Australia. This environment is also characterized by local ecological extremes, including low calcium and iron, and high manganese. The "Angurugu syndrome", described in this paper, shows dysfunction of motor neurones, including upper and lower motor, cerebellar, extrapyramidal and cranial nerves, especially oculomotor. About half the cases emerge in adult life. The others are evident in early childhood. The syndrome is viewed not as simple manganism but as manganism synergistic with accompanying mineral changes. No autopsy studies have been carried out. This paper suggests that this syndrome incurs a loss of the neurotransmitter dopamine. A case study is presented that indicates the unusual range of symptoms, including ataxia, and partial relief by L Dopa (Sinemet).