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A dominant hereditary ataxia resembling Machado-Joseph disease in Arnhem Land, Australia

Menzies School of Health Research (Drs. Burt and Currie), Darwin; the Institute of Medical and Veterinary Science (Dr. Blumbergs), Adelaide; and the Royal Darwin Hospital (Dr. Currie), Darwin, Australia.

A dominant ataxia among four families of the Arnhem Land Aboriginal people of Northern Australia has many clinical features in common with Machado-Joseph disease. Neuropathology on one case showed multiple system involvement, with sparing of the inferior olives and cerebellar cortex, consistent with Machado-Joseph disease. Portuguese ancestry is possible, although not proven.

Address correspondence and reprint requests to Dr. T. Burt, Menzies School of Health Research, P.O. Box 41096, Casuarina NT 0811 Australia.

Received October 9, 1992. Accepted for publication in final form January 6, 1993.

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