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

Burt, T; Blumbergs, P; Currie, B · Neurology · 1993

case_series · Level IV

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Abstract

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.

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