A dominant hereditary ataxia resembling Machado-Joseph disease in Arnhem Land, Australia
- PMID: 8414025
- DOI: 10.1212/wnl.43.9.1750
A dominant hereditary ataxia resembling Machado-Joseph disease in Arnhem Land, Australia
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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