A dominant hereditary ataxia resembling Machado-Joseph disease in Arnhem Land, 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.
Burt
T
T
Menzies School of Health Research, Darwin, Australia.
Blumbergs
P
P
Currie
B
B
D000328:Adult; D001259:Ataxia / Q000208:ethnology / Q000235:genetics / Q000473:pathology*; D001315:Australia; D001927:Brain Diseases / Q000208:ethnology / Q000235:genetics / Q000473:pathology*; D006801:Humans; D017827:Machado-Joseph Disease / Q000208:ethnology / Q000235:genetics / Q000473:pathology*; D008297:Male; D008345:Manganese; D009784:Occupational Diseases; D011174:Portugal
D008345:Manganese