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Prion disease in Indigenous Australians.

Peter K Panegyres1,2, Christiane Stehmann3, Genevieve M Klug4

  • 1Neurodegenerative Disorders Research Pty Ltd, Perth, Western Australia, Australia.

Internal Medicine Journal
|April 3, 2020
PubMed
Summary

Creutzfeldt-Jakob disease (CJD) occurs in Indigenous Australians at rates and with clinical presentations similar to non-Indigenous Australians. This study provides crucial data on prion disease occurrence in this population.

Keywords:
Creutzfeldt-Jakob diseaseIndigenous Australiansprion disease

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Area of Science:

  • Neurology
  • Epidemiology
  • Indigenous Health

Background:

  • Indigenous Australians face a higher risk of dementia.
  • Prion diseases, like Creutzfeldt-Jakob disease (CJD), are poorly understood in Indigenous Australians.
  • Previous reports indicated varying CJD incidence in global Indigenous populations.

Purpose of the Study:

  • To determine the incidence rate of Creutzfeldt-Jakob disease (CJD) in Indigenous Australians.
  • To describe the clinical characteristics and phenotype of CJD in this population.
  • To compare CJD occurrence in Indigenous Australians to the general Australian population.

Main Methods:

  • Assessed crude sporadic CJD (sCJD) incidence rates.
  • Performed indirect age standardization for all CJD cases.
  • Calculated the standardized mortality ratio (SMR) for Indigenous Australians compared to the general population.
  • Analyzed clinical phenotypes of CJD cases.

Main Results:

  • Eight Indigenous Australians were identified with sCJD between 2006-2018.
  • The clinical phenotype and median age at death (61 years) were similar to non-Indigenous individuals.
  • The incidence rate and age-standardized mortality ratio for CJD in Indigenous Australians were not significantly different from the general population.

Conclusions:

  • Creutzfeldt-Jakob disease (CJD) affects Indigenous Australians.
  • The occurrence and clinical presentation of CJD in Indigenous Australians mirror those in non-Indigenous Australians.
  • Findings contrast with prior studies suggesting lower CJD incidence in other Indigenous groups.