Prion disease in Indigenous Australians.

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.