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A focus of sporadic Creutzfeldt-Jakob disease (sCJD) was previously identified in a coastal region of New South Wales (NSW), Australia. Researchers conducted this study to confirm the focus and to identify a possible cause. The authors retrospectively analyzed prospectively collected registry data from all of Australia and assessed regional data within NSW.
The number of sCJD cases observed in the coastal region (14) was considerably more than the 4 to 5 cases expected. A clever statistical method supported significance. Patients in the region had nothing in common that could explain the excess. Although they were older than sCJD patients in other regions, the regional excess of cases remained significant after adjustment for age. The author…