National Prion Disease Surveillance Data

¹ Listed based on the year of death or, if not available, on the year of referral;
² Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted. Includes 40 autopsy-coordinated cases pending tissue receipt;
³ Disease acquired in the United Kingdom;
⁴ Disease acquired in the United Kingdom in one case and Saudi Arabia in the other.
⁵ Disease possibly acquired in a Middle Eastern or Eastern European country;
⁶ Includes 60 cases in which the diagnosis is pending (3 from 2025, and 57 from 2026), and 25 inconclusive cases;
⁷ Includes 49 (1 from 2024, 23 from 2025, and 41 from 2026) cases with type determination pending, in which the diagnosis of vCJD has been excluded.
⁸ The sporadic cases include 5,298 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 96 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 43 cases of sporadic Fatal Insomnia (sFI).
⁹ Total does not include 353 Familial cases diagnosed by blood only.

¹ Cases with prion disease confirmed by neuropathological evaluation of brain tissue.
² Cases with a positive RT-QuIC result in cerebrospinal fluid (CSF) that have not undergone neuropathological evaluation of brain tissue.
³ Values include positive RT-QuIC results tested at the Mayo Clinic (12 from 2024, 88 from 2025, and 56 from 2026).
⁴ Combined total of definite and probable cases.