Maddox et al., Prion disease incidence in the United States 2003-2015 (Neurology 2020)

This is the main recent peer-reviewed estimate of how often human prion disease occurs in the United States. Ryan A. Maddox and six co-authors from the CDC and the National Prion Disease Pathology Surveillance Center (NPDPSC) matched national death records for 2003 to 2015 with the NPDPSC case database and counted 5,212 prion disease decedents, giving an age-adjusted average of about 1.2 cases per million people per year. The citation was checked against PubMed and is correct as queued. Information only, not medical advice.

Key facts

  • Citation: Maddox RA, Person MK, Blevins JE, Abrams JY, Appleby BS, Schonberger LB, Belay ED; Neurology 2020;94(2):e153-e157; DOI 10.1212/WNL.0000000000008680; PMID 31757870.
  • Authors are listed at the CDC National Center for Emerging and Zoonotic Infectious Diseases and at the NPDPSC at Case Western Reserve University.
  • Method: decedents were identified from national multiple cause-of-death records for 2003 to 2015 and matched to the NPDPSC database; NPDPSC cases with positive neuropathology or genetic results and no death-record match were added, and cases with negative neuropathology were removed.
  • The study found 5,212 prion disease decedents and an age-adjusted average annual incidence of about 1.2 per million, with yearly values from 1.0 per million (2004 and 2006) to 1.4 per million (2013).
  • Median age at death was 67 years, and incidence at ages 65 and over averaged about 5.9 per million per year.
  • Ten decedents were under 30 and only two of them had sporadic disease, so very young onset is extremely rare.
  • Search excerpts of the abstract (publisher page not readable) report about 89 percent sporadic, about 10 percent familial and under 1 percent acquired by ingestion or medical exposure; these are unconfirmed on a full page.
  • The same excerpts report White decedents roughly twice as likely as Black decedents to be diagnosed and the highest regional rate in the Midwest; unconfirmed on a full page.
  • CDC clinician guidance gives the US incidence as about 1 to 2 cases per million per year and notes CJD typically affects people aged 55 and older, and it lists a 2020 Neurology prion incidence article among its references.
  • Maddox is described as a CDC Prion and Public Health Office epidemiologist in a 2022 podcast discussing this study.

Timeline

  1. 2019Paper published online (22 November).
  2. 2020Paper appears in print in Neurology volume 94, issue 2 (14 January).
  3. 2022Consultant360 podcast with the first author about the study (1 May).

People

Last checked 1 November 2026

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