Examining an Unexpected Increase in Creutzfeldt-Jakob Disease — New Jersey, 2024

What to know

  • Presentation Day/Time: Friday, April 24, 9:45 AM
  • Presenter: Ryan Snead, PhD, MPH, EIS officer assigned to the New Jersey Department of Health
Ryan Snead, PhD, MPH

The Issue

  • Creutzfeldt-Jakob disease (CJD) is a rare, uniformly fatal neurodegenerative disorder with a potentially decades-long incubation period. During 2018–2023, New Jersey healthcare facilities collectively reported fewer than seven cases per year. However, during 2024, facilities reported 19 cases.

What We Did

  • We studied laboratory and medical records to verify the 2024 cases. We verified the increase in cases during 2018–2023 to 2024 by comparing the yearly CJD reports required by facilities with the laboratory reports faxed by the National Prion Disease Pathology Surveillance Center (NPDPSC) to the New Jersey Department of Health.

What We Found

  • The NPDPSC performs more than 90% of the CJD diagnostic testing in New Jersey and faxes all their results to the state health department. Facilities reported 29% of probable and confirmed CJD cases during 2018–2023 compared to 90% in 2024. The increase in 2024 occurred because of increased case reporting, highlighting historic underreporting.

What This Means

  • Connecting NPDPSC to the New Jersey reporting system to electronically report positive results might improve reliability of surveillance and address underreporting from facilities.