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Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan

Ling Ling Rong1, Nicholas J Lannen1, Evan C Tank1

  • 1Department of Neurosciences, Corewell Health West, Michigan State University, Grand Rapids, MI, United States.

Frontiers in Neurology
|April 6, 2023
PubMed
Abstract

Insights

A study in West Michigan identified a concerning cluster of Creutzfeldt-Jakob disease (CJD) cases, suggesting a higher local incidence rate. This temporal and spatial aggregation of CJD cases requires immediate public health investigation.

Area of Science:

  • Neuroscience
  • Epidemiology
  • Public Health

Background:

  • Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder with a global incidence of 1-2 per million annually.
  • Fewer than 1,000 CJD cases occur in the United States each year.
  • This study investigates a unique series of temporo-spatial CJD clusters in West Michigan.

Observation:

  • Five CJD cases were identified within two temporal clusters between July 2021 and June 2022 at Corewell Health West hospitals.
  • All patients presented with characteristic CJD patterns on MRI, with four showing positive CSF CJD panels and one with typical EEG findings.
  • Four cases were confirmed as sporadic CJD via autopsy, with all patients succumbing within three months of symptom onset.

Findings:

  • The five patients resided within a 90-mile radius of Grand Rapids, MI, with two living in the same county.
  • The incidence rate in the affected region is estimated at 3.1 to 12.5 per million, significantly higher than the national average.
  • The observed dense temporal and spatial cluster suggests a potential underestimation of CJD incidence in West Michigan.

Implications:

  • This CJD case aggregation presents a significant public health concern in West Michigan.
  • The findings underscore the need for urgent epidemiological investigation into the causes of this cluster.
  • Further research is crucial to understand the factors contributing to the elevated CJD incidence in this region.
Keywords:
Creutzfeldt-Jacob diseaseWest Michiganclusterprionrapidly progressive dementiareal-time quaking-induced conversion

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