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

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and uniformly fatal neurodegenerative disease. The reported incidence of CJD is 1 to 2 per million people worldwide annually, with fewer than 1,000 cases in the United States per year. In this study, we report a unique case series on temporo-spatial clusters of CJD cases in West Michigan.

Methods: A total of five CJD cases consisting of two temporal clusters were seen from July 2021 to June 2022 at Corewell Health West hospitals. All patients had brain MRI, EEG, and CSF tests. Four patients underwent autopsies.

Results: All patients' MRIs showed characteristic CJD patterns. Four patients had positive CJD panels in CSF. One patient had typical CJD EEG findings. Four patients were confirmed as sporadic CJD by autopsy. All patients died within 3 months after CJD was suspected.

Discussion: All patients lived within a 90-mile radius of Grand Rapids, MI, and two lived in the same county. West Michigan has a population of 1.6 million people, and the four counties where five patients lived have a combined population of 395,104, indicating CJD's new case rate of 3.1 and 12.5 per million people, respectively. Corewell Health is one of the three major healthcare systems in West Michigan. The actual incidence of CJD in West Michigan is likely even higher. This dense temporal and spatial cluster of CJD cases poses a serious public health challenge and warrants urgent investigation.

Keywords: Creutzfeldt-Jacob disease; West Michigan; cluster; prion; rapidly progressive dementia; real-time quaking-induced conversion.

Figure 1

Timeline of two clusters of five CJD cases.

Figure 2

Axial MRI brain diffusion weighted images of five CID patients. (1a) Symmetric diffusion restriction in bilateral caudate nuclei and putamina. (2a) Subtle diffusion restriction involving the left caudate head, and left parletal cortex. (3a–c) Cortically based diffusion restriction involving the posterior left greater than right parietal lobes and paramedian left frontoparietal region. (4a–c) Intense cortically based restriction involving the paramedian and lateral parietal cortices, bilateral temporal cortices, and to a lesser extent the left greater that right frontal lobes. (5a, b) Relatively symmetric DWI within the caudate nuclei; subtle involvement of the posteromedial left hippocampus. Patient sequence was labeled from 1 to 5; red arrows point to sites of abnormal diffusion restriction.

Figure 3

Typical samples of five patients in a standart anterior to posterior bipolar montage. Patient 1: background slowing and triphasic wave; patient 2: generalized rhythmic delta activity; patient 3: periodic sharp wave complexes (PSWC) at 1 Hz; patient 4: non-periodic triphasic wave; patient 5: normal EEG.