Creutzfeldt-Jakob disease (CJD) is a rare but fatal neurodegenerative disease. Its prevalence is reportedly one in 1-1.5 million worldwide annually with fewer than 1,000 cases per year in the United States.  

We presented two CJD cases seen between July and August in 2021 at the 2022 AAN annual meeting. From May to June in 2022, we encountered three additional CJD cases in our two hospitals in West Michigan where a rough 1.6 million people reside.

Case Summary:

Case three: A 77-year-old white man, a retired attorney, was transferred to our hospital on May 3^rd with complaints of dyscognia and visual disturbance which started in mid-March. Case four was a 78-year-old white woman, a housewife, admitted on June 3^rd for rapidly cognitive decline over 3 months with subacute cognitive dysfunction the year prior.  Case five was a 64-year-old white woman, a nurse, admitted on June 17^th with rapidly cognitive decline and gait instability with initial symptoms including dyscognia, dizziness and fatigue started in January.

All patients’ MRI brain demonstrated characteristic CJD patterns; CSF reported >98% probability of CJD except in patient four who had inconclusive probability of CJD and T-tau level was 19,937 but RT-QuIC was negative due to discolored sample. Only patient 3's EEG showed characteristic CJD patterns. So far, all five patients have died with two of them confirmed sCJD on autopsy, two patients with pending autopsy results and one patient’s family declined autopsy.

These additional cases suggest an expanding cluster of CJD. Five cases within one year in one community is alarming. All five patients lived within 90-mile radius from Grand Rapids with two patients even in the same county, it warrants urgent investigation for possible regional outbreak of CJD.