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Abstract Details

Five Creutzfeldt-Jakob Disease Cases Within One Year in Spectrum Health Hospitals in Grand Rapids, Michigan
General Neurology
P10 - Poster Session 10 (8:00 AM-9:00 AM)
12-007

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.

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Case Summary:

Case three: A 77-year-old white man, a retired attorney, was transferred to our hospital on May 3rd 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 3rd 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 17th 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.   

Authors/Disclosures
Jiangyong Min, MD (Corewell Health West)
PRESENTER
Dr. Min has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbott . Dr. Min has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Medtronic .
Ling Ling Rong, MD (Corewell Health West, Michigan State University) Dr. Rong has nothing to disclose.
Nicholas Lannen, MD Dr. Lannen has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Genentech. Dr. Lannen has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for TG Therapeutics. Dr. Lannen has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Horizon Theraputics.
Evan Christopher *use 387 Tank, DO (UMHS) Dr. Tank has nothing to disclose.
Jessica Feistel, DO Dr. Feistel has nothing to disclose.
Anvita Potluri, MD Dr. Potluri has nothing to disclose.
Michelle Del Castillo DeJesus Brazitis, MD (Spectrum Health) Dr. DeJesus Brazitis has nothing to disclose.
Muhib Khan, MD, FAAN (Mayo Clinic ) The institution of Dr. Khan has received research support from NINDS. The institution of Dr. Khan has received research support from Genentech. The institution of Dr. Khan has received research support from Spectrum Health-MSU alliance. Dr. Khan has received research support from NIH.