Objective:

To investigate the number of (Creutzfeldt-Jakob disease) CJD cases in two tertiary care hospitals located in Staten-Island and Manhattan in 15 months duration. Compare presenting complaints, imaging findings, EEG, zip codes, and significant laboratory results

Background:

CJD is a rare prion disease with an incidence of 1-2 per one million population per year. The population in New York City (NYC) is ~8 million, and the expected CJD incidence should be 8-16 cases annually. 

Design/Methods:

This is a case series and inclusion criteria include CJD cases with positive RT-Quic. Cases were from Staten-Island University and Lenox-Hill Hospitals. EMR was reviewed and data was extracted manually. All cases from February-2023 to April-2024 were included. 

Results:

We found 5 CJD cases (three females and two males; ages 66-81 yo) in only two NYC hospitals in 15 months duration. The most common presenting symptom was acute delirium among five patients. Duration from symptoms onset to time of death was 2-4 months. CSF WBC, protein, and glucose were in the normal range. There was no common pattern of positive lab among these patients. MRI brain showed abnormal restricted diffusion in cerebral cortexes (frontal, parietal, and temporal lobes) in four patients. One patient had an unremarkable MRI. The common EEG finding among all patients was generalized slowing with FIRDA (1 case), and GPDs (2 cases). Locations of the patients include Staten Island (2), Manhattan (2), and Brooklyn (1) with different area zip codes. 

Conclusions:

The incidence of CJD is likely higher than the current estimate. In only two NYC hospitals five CJD cases were reported in15 months period. There are about eleven public NYC-hospitals and numerous private hospitals. Therefore, we suspect a much higher incidence of CJD in NYC than the current incidence rate. There is a need for future epidemiologic studies to recalculate incidence and prevalence of CJD.