Incidence of Creutzfeldt-Jakob Disease in the United States from 2000 to 2019

Background and purpose To test the hypothesis that the incidence of Creutzfeldt-Jakob disease (CJD) has remained constant, we calculated the rate of hospitalizations for CJD in the United States using the National Inpatient Sample (NIS) from 2000 to 2019. Methods We used ICD-9 and ICD-10 codes to identify people hospitalized with presumed CJD in the National Inpatient Sample (NIS) from 2000 to 2019. Survey weights were used to calculate nationally representative estimates. We used 2000 census data to calculate age-adjusted standardized rates of CJD hospitalizations by sex and race-ethnicity and then used Joinpoint regression to evaluate changes in those rates. Results From 2000 to 2019, there were 11,064 admissions for CJD across the U.S. Across this period, the age-adjusted rate of CJD-related hospitalizations increased significantly from 1.25 (95% CI, 1.25-1.26) to 1.98 (95% CI, 1.98-1.99) per million U.S. adults per year, with a significant annual percentage change between 2004 and 2013 of 7.6% (95% CI, 4.4%-10.9%). Conclusions The incidence of CJD increased in the United States from 2000 to 2019, with a significant increase specifically between 2004 and 2013, though the overall case rate remains low. ### Competing Interest Statement Dr. Seitz and Ms. Zhang report no conflicts. Dr. Navi has received personal compensation for medicolegal consulting on neurological disorders. Dr. Kamel serves as a PI for the NIH-funded ARCADIA trial (NINDS U01NS095869), which receives in-kind study drug from the BMS-Pfizer Alliance for Eliquis and ancillary study support from Roche Diagnostics; as Deputy Editor for JAMA Neurology; on clinical trial steering/ executive committees for Medtronic, Janssen, and Javelin Medical; and on endpoint adjudication committees for AstraZeneca, Novo Nordisk, and Boehringer Ingelheim. He has an ownership interest in TETMedical, Inc. Dr. Merkler has received personal compensation for medicolegal consulting on neurological disorders. ### Funding Statement This study did not receive funding. ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: This study was approved by the institutional review board at Weill Cornell Medical College and the need for obtaining informed consent was waived. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes The statistical analysis that supports the findings of this study are available from the corresponding author upon reasonable request. The data that support the findings of this study may be requested from the Agency for Healthcare Research and Quality.