Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014
Alison Seitz1, Cenai Zhang2, Alexander Merkler2
1New York Presbyterian Hospital, 2Weill Cornell Medicine
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 2014.
We used ICD-9-CM codes to identify adults hospitalized with presumed CJD in the National Inpatient Sample (NIS) from 2000 to 2014. National Inpatient Sample survey weights were used to calculate nationally representative estimates. We used logistic regression and Jointpoint regression to evaluate the trend in the rate of hospitalizations for CJD.
From 2000 to 2014, we identified 8,789 admissions for CJD. Across this period, the rate of CJD-related hospitalizations increased significantly from 16 (95% CI, 12-19) to 29 (95% CI, 24-34) per million hospitalizations (OR, 1.05; 95% CI, 1.04-1.07). Joinpoint regression detected that, from 2000 to 2014, the hospitalization rate significantly increased from 1.6 (95% CI, 1.2-2.0) to 2.7 (95% CI, 2.2-3.2) per million persons (annual percentage change, 4.6%; 95% CI, 3.2%-6.0%). 
The incidence of CJD increased in the United States from 2000 to 2014.