Creutzfeldt-Jakob Disease: In-Hospital Demographics Report of National Data in the United States in 2016 and Review of a Rapidly-Progressive Case

Abstract Background This report highlights a rapidly progressive case of Creutzfeldt-Jakob disease (CJD) whose time from symptom onset to death spanned less than two months. We also explore the most recently available in-patient demographics data for discharges with CJD in the United States. Methods We reviewed a CJD case and systematically analyzed a retrospective cohort of CJD discharges using the Healthcare Cost and Utilization Project (HCUP) to evaluate the existing national data on the status of CJD demographics and dispositions in the United States in 2016. Results An estimated total of 710 hospital discharges with a diagnosis of CJD were seen across the United States in 2016. According to HCUP, the average age of patients was 66.15 ± 11.54 years with 48.6% female. Average time to intubation from admission to hospital was 4.71 ± 7.32 days with a rate of intubation of 6.34%. The mean hospital cost was $19,901.25 ± $18,743.48. The rate of in-hospital mortality was 8.45%. No significant geographical differences were noted (p = 0.49). No significant differences were seen among incidence in specific ethnic groups (p = 0.33) or income quartiles (p = 0.90). Conclusions Our data shows that the incidence of CJD in 2016 appears to be equally distributed among individuals in the United States by demographic categories. Additionally, our case-study from 2019 illustrates an important example for diagnosing a rapidly-progressing case of CJD.