Creutzfeldt-Jakob Disease: In-hospital demographics report of national data in the United States from 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 inpatient 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).

Epidemiological and Clinical Characteristics of Sporadic Creutzfeldt–Jakob Disease: A Retrospective Study in Eastern China

“…The mean age of disease onset in this study (64.42 years old) was comparable with the findings from Korea (65.5 years) (18), Japan (65.5 years) (19), the United States (66.15 years), (20) and Europe (66 years) ( 21), but higher than that of a few Chinese patient groups [58 years ( 14) 60.3 years ( 9)]. Another two surveys in China have produced results essentially in agreement with ours at the median onset age, which was 64 years in our study (8,11).…”

“…At the same time, considering that the different regional demographic features might play a role in the heterogeneity of onset ages among domestic studies, more accurate onset age remains to be verified by large national surveys. Additionally, Kotkowski et al found that age (66 ± 11.5 years) was correlated with sCJD onset (20). As with many other neurodegenerative diseases, sCJD generally occurs in late adulthood.…”

Case report: Two clusters of Creutzfeldt–Jakob disease cases within 1 year in West Michigan

“…Our case series does not support that CJD incidence has no geographical differences ( 4 , 54 ). West Michigan has 1.6 million people, and the combined population of four counties where five patients lived is 395,104 in 2022, which makes the CJD new case rate 3.1 and 12.5 per million people in West Michigan and combined four counties, respectively, which is higher than reported 1 to 2 per million people worldwide and 350–710 cases in the United States annually ( 2 – 5 ) 1 .…”

Genetic Creutzfeldt‐Jakob disease in Turkish Jews—demographic and clinical features

“…The prevalence of sporadic CJD (sCJD) is about 1 million/year worldwide, including in Israel, as reported in various national registries. [4][5][6][7][8][9] This figure is stable in time and similar in different populations with no known geographic or ethnic clusters of patients. No association was found to a specific diet or occupation of the patients.…”

“…The majority of CJD cases are sporadic (85%), approximately 10%–15% of cases are inherited, and transmitted CJD constitutes about 1% of all cases. The prevalence of sporadic CJD (sCJD) is about 1 million/year worldwide, including in Israel, as reported in various national registries 4–9 . This figure is stable in time and similar in different populations with no known geographic or ethnic clusters of patients.…”