Human Prion Diseases in the United States

Abstract

BackgroundPrion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. The most common form of human prion disease, Creutzfeldt-Jakob disease (CJD), occurs worldwide. Variant CJD (vCJD), a recently emerged human prion disease, is a zoonotic foodborne disorder that occurs almost exclusively in countries with outbreaks of bovine spongiform encephalopathy.This study describes the occurrence and epidemiology of CJD and vCJD in the United States.Methodology/Principal FindingsAnalysis of CJD and vCJD deaths using death certificates of US residents for 1979–2006, and those identified through other surveillance mechanisms during 1996–2008. Since CJD is invariably fatal and illness duration is usually less than one year, the CJD incidence is estimated as the death rate. During 1979 through 2006, an estimated 6,917 deaths with CJD as a cause of death were reported in the United States, an annual average of approximately 247 deaths (range 172–304 deaths). The average annual age-adjusted incidence for CJD was 0.97 per 1,000,000 persons. Most (61.8%) of the CJD deaths occurred among persons ≥65 years of age for an average annual incidence of 4.8 per 1,000,000 persons in this population. Most deaths were among whites (94.6%); the age-adjusted incidence for whites was 2.7 times higher than that for blacks (1.04 and 0.40, respectively). Three patients who died since 2004 were reported with vCJD; epidemiologic evidence indicated that their infection was acquired outside of the United States.Conclusion/SignificanceSurveillance continues to show an annual CJD incidence rate of about 1 case per 1,000,000 persons and marked differences in CJD rates by age and race in the United States. Ongoing surveillance remains important for monitoring the stability of the CJD incidence rates, and detecting occurrences of vCJD and possibly other novel prion diseases in the United States.

Diagnostic profiles of patients with late-onset Creutzfeldt–Jakob disease differ from those of younger Creutzfeldt–Jakob patients: a historical cohort study using data from the German National Reference Center

“…Agespecific incidence rates increase with age in most neurodegenerative diseases (e.g. Alzheimer's disease, Parkinson's disease) [9]; however, sCJD incidence rates are highest between 60 and 75 years of age and drop down thereafter [1,4,5]. The drop was located at a cut-off of 75 years in a USbased study by Holman et al, whereas it seemed to be slightly higher in two European studies available.…”

Prion transmission

“…Prion diseases are also well documented within human medicine where CreutzfeldtJakob disease (CJD) is the most common disorder, affecting around one in a million of the population. 2 One of the most important developments in prion diseases is the recognition that the BSE agent, a prion disease of cattle, can infect multiple species including cats, goats and humans via the food chain. [3][4][5] In humans, BSE is known to be the causal agent of variant CJD (vCJD), a prion disorder with a novel human pathology.…”

Intracranial Procedures and Expected Frequency of Creutzfeldt-Jakob Disease

“…Deaths due to CJD were defined as death records which included an ICD-9 code of 046.1 or an ICD-10 code of A81.0. Literal text searches of death records using Super MICAR (Mortality Medical Indexing, Classification, and Retrieval system) were used to identify additional or miscoded records (full methodology, Holman et al [6] ).…”

“…Worldwide, CJD occurs at a rate of 0.5-2 cases per million people per year; in the United States, that figure is roughly 1 per million people per year [3][4][5][6] . Around 85% of CJD cases occur sporadically with no known transmission source, 10-15% are genetically inherited, and a small percentage is acquired [7] .…”