Review article

Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy

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.

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.

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).

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.

More than 250 different foodborne diseases have been described to date, annually affecting about one-third of the world's population. The incidence of foodborne diseases has been underreported and underestimated, and the asymptomatic presentation of some of the illnesses, worldwide heterogeneities in reporting, and the alternative transmission routes of certain pathogens are among the factors that contribute to this. Globalization, centralization of the food supply, transportation of food products progressively farther from their places of origin, and the multitude of steps where contamination may occur have made it increasingly challenging to investigate foodborne and waterborne outbreaks. Certain foodborne pathogens may be transmitted directly from animals to humans, while others are transmitted through vectors, such as insects, or through food handlers, contaminated food products or food-processing surfaces, or transfer from sponges, cloths, or utensils. Additionally, the airborne route may contribute to the transmission of certain foodborne pathogens. Complicating epidemiological investigations, multiple transmission routes have been described for some foodborne pathogens. Two types of transmission barriers, primary and secondary, have been described for foodborne pathogens, each of them providing opportunities for preventing and controlling outbreaks. Primary barriers, the most effective sites of prophylactic intervention, prevent pathogen entry into the environment, while secondary barriers prevent the multiplication and dissemination of pathogens that have already entered the environment. Understanding pathogen dynamics, monitoring transmission, and implementing preventive measures are complicated by the phenomenon of superspreading, which refers to the concept that, at the level of populations, a minority of hosts is responsible for the majority of transmission events.

Transmissible spongiform encephalopathies (TSE), also known as prion diseases, are characterized by rapid and fatal neurological decline. They not only detrimentally affect the patient, but also present additional challenges to healthcare systems due to the infectivity of the tissues and the difficulty of inactivating the prion. The most common TSE is Creutzfeldt-Jakob disease (CJD), which can occur after familial, spontaneous or acquired transmission. TSEs received more attention after the development of variant CJD (vCJD), also known as Mad Cow Disease, in the UK during the mid-1990s. Unlike familial or spontaneous CJD, this variant was connected to consumption of cattle contaminated with the prion disease, bovine spongiform encephalopathy.This development increased interest in the etiology of CJD and other TSEs and the risk it presents as an infectious disease. The following details the case of a 59-year-old male infected with CJD presented to our level II trauma center for treatment following a self-inflicted gunshot wound to the head.

It is currently unknown whether chronic wasting disease (CWD), a transmissible spongiform encephalopathy of cervids, is transmissible to humans. Reported on here are the behavioural risk factors and health conditions associated with a six-year follow-up of a known point-source exposure to a CWD infected deer in an Upstate New York community.

Longitudinal.

The Oneida County Chronic Wasting Disease Surveillance Project was launched in 2005 in response to a point-source exposure to a CWD infected deer at a March 2005 Sportsmen's feast in Upstate New York. Eighty-one exposed individuals participated in the 2005 baseline data collection, and were sent follow-up questionnaires following each deer hunting season between 2005 and 2011.

Over a six year period, participants reported a reduction in overall venison consumption. Participants reported no significant changes in health conditions, although several conditions (vision loss, heart disease, type 2 diabetes, weight changes, hypertension, and arthritis), were significantly associated with age.

To this day, this incident remains the only known large-scale point-source exposure to a CWD infected deer. Prion diseases can incubate for multiple decades before the manifestation of clinical symptoms; thus, continued surveillance of this exposed study population represents a unique opportunity to assess the risk of CWD transmission to humans. This project is uniquely situated to provide the first epidemiological evidence of CWD transmission to humans, should it occur.