Atlas of foodborne infections
transmitted by contaminated food and water

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Creutzfeldt-Jacob disease and new variant CJD

CZ: Creutzfeld-Jakobova nemoc
EN: Creutzfeldt-Jacob disease

Occurrence:
Meat and Meat Products


Category:
Prions


Foodborne Disease:
Untitled document

Early in the illness, patients usually experience behavioral changes, which most commonly take the form of depression or, less often, a schizophrenialike disorder.

Neurological signs such as unsteadiness, difficulty walking and involuntary movements develop as the illness progresses and, by the time of death, patients become completely immobile and mute.


Description:
Untitled document

Creutzfeldt-Jacob disease and new variant CJD

CJD is a fatal disease of humans, first described in the 1920s and found worldwide. CJD is predominantly a sporadic disease, but about 14% of cases are familial (inherited) and associated with genetic mutations. Less than 1% are iatrogenic (i.e. accidentally transmitted from person to person as a result of medical or surgical procedures).

Classically, sporadic CJD occurs in those over 65 years of age and presents as a rapidly progressive dementia with myoclonus (shock-like contractions of isolated muscles), usually fatal within 6 months. Surveillance of CJD, a human neurological disease, was reinstituted in the UK in 1990 to evaluate any changes in the pattern of the disease that might be attributable to BSE. The overall incidence of CJD rose in the UK in the 1990s, although a portion of this increase was due to improved ascertainment of CJD in the elderly as a result of the reinstitution of surveillance.

New variant CJD, also referred to as variant CJD (vCJD), is a newly recognized disease in humans, which was first diagnosed in the UK in the mid-1990s. In contrast to the traditional forms of CJD, vCJD has affected younger patients (average age 29 years) and has a longer duration of illness (approximately 14 months).

The link between BSE and vCJD
A geographical association exists, whereby the majority of BSE cases occurred in the UK and the majority of vCJD cases were also reported there. The emergence of BSE preceded vCJD, indicating a temporal association. Studies of stored human brain tissue internationally have not identified the histopathological changes characteristic of vCJD before the current BSE epidemic. Incubation period and pathological lesion studies in mice and molecular typing studies demonstrate that vCJD is similar to BSE but different from other TSE. It is now widely accepted that vCJD was transmitted to humans through the consumption of contaminated food.
Estimates of future prevalence of vCJD vary widely as too little is known about the disease, especially regarding the incubation period between exposure to the infective agent and the emergence of symptoms.


 


Pictures:

Creutzfeldt
Source: Look at more pictures - part BSE

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