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Q J Med 2000; 93: 617-631
© 2000 Association of Physicians

Commentary papers

Is there evidence for exogenous risk factors in the aetiology and spread of Creutzfeldt-Jakob disease?

C. E. M. Hillier and R. L. Salmon

From the Welsh Combined Centres for Public Health, University of Wales College of Medicine, Cardiff, UK

Introduction

The transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases of unknown aetiology, and affect both animals and man. As a group, they have several unique features that separate them from other neurodegenerative conditions, notably characteristic neuropathology and the ability to be transmitted to experimental laboratory animals by inoculation.

Creutzfeldt-Jakob disease is a rapidly progressive dementia and is uniformly fatal. There appears to be a genetic basis in 10–15% cases, termed familial, and a small number of cases are associated with medical interventions and are termed iatrogenic. The majority of cases appear to arise spontaneously and are, thus, termed sporadic Creutzfeldt-Jakob disease (spCJD). Recently, a number of atypical cases of CJD have arisen, almost exclusively in young people in the UK. This new disease has been termed variant CJD (vCJD) and there is mounting evidence that it is the same disease as the animal TSE, bovine spongiform encephalopathy (BSE).

Aetiology

. . . [Full Text of this Article]

Methods

Definitions

Transmissible virus dementia (synonymous with definite CJD)
Definite or probable CJD
Definite CJD
Probable CJD
Possible CJD
Animal studies

The cause of CJD: findings of case reports, series and surveys

National and regional surveys
Clusters of cases
Libyan Jews
The English clusters
An Italian cluster
The Chilean cluster
Case-to-case transmission in humans: case reports and series in which spread through everyday human contact is suggested
Iatrogenic case-to-case transmission in humans: case reports and series in which iatrogenic transmission has been proposed
Blood transfusion
Dietary factors: case reports and series compatible with the dietary route of infection
Zoonotic transmission: case reports and series compatible with infection from animals
Occupational exposure: observations from case reports and series
Case-control studies

Methods
Selection of cases
Selection of controls
Measurement
Interpreting results
Variant Creutzfeldt-Jakob disease (vCJD)

Background
Cluster
Case-control study
Explaining the source and spread of CJD—hypotheses

Future epidemiological studies

Conclusions

Notes

References


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