Health Protection - Creutzfeldt-Jakob Disease (CJD)

Creutzfeldt-Jakob Disease (CJD) in its classical form was first described in the 1920s and is predominantly (85% of cases) a sporadic disease, with no identifiable cause. It is a rare disease and occurs worldwide at a similar rate of about 0.5 – 1 case per million of the population per year. Approximately 14% of cases are associated with gene mutations (known as familial CJD). Less than 1% of cases are iatrogenic (accidentally transmitted from one patient to another as a result of medical interventions). In sporadic cases the average age at onset is between 55 and 75, although younger patients are found in iatrogenic and familial cases.

In 1996 the National CJD Surveillance Unit in Edinburgh identified a previously unrecognised disease pattern, New Variant CJD, now known just as Variant CJD (vCJD). vCJD is a similar neuro-degenerative disease to CJD, however, the peak incidence is much lower (around age 27) and at post mortem examination the brain tissue displays a significantly different pattern under the microscope.

Statistics on the numbers of cases of CJD are published monthly

Department of Health monthly figures

A number of expert committees provide advice to the Department on CJD and BSE issues  including:

For more information go to the Department of Health guidance documents.

Guidance on local reporting of Creutzfeldt-Jakob Disease (CJD) and local action by Consultants in Communicable Disease Control (CsCDC).

This document describes a system for reporting cases of CJD to Consultants in Communicable Disease Control and their equivalents in the UK.

Protocol for the investigation of Geographically Associated Cases of variant Creutzfeldt-Jakob Disease.

This document describes a system for investigating geographically associated cases of vCJD.

Variant Creutzfeldt-Jakob Disease (vCJD): Advice to Practitioners of Complementary and Alternative Medicine.

Advisory Committee on Dangerous Pathogens HSE, 1996
ISBN 0 7176 1212 0
Price: £5.50

Spongiform Encephalopathy Advisory Committee
MSO 1995
ISBN 0 11 242 9874
£35.00

Advisory Committee on Dangerous Pathogens
HMSO, 1994
ISBN 0 11 321805 2
Price £6.50.