The 65+ Dementia Study: Enhanced surveillance of Creutzfeldt-Jakob Disease in the older population

Principal Investigator:  Professor RSG Knight

The 65+ Dementia Study aims to determine whether there is unrecognised prion disease (including variant CJD, sporadic CJD and other forms of prion disease) in the older population. There have been a total of 178 cases of vCJD to date, however it is estimated that 1 in 2000 people in the UK general population may be infected. The apparent discrepancy between the observed vCJD cases and the relatively high prevalence of infection is poorly understood. However, the possibility exists that doctors could be missing some cases, particularly in older age groups, perhaps because their symptoms are atypical of vCJD and not recognised as a prion disease. A similar situation may also exist for sporadic CJD (sCJD), and other forms of prion disease.

The 65+ Dementia Study involves two groups of participants. Firstly, the study involves the neuropathological screening of Edinburgh Brain & Tissue Bank donations from patients in the 65+ age-group for evidence of prion disease. Specimens undergo standard neurological disease histopathology, prion protein immunocytochemistry, biochemistry and genotyping for evidence of prion disease.

The second approach involves patients aged 65 years or over who have been seen by local Old Age Psychiatry, Medicine of the Elderly or Neurology services in Edinburgh and the Lothians, and are identified as having atypical features of dementia. The patients are invited to meet with the research team to discuss the study, and offered:

1. Clinical assessment, including a review of medical notes and the possibility of a brain scan to help us better understand the illness;

2. Medical history review, which may help identify possible causes for dementia.

3. A small donation of blood to be used for codon-129 polymorphism genotyping and possible use in future research.

4. Telephone follow up within 1 month of joining the study and at 3 monthly intervals thereafter, with further clinical review offered if needed.

5. Post-mortem examination of brain tissue to look for evidence of CJD.

Further information can be found in the study protocol at the link below or if you would like to find out more information about the study, or have any questions, please do not hesitate to contact Dr. Lovney Kanguru, our Senior Epidemiologist managing the study on the contact details below:

Dr Lovney Kanguru

Email: loth.securecjd@nhs.scot

Telephone: 0131 537 2128