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Surveillance and scientific research are integral and interdependent components of the continued monitoring and investigation of CJD.  Surveillance activities are wide-ranging and involve investigating the clinical and diagnostic features, epidemiology, neuropathology, prion protein biochemistry, molecular genetics and CSF biochemistry of referred cases. The Unit also works on a significant number of research projects, in a wide range of professional fields, with collaborators in the UK, Europe and internationally.

Whilst surveillance underpins research, the findings from research can play an important role in surveillance, for example in developing sensitive and specific methods for diagnosis or by identifying risk factors for disease. This provides an opportunity to improve the understanding of this fatal group of diseases and inform decisions on public health. Our current focus includes the evaluation of the risk of secondary transmission of vCJD, characterising infection in different genetic subgroups, determining whether atypical animal TSEs can infect humans, and the development of diagnostic tests for CJD. Further details are given in the project tabs.

The Transfusion Medicine Epidemiology Review (TMER)

The Transfusion Medicine Epidemiology Review (TMER) is a collaborative project between the UK NCJDRSU and the UK Blood Services.  The main purpose is to investigate whether there is any evidence that Creutzfeldt-Jakob disease (CJD) or variant Creutzfeldt-Jakob disease (vCJD) may have been transmitted via the blood supply.

Prion Surveillance in Primary Immunodeficiency Patients

This study started in 2006, and aimed to find out whether any evidence of prion infection could be found in antibody deficient patients who received certain UK-sourced immunoglobulin products between 1996 & 2000. The products were made from plasma from UK donors and have been infected with a particular type of prion that causes variant Creutzfeldt-Jakob Disease (vCJD).

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

The 65+ Dementia Study aims to determine whether there is unrecognised prion disease in the older Lothian population. Around 100 people in the UK are diagnosed with prion disease every year, however we think that more might be infected but their illness may not have been recognised, perhaps because the signs and symptoms are similar to different forms of dementia. This research will use patient assessment, blood samples, brain scans and samples of brain tissue from people in Lothian when they die, to find if prion disease is being missed and why.