Enhanced CJD Surveillance in the Older Population

Study Overview
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Brief Summary
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The purpose of this study to investigate if cases of Creutzfeldt-Jakob Disease (CJD) and other forms of prion disease are being missed in older adults living within Lothian.

Detailed Description
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The study involves patients aged 65 years or over who have been seen by a neurologist or psycho-geriatrician and identified as having features that are atypical for the known types of dementia. All patients will be invited to an initial meeting with the research team to discuss the study and, if they agree to participate, for clinical assessment and epidemiological review. Participants will be asked to donate blood or a buccal sample for codon-129 polymorphism genotyping and may be offered a MRI brain scan if no such imaging has been previously undertaken. Participants will also be asked to consider if, in the event of their death, they would donate samples of brain tissue to the Edinburgh Brain Bank for use in this and future research. Telephone follow up will be offered within 1 month of joining the study and at 3 monthly intervals thereafter, with further clinical review offered if deemed appropriate.

Official Title
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Enhanced CJD Surveillance in the Older Population

Conditions
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Prion Disease, Dementia

Intervention / Treatment
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* Other: Medical history questionnaire
* Other: Clinical assessment and review
* Other: Participant follow-up
* Other: Blood or buccal sample
* Other: Post mortem examination


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients aged 65 years or above Patients accessing NHS Lothian neurology and psychogeriatric services Patients who have features atypical for the recognised forms of dementia Appropriate informed consent Exclusion Criteria: Patients aged below 65 at time of recruitment Patients diagnosed with a clear alternative demonstrable pathology

Ages Eligible for Study
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Minimum Age: 65 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Research Participants<br>Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination. | Other: Medical history questionnaire<br>* Epidemiological review of past medical/dental history; residential history; occupational history; family history of neurodegenerative disease.<br>Other: Clinical assessment and review<br>* History of current illness; neurological examination including general appearance, full cognitive assessment and physical examination; MRI research brain scan.<br>Other: Participant follow-up<br>* Hospital patient management system; regular telephone contact with patient/representative.<br>Other: Blood or buccal sample<br>* Codon-129 genotyping; storage for future research.<br>Other: Post mortem examination<br>* Brain tissue donation to the Edinburgh Brain and Tissue Bank for research,including investigations for evidence of prion disease.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evidence of prion disease pathology in the Lothian population, a description of the associated clinical/pathological and epidemiological features and referral characteristics, and how this compares with other cases of prion disease. |  | 4.5 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Description of clinical, pathological and epidemiological characteristics of patients with atypical features of dementia in the Lothian population. |  | 4.5 years | 
| Assessment of the suitability of methods to support an extended system of enhanced CJD surveillance in the rest of the UK. |  | 4.5 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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vCJD, prion disease, dementia, old-age, surveillance, brain banking, public health