Enhanced CJD Surveillance in the Older Population
Study Details
Study Description
Brief Summary
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.
| Condition or Disease | Intervention/Treatment | Phase |
|---|---|---|
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Detailed Description
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.
Study Design
Arms and Interventions
| Arm | Intervention/Treatment |
|---|---|
| Research Participants Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination. |
Other: Medical history questionnaire
Epidemiological review of past medical/dental history; residential history; occupational history; family history of neurodegenerative disease.
Other: Clinical assessment and review
History of current illness; neurological examination including general appearance, full cognitive assessment and physical examination; MRI research brain scan.
Other: Participant follow-up
Hospital patient management system; regular telephone contact with patient/representative.
Other: Blood or buccal sample
Codon-129 genotyping; storage for future research.
Other: Post mortem examination
Brain tissue donation to the Edinburgh Brain and Tissue Bank for research,including investigations for evidence of prion disease.
|
Outcome Measures
Primary Outcome Measures
- 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
- 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]
Eligibility Criteria
Criteria
Inclusion Criteria:
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Patients aged 65 years or above
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Patients accessing NHS Lothian neurology and psychogeriatric services
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Patients who have features atypical for the recognised forms of dementia
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Appropriate informed consent
Exclusion Criteria:
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Patients aged below 65 at time of recruitment
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Patients diagnosed with a clear alternative demonstrable pathology
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | CJD Research and Surveillance Unit, Western General Hospital | Edinburgh | United Kingdom | EH4 2XU |
Sponsors and Collaborators
- University of Edinburgh
- NHS Lothian
- Department of Health, United Kingdom
Investigators
- Principal Investigator: Anna M Molesworth, PhD, University of Edinburgh
Study Documents (Full-Text)
None provided.More Information
Publications
None provided.- 15/SS/0196