Enhanced CJD Surveillance in the Older Population

Completed

• Conditions: Dementia; Prion Disease
• Interventions: Other: Medical history questionnaire; Other: Clinical assessment and review; Other: Participant follow-up; Other: Blood or buccal sample; Other: Post mortem examination

• Registration Number: NCT02629640
• Lead Sponsor: University of Edinburgh

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.

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 Timeline

• Study First Submitted: 2015-12-10
• Study First Submitted QC: 2015-12-11
• Study First Posted: 2015-12-14
• Study Start: 2016-01-19
• Primary Completion: 2022-03-31
• Study Completion: 2022-03-31
• Status Verified: 2024-06
• Last Update Submitted: 2024-06-07
• Last Update Posted: 2024-06-10

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 30

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Research Participants	Participant follow-up	Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination.
Research Participants	Medical history questionnaire	Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination.
Research Participants	Blood or buccal sample	Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination.
Research Participants	Clinical assessment and review	Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination.
Research Participants	Post mortem examination	Medical history questionnaire; clinical assessment and review; participant follow-up; blood or buccal sample; post mortem examination.

Primary Outcome Measures

Name	Time	Method
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

Name	Time	Method
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

Trial Locations

Locations (1)

CJD Research and Surveillance Unit, Western General Hospital; 🇬🇧 Edinburgh, United Kingdom