National Prion Monitoring Cohort
Research type
Research Study
Full title
A longitudinal observation study of all patients diagnosed with or at high risk of developing human prion disease
IRAS ID
345370
Contact name
John Collinge
Contact email
Sponsor organisation
University College London
Clinicaltrials.gov Identifier
5926, CPMS code ; 08/H0716/85, REC reference
Duration of Study in the UK
19 years, 5 months, 31 days
Research summary
The National Prion Monitoring Cohort protocol outlines an observational study collecting prospective data and samples from patients diagnosed with or at high risk for human prion disease. This study, informed by the experience of the National Prion Clinic (NPC) and the MRC PRION-1 trial, aims to monitor clinical progression and response to treatments in prion diseases. It will help determine the natural history of prion diseases, identify standardized outcome measures, assess surrogate markers, and provide prognostic data for patients and their families.
The cohort consists of three groups:
- Symptomatic patients with confirmed prion disease.
- Asymptomatic individuals with confirmed preclinical prion infection or disease.
- Asymptomatic individuals at high risk due to known exposure or genetic factors.Data will be collected systematically, with intensity and frequency tailored to each group. Standard data will include routine clinical management and basic neurological exams, while additional consent will be required for optional more extensive procedures like MRI, EEG, and lumbar punctures. Future therapeutic studies may be nested within this cohort, requiring separate ethical approval.
Clinical and neuropsychological assessments, non-invasive procedures, and some invasive tests will be performed based on patient consent. Group-based psychological interventions will also be included. Results from individual research projects involving genetic analysis or additional invasive tests will be collected but not conducted routinely within the cohort. The detailed data collection process will support study design and allow comparison across different studies.
REC name
London - Queen Square Research Ethics Committee
REC reference
24/LO/0510
Date of REC Opinion
4 Sep 2024
REC opinion
Further Information Favourable Opinion