Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale
Abstract Background The Medical Research Council-Prion Disease Rating Scale (MRC-PDRS) is a 20-point clinician-reported outcome scale to assess disease progression in patients with prion disease, an invariably fatal neurodegenerative disease caused by misfolded prion protein. This study aims to eval...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
SpringerOpen
2025-05-01
|
| Series: | Journal of Patient-Reported Outcomes |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s41687-025-00884-3 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849312061352312832 |
|---|---|
| author | Leah Leidy Aaron Yarlas Robert S. Pulido Jessica Ludwig Kathleen Glisic Brian S. Appleby |
| author_facet | Leah Leidy Aaron Yarlas Robert S. Pulido Jessica Ludwig Kathleen Glisic Brian S. Appleby |
| author_sort | Leah Leidy |
| collection | DOAJ |
| description | Abstract Background The Medical Research Council-Prion Disease Rating Scale (MRC-PDRS) is a 20-point clinician-reported outcome scale to assess disease progression in patients with prion disease, an invariably fatal neurodegenerative disease caused by misfolded prion protein. This study aims to evaluate the measurement properties and interpretability of the MRC-PDRS to support the measure’s use for effective disease management and research evaluating effectiveness of treatment options for prion diseases. Methodology Utilizing patient data from the Telemedicine Assessment Program for CJD (TAPCJD), statistical assessment was conducted of internal consistency, construct validity (including convergent, divergent validity, and known-groups discriminant validity), responsiveness, and interpretation guidelines using distribution-based approaches to estimate thresholds indicating minimal important change (MIC) in MRC-PDRS scores. Criterion measures used for evaluating construct validity and responsiveness included the Telephone Interview for Cognitive Status (TICS) and Neuropsychiatric Inventory–Questionnaire (NPI-Q). Results/Conclusions These findings provide strong preliminary evidence that the MRC-PDRS is reliable, valid, and responsive as a tool for measuring disease progression in patients with prion disease, with preliminary MIC estimates ranging from 1 to 3 points. This supports the use of MRC-PDRS in evaluating potential treatment benefits of prion disease clinical trials, and potentially in clinical practice settings. |
| format | Article |
| id | doaj-art-f4833fa1e55e4c6e8f56c653f803b5ce |
| institution | Kabale University |
| issn | 2509-8020 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Journal of Patient-Reported Outcomes |
| spelling | doaj-art-f4833fa1e55e4c6e8f56c653f803b5ce2025-08-20T03:53:12ZengSpringerOpenJournal of Patient-Reported Outcomes2509-80202025-05-019111110.1186/s41687-025-00884-3Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating ScaleLeah Leidy0Aaron Yarlas1Robert S. Pulido2Jessica Ludwig3Kathleen Glisic4Brian S. Appleby5Department of Pathology, Case Western Reserve University/University Hospitals Cleveland Medical CenterIonisIonisDepartment of Pathology, Case Western Reserve University/University Hospitals Cleveland Medical CenterDepartment of Pathology, Case Western Reserve University/University Hospitals Cleveland Medical CenterDepartments of Neurology and Psychiatry, Case Western Reserve University/University Hospitals Cleveland Medical CenterAbstract Background The Medical Research Council-Prion Disease Rating Scale (MRC-PDRS) is a 20-point clinician-reported outcome scale to assess disease progression in patients with prion disease, an invariably fatal neurodegenerative disease caused by misfolded prion protein. This study aims to evaluate the measurement properties and interpretability of the MRC-PDRS to support the measure’s use for effective disease management and research evaluating effectiveness of treatment options for prion diseases. Methodology Utilizing patient data from the Telemedicine Assessment Program for CJD (TAPCJD), statistical assessment was conducted of internal consistency, construct validity (including convergent, divergent validity, and known-groups discriminant validity), responsiveness, and interpretation guidelines using distribution-based approaches to estimate thresholds indicating minimal important change (MIC) in MRC-PDRS scores. Criterion measures used for evaluating construct validity and responsiveness included the Telephone Interview for Cognitive Status (TICS) and Neuropsychiatric Inventory–Questionnaire (NPI-Q). Results/Conclusions These findings provide strong preliminary evidence that the MRC-PDRS is reliable, valid, and responsive as a tool for measuring disease progression in patients with prion disease, with preliminary MIC estimates ranging from 1 to 3 points. This supports the use of MRC-PDRS in evaluating potential treatment benefits of prion disease clinical trials, and potentially in clinical practice settings.https://doi.org/10.1186/s41687-025-00884-3Prion diseaseCreutzfeldt-Jakob diseaseMRC Prion Disease Rating ScalePsychometric validation |
| spellingShingle | Leah Leidy Aaron Yarlas Robert S. Pulido Jessica Ludwig Kathleen Glisic Brian S. Appleby Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale Journal of Patient-Reported Outcomes Prion disease Creutzfeldt-Jakob disease MRC Prion Disease Rating Scale Psychometric validation |
| title | Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale |
| title_full | Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale |
| title_fullStr | Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale |
| title_full_unstemmed | Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale |
| title_short | Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale |
| title_sort | internal consistency construct validity and responsiveness of the mrc prion disease rating scale |
| topic | Prion disease Creutzfeldt-Jakob disease MRC Prion Disease Rating Scale Psychometric validation |
| url | https://doi.org/10.1186/s41687-025-00884-3 |
| work_keys_str_mv | AT leahleidy internalconsistencyconstructvalidityandresponsivenessofthemrcpriondiseaseratingscale AT aaronyarlas internalconsistencyconstructvalidityandresponsivenessofthemrcpriondiseaseratingscale AT robertspulido internalconsistencyconstructvalidityandresponsivenessofthemrcpriondiseaseratingscale AT jessicaludwig internalconsistencyconstructvalidityandresponsivenessofthemrcpriondiseaseratingscale AT kathleenglisic internalconsistencyconstructvalidityandresponsivenessofthemrcpriondiseaseratingscale AT briansappleby internalconsistencyconstructvalidityandresponsivenessofthemrcpriondiseaseratingscale |