| Title: |
Development of prognostic models for survival and care status in sporadic Creutzfeldt-Jakob disease |
| Authors: |
Nihat, Akın; Ranson, Janice M; Harris, Dominique; McNiven, Kirsty; Mok, TzeHow; Rudge, Peter; Collinge, John; Llewellyn, David J; Mead, Simon |
| Contributors: |
Creutzfeldt-Jakob Disease Support Network; Alzheimer’s Research; UK; Alan Turing Institute/Engineering and Physical Sciences Research Council; National Institute for Health Research; Applied Research Collaboration; South-West Peninsula; National Health and Medical Research Council; National Institute on Aging; National Institutes of Health; Alan Turing Institute; Engineering and Physical Sciences Research Council; Medical Research Council; National Institute for Health Research Senior Investigators; Department of Health and Social Care; National Institute for Health Research’s Biomedical Research Centre; University College London Hospitals NHS Foundation Trust; Medical Research Council Clinical Research Training Fellowship; National Institute for Health Research’s Comprehensive Local Research Network |
| Source: |
Brain Communications ; volume 4, issue 4 ; ISSN 2632-1297 |
| Publisher Information: |
Oxford University Press (OUP) |
| Publication Year: |
2022 |
| Description: |
Sporadic Creutzfeldt-Jakob disease, the most common human prion disease, typically presents as a rapidly progressive dementia and has a highly variable prognosis. Despite this heterogeneity, clinicians need to give timely advice on likely prognosis and care needs. No prognostic models have been developed that predict survival or time to increased care status from the point of diagnosis. We aimed to develop clinically useful prognostic models with data from a large prospective observational cohort study. Five hundred and thirty-seven patients were visited by mobile teams of doctors and nurses from the National Health Service National Prion Clinic within 5 days of notification of a suspected diagnosis of sporadic Creutzfeldt-Jakob disease, enrolled to the study between October 2008 and March 2020, and followed up until November 2020. Prediction of survival over 10-, 30- and 100-day periods was the main outcome. Escalation of care status over the same time periods was a secondary outcome for a subsample of 113 patients with low care status at initial assessment. Two hundred and eighty (52.1%) patients were female and the median age was 67.2 (interquartile range 10.5) years. Median survival from initial assessment was 24 days (range 0–1633); 414 patients died within 100 days (77%). Ten variables were included in the final prediction models: sex; days since symptom onset; baseline care status; PRNP codon 129 genotype; Medical Research Council Prion Disease Rating Scale, Motor and Cognitive Examination Scales; count of MRI abnormalities; Mini-Mental State Examination score and categorical disease phenotype. The strongest predictor was PRNP codon 129 genotype (odds ratio 6.65 for methionine homozygous compared with methionine-valine heterozygous; 95% confidence interval 3.02–14.68 for 30-day mortality). Of 113 patients with lower care status at initial assessment, 88 (78%) had escalated care status within 100 days, with a median of 35 days. Area under the curve for models predicting outcomes within 10, 30 and ... |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1093/braincomms/fcac201 |
| DOI: |
10.1093/braincomms/fcac201/45271067/fcac201.pdf |
| Availability: |
https://doi.org/10.1093/braincomms/fcac201; https://academic.oup.com/braincomms/advance-article-pdf/doi/10.1093/braincomms/fcac201/45271067/fcac201.pdf; https://academic.oup.com/braincomms/article-pdf/4/4/fcac201/45407945/fcac201.pdf |
| Rights: |
https://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.AC70248B |
| Database: |
BASE |