ArticleJournal of neurology, neurosurgery, and psychiatry2022
Development of novel clinical examination scales for the measurement of disease severity in Creutzfeldt-Jakob disease.
Article in Journal of neurology, neurosurgery, and psychiatry, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 9 citations in OpenAlex.
- Clinical phenotype associated with A118V mutation of PRPN gene.Journal of neurology · 2025Article
- PRNP E146G mutation inherited prion disease: distinctive clinical, pathological and fluid biomarker features.Journal of neurology · 2025Article
- Article
- Prevalence and Treatments of Movement Disorders in Prion Diseases: A Longitudinal Cohort Study.Movement disorders : official journal of the Movement Disorder Society · 2022Article
- Development of prognostic models for survival and care status in sporadic Creutzfeldt-Jakob disease.Brain communications · 2022Article
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
Abstract
objectiveTo use a robust statistical methodology to develop and validate clinical rating scales quantifying longitudinal motor and cognitive dysfunction in sporadic Creutzfeldt-Jakob disease (sCJD) at the bedside.
methodsRasch analysis was used to iteratively construct interval scales measuring composite cognitive and motor dysfunction from pooled bedside neurocognitive examinations collected as part of the prospective National Prion Monitoring Cohort study, October 2008-December 2016.A longitudinal clinical examination dataset constructed from 528 patients with sCJD, comprising 1030 Motor Scale and 757 Cognitive Scale scores over 130 patient-years of study, was used to demonstrate scale utility.
resultsThe Rasch-derived Motor Scale consists of 8 items, including assessments reliant on pyramidal, extrapyramidal and cerebellar systems. The Cognitive Scale comprises 6 items, and includes measures of executive function, language, visual perception and memory. Both scales are unidimensional, perform independently of age or gender and have excellent inter-rater reliability. They can be completed in minutes at the bedside, as part of a normal neurocognitive examination. A composite Examination Scale can be derived by averaging both scores. Several scale uses, in measuring longitudinal change, prognosis and phenotypic heterogeneity are illustrated.
conclusionsThese two novel sCJD Motor and Cognitive Scales and the composite Examination Scale should prove useful to objectively measure phenotypic and clinical change in future clinical trials and for patient stratification. This statistical approach can help to overcome obstacles to assessing clinical change in rapidly progressive, multisystem conditions with limited longitudinal follow-up.
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