{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T03:26:18Z","timestamp":1781839578195,"version":"3.54.5"},"reference-count":51,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2015,4,7]],"date-time":"2015-04-07T00:00:00Z","timestamp":1428364800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Department of Health"},{"DOI":"10.13039\/501100012317","name":"UCLH\/UCL Biomedical Research Centre","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100012317","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Medical Research Council"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2015,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Prion diseases are dementing illnesses with poorly defined neuropsychological features. This is probably because the most common form, sporadic Creutzfeldt\u2010Jakob disease, is often rapidly progressive with pervasive cognitive decline making detailed neuropsychological investigation difficult. This study, which includes patients with inherited, acquired (iatrogenic and variant) and sporadic forms of the disease, is the only large\u2010scale neuropsychological investigation of this patient group ever undertaken and aimed to define a neuropsychological profile of human prion diseases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A tailored short cognitive examination of all of the patients (<jats:italic>n<\/jats:italic>\u00a0=\u00a081), with detailed neuropsychological testing in a subset with mild disease (<jats:italic>n<\/jats:italic>\u00a0=\u00a030) and correlation with demographic, clinical, genetic (<jats:italic><jats:styled-content style=\"fixed-case\">PRNP<\/jats:styled-content><\/jats:italic> mutation and polymorphic codon 129 genotype), and other variables (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> brain signal change in cortex, basal ganglia or thalamus; quantitative research imaging, cerebrospinal fluid 14\u20103\u20103 protein).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Comparison with healthy controls showed patients to be impaired on all tasks. Principal components analysis showed a major axis of fronto\u2010parietal dysfunction that accounted for approximately half of the variance observed. This correlated strongly with volume reduction in frontal and parietal gray matter on <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>. Examination of individual patients' performances confirmed early impairment on this axis, suggesting characteristic cognitive features in mild disease: prominent executive impairment, parietal dysfunction, a largely expressive dysphasia, with reduced motor speed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Taken together with typical neurological features, these results complete a profile that should improve differential diagnosis in a clinical setting. We propose a tailored neuropsychological battery for early recognition of clinical onset of symptoms with potential for use in clinical trials involving at\u2010risk individuals.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.195","type":"journal-article","created":{"date-parts":[[2015,4,7]],"date-time":"2015-04-07T15:25:14Z","timestamp":1428420314000},"page":"548-558","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["The cognitive profile of prion disease: a prospective clinical and imaging study"],"prefix":"10.1002","volume":"2","author":[{"given":"Diana","family":"Caine","sequence":"first","affiliation":[{"name":"NHS National Prion Clinic National Hospital for Neurology and Neurosurgery (NHNN) University College London Hospitals NHS Foundation Trust London United Kingdom"},{"name":"Department of Neuropsychology NHNN University College London Hospitals NHS Foundation Trust London United Kingdom"},{"name":"Department 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