18FDG PET-CT in sporadic Creutzfeldt-Jakob disease, correlated with MRI and histology

Journal article

Sporadic Creutzfeldt-Jakob disease (sCJD) is the commonest human prion disease. sCJD is rapidly-progressive, universally fatal and transmissible. Rapid, accurate in-life diagnosis is imperative for epidemiological surveillance and public health activities, to exclude treatable differentials and facilitate supportive care. In 2017 the International CJD Support Network diagnostic criteria were revised to incorporate i) cortical ribboning on magnetic resonance imaging (MRI) and ii) the real-time quaking-induced conversion (RT-QuIC) assay. In this thesis the revised criteria were validated using a three-year clinicopathological cohort of all neuropathologically-confirmed sCJD cases from the UK. France, Germany and Italy, with a control group with alternative neuropathological diagnoses. The sensitivity and specificity of criteria was compared with prior criteria. Sub-analyses were performed assessing sCJD cases grouped by prion protein genotype, neuropathological classification, disease duration and age. The revised criteria were found to be 98.5% sensitive, a 21.5% increase from previous criteria, with no loss of specificity. Revisions have led to increases in case ascertainment, including among cases with limited clinical features and atypically long disease duration. This increase in sensitivity is of great utility for prion disease surveillance

Authors: Morley, N; Hofer, M; Wilkinson, P; Bradley, K

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: Thieme Gruppe
• Journal: World Journal of Nuclear Medicine
• Volume: 20
• Issue: 04
• Pages: 411-413
• Publication date: 2022-03-24
• DOI: 10.4103/wjnm.wjnm_5_21
• EISSN: 1607-3312
• ISSN: 1450-1147
• Language: English
• Keywords: Radiology; Medicine; Computed tomography; Stage (stratigraphy); Histology; Nuclear medicine; Positron emission tomography; Magnetic resonance imaging; Pathology