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      Cerebrospinal fluid real‐time quaking‐induced conversion is a robust and reliable test for sporadic creutzfeldt–jakob disease: An international study

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          Abstract

          Real‐time quaking‐induced conversion (RT‐QuIC) has been proposed as a sensitive diagnostic test for sporadic Creutzfeldt–Jakob disease; however, before this assay can be introduced into clinical practice, its reliability and reproducibility need to be demonstrated. Two international ring trials were undertaken in which a set of 25 cerebrospinal fluid samples were analyzed by a total of 11 different centers using a range of recombinant prion protein substrates and instrumentation. The results show almost complete concordance between the centers and demonstrate that RT‐QuIC is a suitably reliable and robust technique for clinical practice. Ann Neurol 2016;80:160–165

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          The role of cerebrospinal fluid 14-3-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review.

          It is 10 years since the detection of cerebrospinal fluid (CSF) 14-3-3 was included in the diagnostic criteria for sporadic Creutzfeldt-Jakob disease (sCJD) by the WHO. Since that time, other CSF proteins, such as S100b and tau protein, have been proposed as surrogate markers for sCJD. The authors aimed to investigate the diagnostic value of each of these three proteins. CSF samples collected from patients who were referred to the National CJD Surveillance Unit as suspected cases of sCJD during the period 1997-2007 were analysed for 14-3-3, S100b and tau protein. The sensitivity, specificity, positive predictive value and negative predictive value of each of these markers, either alone or in combination for the diagnosis of sCJD, were assessed. The impact of CSF 14-3-3 analysis on the case classification of sCJD was investigated. CSF 14-3-3 had the greatest sensitivity (86%) when compared with tau protein (81%) and S100b (65%). The combination of a positive CSF 14-3-3 or an elevated tau protein with a raised S100b had the highest positive predictive power for sCJD. During the study period, 100 patients were classified as probable sCJD solely on the basis of the clinical features and a positive CSF 14-3-3. The most sensitive marker for sCJD was a positive CSF 14-3-3. The analysis of CSF 14-3-3 plays a crucial role in the case classification of sCJD.
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            Variability in diagnosing Creutzfeldt-Jakob disease using standard and proposed diagnostic criteria.

            Creutzfeldt-Jakob disease (CJD) is a rapidly progressive dementia with a median survival of 2-14 months. The diagnosis can only be made accurately by biopsy/autopsy. However, this is not always feasible or desirable. Thus, diagnostic criteria have been proposed by UCSF, European MRI-CJD Consortium, and WHO. We will compare these criteria.
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              Author and article information

              Journal
              Ann Neurol
              Ann. Neurol
              10.1002/(ISSN)1531-8249
              ANA
              Annals of Neurology
              John Wiley and Sons Inc. (Hoboken )
              0364-5134
              1531-8249
              01 June 2016
              July 2016
              : 80
              : 1 ( doiID: 10.1002/ana.v80.1 )
              : 160-165
              Affiliations
              [ 1 ] National CJD Research & Surveillance Unit, Western General HospitalUniversity of Edinburgh Edinburgh ScotlandUnited Kingdom
              [ 2 ] Department of Neurological SciencesNational Institute of Health RomeItaly
              [ 3 ] Institute of Neurological SciencesScientific Institute for Research, Hospitalization and Health Care BolognaItaly
              [ 4 ] Department of Neurodegenerative DiseasesScientific Institute for Research, Hospitalization and Health Care, Carlo Besta Neurological Institute MilanItaly
              [ 5 ] Sorbonne UniversitiesPierre and Marie Curie University, Brain and Spine Institute Paris, France
              [ 6 ]National Reference Centre for Unconventional Transmissible Agents ParisFrance
              [ 7 ] Center for Neuropathology and Prion ResearchLudwig Maximilian University MunichGermany
              [ 8 ] Department of Neurology, Institute of Born BungeUniversity of Antwerp AntwerpBelgium
              [ 9 ] Florey Institute of Neuroscience and Mental HealthUniversity of Melbourne Melbourne VictoriaAustralia
              [ 10 ] Department of Molecular Microbiology and ImmunologyNagasaki University Graduate School of Biomedical Sciences NagasakiJapan
              [ 11 ]Prion Laboratory Section, Public Health Agency of Canada Winnipeg ManitobaCanada
              [ 12 ] Department of Neurology, University Medical Center and German Center for Neurodegenerative Diseases University of Göttingen GöttingenGermany
              [ 13 ] Department of MedicineUniversity of Melbourne Melbourne VictoriaAustralia
              [ 14 ]APHP, Pitié‐Salpêtrière Hospital ParisFrance
              [ 15 ]Department of Biomedical and Neuromotor Sciences, University of Bologna Italy
              Author notes
              [*] [* ]Address correspondence to Dr Green, University of Edinburgh, National CJD Research & Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU Scotland, United Kingdom. E‐mail: Alison.Green@ 123456ed.ac.uk
              Article
              ANA24679
              10.1002/ana.24679
              4982084
              27130376
              3f646688-8b25-402e-b736-1ab96b0c2984
              © 2016 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.

              This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

              History
              : 22 December 2015
              : 18 April 2016
              : 19 April 2016
              Page count
              Pages: 6
              Funding
              Funded by: Joint Programming for Neurodegenerative Disease
              Funded by: JPND
              Funded by: Chief Scientific Office, Scotland
              Funded by: Department of Health and the Scottish Home Office Department of Health
              Categories
              Brief Communication
              Brief Communications
              Custom metadata
              2.0
              ana24679
              July 2016
              Converter:WILEY_ML3GV2_TO_NLMPMC version:4.9.4 mode:remove_FC converted:12.08.2016

              Neurology
              Neurology

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