title: A hospital-based registry of Creutzfeldt-Jakob disease: Can neuroimaging serve as a surrogate biomarker?
abstract: OBJECTIVE
  This study addresses the role of neuroimaging in addition to the available clinical criteria for Creutzfeldt-Jakob disease (CJD) and its impact on its diagnosis in the absence of cerebrospinal fluid (CSF) biomarkers and tissue-based approaches.
  
  
  METHODS
  From a tertiary referral center in the city of Trivandrum, Kerala, South India, patients with rapidly progressive dementia (RPD) who fulfilled the World Health Organization (WHO) 1998 diagnostic criteria for CJD were included in this study. Their electrophysiological-clinical-radiological data were retrospectively studied and the results were analyzed. The other biomarkers of CJD were not assessed in the study.
  
  
  RESULTS
  Of the 96 patients with RPD, 41 patients were diagnosed as having a 'probable' and 'possible' CJD using the WHO 1998 diagnostic criteria between 2000 and 2013. While 92% patients satisfied the University of California, San Francisco (UCSF) 2007 and European magnetic resonance imaging (MRI)-CJD consortium criteria (2009), only 73% satisfied the MRI components of these criteria in addition to the more stringent, proposed UCSF MRI criteria (2011). The latter required the presence of diffusion weighted imaging abnormalities more than fluid attenuation inversion recovery abnormalities in the cortical and subcortical regions for the establishment of diagnosis on MRI of 'definite' (53.7%) and 'probable' CJD (19.5%).
  
  
  CONCLUSIONS
  Significant heterogeneity exists in the presentation of CJD with only 48.8% patients simultaneously satisfying the MRI and electrophysiological criteria, suggesting that the diagnosis is impacted by these components in any of the currently prevalent criteria. With 27% of the cohort not meeting the radiological criteria, CSF and molecular biomarker assays may be reserved for MRI negative patients with suspected CJD and in atypical presentations.
authors.forename: K P
authors.initials: KP
authors.lastname: Divya
authors.affiliation: Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
authors.forename: Ramshekhar N
authors.initials: RN
authors.lastname: Menon
authors.forename: Bejoy
authors.initials: B
authors.lastname: Thomas
authors.forename: Muralidharan
authors.initials: M
authors.lastname: Nair
mesh_terms: D015415:Biomarkers; D001921:Brain / Q000473:pathology; D015331:Cohort Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging*; D038524:Diffusion Magnetic Resonance Imaging; D006801:Humans; D007194:India; D008279:Magnetic Resonance Imaging; D059906:Neuroimaging; D012042:Registries; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers