Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 3248  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 »   Next article
 »   Previous article
 »   Table of Contents

 Resource Links
 »   Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
 »   Citation Manager
 »   Access Statistics
 »   Reader Comments
 »   Email Alert *
 »   Add to My List *
 * Requires registration (Free)

 Article Access Statistics
    PDF Downloaded100    
    Comments [Add]    
    Cited by others 2    

Recommend this journal


Year : 2016  |  Volume : 64  |  Issue : 3  |  Page : 411--418

A hospital-based registry of Creutzfeldt–Jakob disease: Can neuroimaging serve as a surrogate biomarker?

1 Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
2 Department of Neuroimaging and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Correspondence Address:
Dr. Ramshekhar N Menon
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011, Kerala
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.181538

Rights and Permissions

Aim: 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.


Print this article     Email this article

Online since 20th March '04
Published by Wolters Kluwer - Medknow