title: A study of clinical profile, radiological and electroencephalographic characteristics of suspected Creutzfeldt-Jakob disease in a tertiary care centre in South India.
abstract: BACKGROUND
  Creutzfeldt-Jakob disease (CJD) is a progressive, fatal, neurodegenerative disease classified under transmissible spongiform encephalopathies (TSE) or prion diseases. It is characterized by long asymptomatic period followed by rapid clinical deterioration leading to the death within months. The disease is still under-reported in India.
  
  
  OBJECTIVE
  The aim of this study was to describe the clinical, radiological and electroencephalographic characteristics of eight cases of CJD encountered in MS Ramaiah Medical college and Hospital, Bangalore over the past 3 years (2010-2013). This was retrospective, observational, hospital-based study.
  
  
  RESULTS
  The mean age of patients was 66.6 years (range: 54-82) and there was female predominance (five patients). The main clinical manifestations were cognitive disturbance (8/8) and myoclonus (8/8), followed by behavioral disturbance (5/8), ataxia (5/8) and extra-pyramidal symptoms/signs (4/8). Time interval (mean) between onset of disease to death was 6.6 months (range: 3-14). Brain MRI abnormalities were noted in 6 patients: Fluid-attenuated inversion recovery hyperintensities with restriction on diffusion-weighted image/apparent diffusion coefficient (DWI/ADC) in caudate and putamen, and diffusion hyperintensities without restriction on ADC in parieto-occipital, frontal and temporal regions. Classical electroencephalogram (EEG) changes of periodic triphasic waves were seen in 87% of patients. The CSF 14-3-3 protein assay was positive in two patients (out of four). Seven cases were probable CJD and one was possible CJD.
  
  
  CONCLUSIONS
  A strong clinical suspicion aided by characteristic brain MRI and EEG abnormalities is essential for timely diagnosis of this fatal disease.
authors.affiliation: Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India.
authors.forename: Rohan R
authors.initials: RR
authors.lastname: Mahale
authors.affiliation: Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India.
authors.forename: Mahendra
authors.initials: M
authors.lastname: Javali
authors.affiliation: Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India.
authors.forename: Anish
authors.initials: A
authors.lastname: Mehta
authors.affiliation: Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India.
authors.forename: Suryanarayana
authors.initials: S
authors.lastname: Sharma
authors.affiliation: Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India.
authors.forename: Purushottam
authors.initials: P
authors.lastname: Acharya
authors.affiliation: Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India.
authors.forename: Rangasetty
authors.initials: R
authors.lastname: Srinivasa
keywords: 14-3-3 protein; Clinical manifestation; Creutzfeldt-Jakob disease; diagnosis; prion diseases; prion protein