The Generative Brain

MRI and EEG

Brain MRI (cortical and basal ganglia diffusion signal) and EEG (periodic sharp wave complexes) are the widely available first-line tests in CJD criteria; the 2017 revision added cortical ribboning and RT-QuIC and raised criteria sensitivity to 98.5 percent without losing specificity.

Key facts

  • Indian case series: diffusion restriction with low ADC in the striatum in all sporadic CJD patients; periodic complexes in two thirds.
  • The US centre offers MRI interpretation at no cost.
  • Limits: Not specific alone; genetic forms vary.
  • What it asks of the person: Non-invasive.
  • CDC 2018 criteria list a typical EEG with periodic sharp wave complexes and MRI high signal in caudate/putamen or in at least two cortical regions (temporal, parietal, occipital) on DWI or FLAIR as clinical features supporting probable CJD.
  • The 2017 International CJD Surveillance Network criteria added cortical ribboning on MRI and RT-QuIC; validation found them 98.5 percent sensitive, a 21.5 percent increase over earlier criteria, with no loss of specificity.
  • A multinational validation of the revised criteria used 274 cases and 77 non-cases and found investigation sensitivity varied with genetic and pathological features, disease duration and age.
  • The 1998 WHO criteria included akinetic mutism, an end-stage sign, which is unhelpful for early diagnosis.
  • In Indian centres where RT-QuIC and CSF markers are unavailable, probable CJD series rely on MRI and EEG; one tertiary-centre series had no CSF 14-3-3 and no histology for any patient.

Timeline

  1. 2009European MRI-CJD consortium criteria.
  2. 2017International criteria revised.
  3. 2018CDC criteria published.
  4. 2022Multinational validation of the revised criteria published.
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