Zerr I et al. 2009, Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease (Brain 132:2659-2668, PMID 19773352, doi 10.1093/brain/awp191)

A multicentre international study led from the National TSE Reference Center in Goettingen that tested whether brain MRI findings should be added to the clinical criteria for probable sporadic CJD. It reported that two MRI patterns were useful enough to count alongside EEG and CSF 14-3-3, and proposed adding them. The bibliographic details (journal, volume, pages, DOI, PMID) were confirmed in Europe PMC and Crossref. The full text is open access; the figures below come from reading it through an automated page summary, so they should be re-checked against the PDF before reuse. The CDC lists it among sources on its current diagnosis page, but later criteria that include RT-QuIC have moved beyond it. Information only, not clinical advice.

Key facts

  • Bibliographic record: 27 authors, first author I Zerr, published in Brain volume 132 issue 10, pages 2659-2668, DOI 10.1093/brain/awp191, PMID 19773352, PMCID PMC2759336.
  • Design: MRI scans from 436 sporadic CJD patients and a control group (141 in the abstract) from 12 countries; controls were people first suspected of sCJD and later given another diagnosis. Most sCJD cases were confirmed at autopsy, while only part of the controls were.
  • The best-performing MRI patterns were high signal on FLAIR or diffusion-weighted imaging in at least two cortical regions (temporal, parietal, occipital) or in both caudate nucleus and putamen.
  • MRI was positive in about 83 percent of cases, and the proposed amended criteria were positive in about 98 percent of definite cases.
  • Reported comparators: the existing criteria had sensitivity near 92 percent and specificity near 71 percent; CSF 14-3-3 about 86 and 68 percent; EEG about 44 percent sensitivity and 92 percent specificity.
  • Proposal: either MRI pattern should count as equivalent to a positive EEG or positive 14-3-3 when classifying probable sCJD. The authors present MRI as a complement to those tests, not a replacement.
  • Stated limits: more cases than controls, scans mostly on paper, moderate to high inter-rater agreement, controls mostly lacking autopsy, and false-positive MRI in 19 controls, mainly inflammatory or infectious conditions.
  • Funding was mainly the German federal research ministry and the Robert Koch Institute, plus national and EU programmes.
  • Crossref reports 738 citing works at the time of fetch (a moving number).
  • The CDC diagnosis page dated 21 January 2026 lists this paper among its sources. Its current probable-sCJD wording includes positive RT-QuIC, which this paper does not cover. The page does not state that its criteria derive from this paper.

Timeline

  1. 2009Article issued online (Crossref); print issue October 2009.
  2. 2009Published in Brain 132(Pt 10):2659-2668.
  3. 2026CDC clinical overview page that lists the paper as a source is dated.

People

Last checked 1 November 2026

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