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. Author manuscript; available in PMC: 2016 May 25.
Published in final edited form as: Continuum (Minneap Minn). 2015 Dec;21(6 NEUROINFECTIOUS DISEASE):1612–1638. doi: 10.1212/CON.0000000000000251

TABLE 4-3.

Commonly Used Diagnostic Criteria for Sporadic Jakob-Creutzfeldt Disease

  • World Health Organization Criteria (WHO) (1998)a

    1. Progressive dementia

    2. Two of the following four signs/symptoms:

      • Myoclonus

      • Pyramidal/extrapyramidal symptoms

      • Visual/cerebellar dysfunction

      • Akinetic mutism

    3. Typical EEG or elevated CSF protein 14-3-3 with total disease duration <2 years

    4. Routine investigations should not suggest an alternative diagnosis

  • University of California, San Francisco Criteria (UCSF) (2007)b

    1. Rapid cognitive decline

    2. Two of the following six signs/symptoms:

      • Myoclonus

      • Pyramidal/extrapyramidal dysfunction

      • Visual dysfunction

      • Cerebellar dysfunction

      • Akinetic mutism

      • Focal cortical signs (eg, neglect, aphasia, acalculia, apraxia)

    3. Typical EEG and/or MRIb

    4. Other investigations should not suggest an alternative diagnosis

  • European MRI-CJD Consortium Criteria (2009)c

    1. Progressive dementia

    2. One of the following signs/symptoms:

      • Myoclonus

      • Pyramidal/extrapyramidal symptoms

      • Visual/cerebellar dysfunction

      • Akinetic mutism

      • AND

    3. Either

      • Typical EEG

      • Elevated CSF protein 14-3-3 (with total disease duration <2 years)

      • OR

      • Typical MRId

    4. Routine investigations should not suggest an alternative diagnosis

CJD = Creutzfeldt-Jakob disease; CSF = cerebrospinal fluid; EEG = electroencephalography; MRI = magnetic resonance imaging.

a

WHO revised criteria allow either a positive EEG or a positive CSF 14-3-3 protein provided the disease duration to death is <2 years.15,16,27

b

See Table 1 in Vitali P, et al, Neurology.16 Briefly, UCSF MRI criteria require diffusion-weighted imaging (DWI) brighter than fluid-attenuated inversion recovery (FLAIR) hyperintensity in the cingulate, striatum and/or greater than one neocortical gyrus, ideally with sparing of the precentral gyrus and apparent diffusion coefficient map supporting restricted diffusion.

c

Of note, there were typographical errors in the symptom criteria in Zerr 2009 article Figure 1; progressive dementia was not required and dementia was substituted for myoclonus as one of four possible clinical symptoms in the criteria. In fact, European MRI-CJD Consortium clinical symptom criteria are unchanged from WHO 1998 criteria.15,27

d

High-signal intensity on either FLAIR or DWI in both the putamen and the caudate nucleus or in at least two cerebral cortical regions (from either the temporal, occipital, or parietal cortices, not including frontal or limbic regions).