TABLE 2

Criteria for probable diagnosis of sporadic CJD

  1. Neuropsychiatric disorder plus positive RT-QuIC in cerebrospinal fluid or other tissues

    OR

  2. All 3 of the following subcriteria:

    1. Rapidly progressive dementia and at least 2 of these 4 clinical features:

      • Myoclonus

      • Visual or cerebellar disturbances

      • Pyramidal or extrapyramidal dysfunction

      • Akinetic mutism

    2. A positive result on at least 1 of the following laboratory tests:

      • Typical electroencephalogram (periodic sharp-wave complexes) during an illness of any duration

      • Positive 14-3-3 protein cerebrospinal fluid assay in patient with a disease duration of less than 2 years

      • High signal in caudate and/or putamen on MRI, or in at least 2 cortical regions (temporal, parietal, occipital) on DWI or FLAIR

    3. No routine investigation indicates an alternative diagnosis

  • CJD = Creutzfeldt-Jakob disease DWI = diffusion-weighted imaging; FLAIR = fluid-attenuated inversion recovery; MRI = magnetic resonance imaging; RT-QulC = real-time quaking-induced conversion.

    From US Centers for Disease Control and Prevention, reference 41.