Neuropsychiatric disorder plus positive RT-QuIC in cerebrospinal fluid or other tissues OR All 3 of the following subcriteria:
Rapidly progressive dementia and at least 2 of these 4 clinical features:
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
No routine investigation indicates an alternative diagnosis
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