Table 1. Proposed University of California-San Francisco (UCSF) 2011 revised criteria*.

1. Supportive for cortical involvement: Asymmetric involvement of midline neocortex or cingulate or sparing of the precentral gyrus or ADC cortical ribboning with low values.

2. Supportive for subcortical involvement: Striatum with anterior-posterior gradient or subcortical low ADC values.

*Adapted from original table of proposed criteria [4].

DWI: Diffusion-weighted image; T2-FLAIR: T2-weighted fluid attenuation inversion recovery; MRI: Magnetic resonance imaging; CJD: Creutzfeldt-Jakob disease; ADC: Apparent diffusion coefficient.

Diagnosis	Criteria
MRI definitely CJD	DWI > T2-FLAIR hyperintensity in:
	Classic pathognomonic: cingulate, striatum, and >1 neocortical gyrus (often precuneus, angular, or superior/frontal gyrus)
	Cortex only involvement (>3 gyri); see supportive for cortical1
MRI probably CJD	Unilateral striatum or cortex (≤3 gyri); see supportive for cortical and subcortical1,2
	Bilateral striatum or posteromesial thalamus; see supportive for subcortical2
MRI probably not CJD	Only T2-FLAIR/DWI abnormalities in limbic areas, where hyperintensity can be normal (e.g., insula, anterior cingulate, hippocampi) and ADC map does not show restricted diffusion in these areas
	DWI hyperintensities due to artifact (signal distortion); see other MRI issues (below)
	T2-FLAIR > DWI hyperintensities; see other MRI issues (below)
MRI definitely not CJD	Normal imaging
	Abnormalities not consistent with CJD
Other MRI issues	In prolonged courses of CJD (>1 year) brain MRI might show significant atrophy with loss of DWI hyperintensity, particularly in areas previously with restricted diffusion
	To help distinguish abnormality from artifact, obtain sequences in multiple directions (e.g., axial and coronal)