Table 2.

Sporadic Creutzfeldt-Jakob disease (sCJD) diagnostic criteria.

World Health organization (WHO) criteria, 1998 (3)	University of California, San Francisco (UCSF) criteria, 2007 (4)	European MRI-CJD Consortium criteria, 2009 (5)	National CJD research and surveillance unit (NCJDRSU) diagnostic criteria, 2017 (6)1
DEFINITE: -1.1 I + 2 of II and Neuropathologically or immunocytochemically or biochemically confirmed PROBABLE: - 1.2 I +2 of II and 1 of III POSSIBLE: -1.3 I + 2 of II + duration < 2 years	DEFINITE: - 1.1 I + 2 of II and Neuropathologically or immunocytochemically or biochemically confirmed PROBABLE: - I +2 of II and 1 of III POSSIBLE: - not defined in this criteria set	DEFINITE: - 1.1 I + 2 of II and Neuropathologically or immunocytochemically or biochemically confirmed PROBABLE: - I +2 of II and 1 of III POSSIBLE: - 1.3 I + 2 of II + duration < 2 years	DEFINITE: - 1.1 Progressive neurological syndrome AND Neuropathologically or immunocytochemically or biochemically confirmed PROBABLE: - 1.2.1 I + 2 of II and 1 of III (EEG *a)                     OR - 1.2.2 I + 2 of II and 1 of III (MRI*3)                     OR - 1.2.3 I + 2 of II and 1 of III (positive 14-3-3)                     OR - 1.2.4 Progressive neurological syndrome and positive RT-QuIC in CSF or other tissues POSSIBLE: - 1.3 I + 2 of II + duration < 2 years
I.Progressive dementia	I. Rapid cognitive decline	I. Progressive dementia*	I. Rapidly progressive cognitive impairment
II. Two among: - Myoclonus - Piramidal/extrapiramidal symptoms - Visual/cerebellar dysfunction - Akinetic mutism	II. Two among: - Myoclonus - Piramidal/extrapiramidal symptoms - Visual Impairment - Cerebellar dysfunction - Akinetic mutism - Focal cortical signs (e.g. neglect, aphasia, acalculia, apraxia	II. Two among: - Myoclonus - Piramidal/extrapiramidal symptoms - Visual/cerebellar dysfunction - Akinetic mutism	II. Two among: - Myoclonus - Visual or cerebellar problems - Piramidal or extrapiramidal features - Akinetic mutism
III. One among: - Typical EEG *a                     OR - Elevated CSF 14.3.3 protein (total disease duration < 2y)	III. One among: - Typical EEG *b AND/OR - Typical MRI *1	III. One among: - Typical EEG *a                     OR - Elevated CSF 14.3.3 protein (total disease duration < 2y)                     OR - Typical MRI*2	III. One among: - Typical EEG *a                     OR - Elevated CSF 14.3.3 protein                     OR - Typical MRI *3                     OR - positive real-time quaking-induced conversion in CSF or other tissues

^*aGeneralized periodic complexes.

^*bPeriodic latelalized epileptiform discharges (PLEDs).

^*1Subcortical or cortical gyral hyperintensity (cortical ribboning) on DWI and preferably restricted diffusion on ADC map.

^*2High-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).

^*3High signal in caudate/putamen on MRI brain scan or at least two cortical regions (temporal, parietal, occipital) either on DWI or FLAIR.

^*European MRI-CJD Consortium criteria (2009) clinical criteria are equal to those included in World Health organization criteria (1998). However, due to a typographical error in the Figure 1 of Zerr article (13), progressive dementia was not required in the clinical criteria and dementia was wrongly incorporated as a clinical symptom instead of myoclonus (1).