WHO 1998
1. Progressive dementia with any 2 of (myoclonus, pyramidal/EP, visual/cerebrellar/akinetic mutism)
AND
2, typical EEG and/or if 2 year duration + CSF 14,3,3
AND
3. Routine evaluation shows no other diagnosis
UCSF Modified
Rapid Cognitive Decline with any 2 of following: myoclonus, pyramidal/EP, visual, cerebellar, akinetic mutism, other focal cortical signs
AND
typical MRI and/or EEG
Comments of MRI on CJD: 91 % sensitive and 93-95 % specific. Findings include abnormal FLAIR and DWI in cortical gyri (ribboning) especially in CN, PUT and THAL. Only one form of CJD-- the MM2 subtype may not always show the DWI and FLAIR changes. vCJD shows "pulvinar" sign in thalamus.
Reviewing Geschwind's lecture and handout, the most useful table was the "lab finding not consistent with CJD". They include normal DWI and FLAIR, FLAIR without corresponding DWI abnormalities, abnormal contrast enhancement, leukoencephalopathy or significant white matter abnormality not attributable to another condition, mass effect or edema, CSF pleocytosis, CSF protein > 100, or elevated IgG index.
Clinical finding not typically found in CJD included early seizures, significant GI distress, ataxia without cognitive impairment, cranial neuropathy, acute stroke like onset weakness, and chorea. By contrast, aphasia, neglect and other "cortical" findings are common.
Body CT/PET can be considered for cases of possible paraneoplastic syndrome. Sarcoid also can be discovered.
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