Characteristic | Variant CJD | Classic CJD |
Median age at onset | 29 years | 65 years |
Median duration of illness | 13 to 14 months | 4 to 6 months |
Clinical signs and symptoms | Early onset of prominent psychiatric/behavioral symptoms and painful sensory symptoms; other neurologic signs develop later | Presents with dementia; early neurologic signs |
Periodic sharp waves on electroencephalogram | Often absent | Often present |
Pulvinar sign on MRI | Present in >75% of cases | Not reported |
CSF RT-QuIC | Typically negative | Positive in >90% of cases |
Posterior thalamic neuronal loss | Severe | Minimal |
Florid plaques on neuropathology | Present in large numbers | Absent |
PrP immunohistochemistry | Widespread cluster plaques | Cluster plaques not identified |
Presence of agent in lymphoid tissue | Readily detected | Not readily detected |
Immunoblot analysis of protease-resistant prion protein | Overrepresentation of diglycosylated protein fragments | Predominance of monoglycosylated protein fragments |
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