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Clinical and pathologic characteristics distinguishing variant CJD from classic sporadic CJD[1]

Clinical and pathologic characteristics distinguishing variant CJD from classic sporadic CJD[1]
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
CJD: Creutzfeldt-Jakob disease; CSF: cerebrospinal fluid; RT-QuIC: real-time quaking-induced conversion; PrP: prion protein.
Reference:
  1. Belay E, Schonberger L. Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Clin Lab Med 2002; 22:849.
Adapted from: Clinical and Pathologic Characteristics Distinguishing Classic CJD from Variant CJD. Centers for Disease Control and Prevention. Available at: https://www.cdc.gov/prions/vcjd/clinical-pathologic-characteristics.html (Accessed on January 7, 2020).
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