Related Subjects:
| Dementias
| Gerstmann-Straussler-Scheinker Syndrome (GSS)
| Fatal Familial Insomnia (FFI)
| Creutzfeldt Jakob disease (CJD)
| Variant Creutzfeldt Jakob disease (vCJD)
| Kuru
About
Types
Criteria
Clinical and Pathologic Characteristics Distinguishing Classic CJD from Variant CJD
Characteristic
Classic CJD
Variant CJD
Median age at death
68 years
28 years
Median duration of illness
4-5 months
13-14 months
Clinical signs and symptoms
Dementia; early neurologic signs
Prominent psychiatric/behavioral symptoms; painful dysesthesias; delayed neurologic signs
Periodic sharp waves on electroencephalogram
Often present
Often absent
“Pulvinar sign” on MRI*
Not reported
Present in >75% of cases
Presence of “florid plaques” on neuropathology
Rare or absent
Present in large numbers
Immunohistochemical analysis of brain tissue
Variable accumulation
Marked accumulation of protease-resistance prion protein
Presence of agent in lymphoid tissue
Not readily detected
Readily detected
Increased glycoform ratio on immunoblot analysis of protease-resistance prion protein
Not reported
Marked accumulation of protease-resistance prion protein
Clinical
Investigations
Management
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Creutzfeldt Jakob disease
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