Clinical and Pathologic Characteristics
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Variant CJD characteristics, as compared to classic CJD, are presented in the table below.CJD.
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 dyesthesiasis; 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 |
| Immunohitochemical 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 |
*An abnormal signal in the posterior thalami on T2- and diffusion-weighted images and fluid-attenuated inversion recovery sequences on brain magnetic resonance imaging (MRI); in the appropriate clinical context, this signal is highly specific for vCJD.
Source: Adapted from Belay E., Schonberger L. Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy. Clin Lab Me. 2002;22:849-862.
- Page last reviewed: February 10, 2015
- Page last updated: February 10, 2015
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