Clinical and Pathologic Characteristics
Variant CJD characteristics, as compared to classic CJD, are presented in the table below.
|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.