Variant Creutzfeldt-Jakob disease (vCJD), originally known as new variant CJD (nvCJD), is a rare, degenerative, fatal brain disorder.1 vCJD has been linked to ingestion of beef contaminated with bovine spongiform encephalopathy (BSE), commonly referred to as "mad cow disease," and most cases have occurred in the United Kingdom.2 However, according to the Centers for Disease Control and Prevention (CDC), data from the United Kingdom indicating that relatively few of the presumably large number of persons exposed to BSE have developed vCJD suggests that the risk of contracting vCJD is low, even after consumption of contaminated product.3 No cases of vCJD have been connected to beef consumed in the United States.4

Because of the similarities in disease names, vCJD and classic CJD (any of the several categories of CJD that are not vCJD, including sporadic CJD, hereditary or familial CJD and iatrogenic CJD), often are confused. Classic CJD is discussed in further detail in the CJD section of the resource. Because of this confusion, throughout the resource, the term "classic CJD" or "CJD" with no qualifying adjective will be used to refer to any of the several categories of CJD other than vCJD.

vCJD begins primarily with psychiatric symptoms, affects younger patients than other types of CJD and has a longer duration than classic CJD from onset of symptoms to death.1

Table 1 illustrates some of the clinical and pathologic differences between classic CJD and vCJD.

(Table 1) Click here to print this table

Clinical and Pathologic Characteristics
Distinguishing Classic CJD from Variant CJD


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 (distortion of sense of touch); delayed neurologic signs

Periodic sharp waves on electroencephalogram

Often present

Reported rarely late in the disease

"Pulvinar sign" on MRI*

Reported rarely in iatrogenic cases

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


Source: Adapted from Belay E., Schonberger L. Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy. Clin Lab Med 2002;22:849-62.

*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.


Date:April 13, 2007
Content source: National Center for Infectious Diseases


Source: Centers for Disease Control, and personal correspondence with Lawrence Schonberger, M.D., M.P.H.

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