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Rule out of variant CJD as the cause of death of a Virginia resident
NEW: June 12, 2008
Recently, the National Prion Disease Pathology Surveillance Center (NPDPSC) ruled out the presence of variant CJD (vCJD) as the cause of death of a young Virginia woman who died earlier this year.
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About vCJD

Variant CJD was first described in 1996 in the United Kingdom. It has different clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile of the prion protein gene. (See table below). The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD. The median duration of illness for vCJD is 14 months, compared to 5 months for classic 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
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.

Evidence for Relationship with BSE (Mad Cow Disease)

Percent distribution of non-iatrogenic UK vCJD and US CJD deaths, by age group, 1995-2003. Larger Picture
(Courtesy Ermias Belay)
Since 1996, evidence has been increasing for a causal relationship between ongoing outbreaks in Europe of a disease in cattle, called bovine spongiform encephalopathy (BSE, or 'mad cow' disease), and vCJD. There is now strong scientific evidence that the agent responsible for the outbreak of prion disease in cows, BSE, is the same agent responsible for the outbreak of vCJD in humans. Both disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent. However, this evidence also suggests that the risk is low for having vCJD, even after consumption of contaminated product. In 1996, because of the emergence of vCJD in the United Kingdom, CDC enhanced its surveillance for CJD in the United States.

'See also' link icon For more information about BSE and its occurrence in the United States and Canada, also see Bovine Spongiform Encephalopathy

Reference in this website to any specific commercial products, process, service, manufacturer, or company does not constitute its endorsement or recommendation by the U.S. Government or CDC. CDC is not responsible for the contents of any "off-site" web page referenced from this server.

Date:January 4, 2007
Content source: National Center for Infectious Diseases
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Related Topics

Prion Diseases

Bovine spongiform encephalopathy

Chronic Wasting Disease (CWD)

Creutzfeldt-Jakob Disease (CJD)

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