Variant Creutzfeldt-Jakob Disease
all information

Variant Creutzfeldt-Jakob Disease

(Human Mad Cow Disease; vCJD)

En Español (Spanish Version)

Definition

Variant Creutzfeldt-Jakob disease (vCJD) is a type of prion disease. Bovine spongiform encephalopathy is a prion disease that affects cows; there is evidence that this illness can be transmitted to humans, producing vCJD. This illness is often called “mad cow disease.”

Causes

Prion diseases are a unique form of infectious diseases. The disease is not produced by a bacterial or viral infection; instead, the illness is related to progressive accumulation of prions (infectious protein particles). The central nervous system is progressively damaged as these prions accumulate.

The Nervous System

Nucleus factsheet image

© 2008 Nucleus Medical Art, Inc.

Risk Factors

Exposure to prion containing tissue is the primary risk factor. Other risk factors include:

  • Eating beef from infected cows
  • Receiving human growth hormone (HGH) injections prior to the mid-1980s—Although, changes in the preparation of HGH in the mid-1980s eliminated this risk.
  • If you are in the healthcare field, working with brain tissue
  • Receiving a corneal or dura mater (brain lining) transplantation

Five to ten percent of all cases of the nonvariant form of Creutzfeldt-Jakob are inherited.

Symptoms

It can take up to 20 years from the time of exposure to the infectious tissue until the onset of symptoms.

Early Phase (0 to 6 Months)

Psychiatric symptoms predominate:

Middle Phase

Neurologic symptoms predominate:

  • Abnormal gait
  • Ataxia (problems with coordination)
  • Involuntary movements (muscle jerks and stiffness)
  • Cognitive decline (impaired speech)

Late Phase

  • Mute
  • Immobility

The average duration from first symptoms to death is 13 months (with a range of 6 to 39 months).

Diagnosis

The clinical history and physical exam are the primary diagnostic tools. If your physician suspects vCJD, additional tests including lumbar puncture and CT or MRI of the brain. Electroencephalogram (EEG) may be necessary.

Biopsy of tonsil tissue has been used to confirm vCJD. In some cases, blood tests and cerebrospinal fluid analysis may be helpful in distinguishing this disease from other disease such as Alzheimer's disease . In many cases, final diagnosis requires autopsy and pathologic studies.

Treatment

Currently, there is no cure for vCJD. Treatment is primarily supportive, maximizing level of function and minimizing discomfort.

Prevention

About 150 worldwide cases of vCJD have occurred to date, nearly all associated with beef consumption in the United Kingdom. There is a great deal of controversy regarding safety of US beef. Two cases of bovine spongiform encephalopathy have been detected in the US. No cases of vCJD attributable to consumption of US beef have been detected.

To minimize risk, it is generally recommended that you avoid beef products, particularly processed meat (eg, sausage and hotdogs), or beef items containing brain, spinal cord, or bone marrow.

RESOURCES:

Creutzfeldt-Jakob Disease Foundation
http://www.cjdfoundation.org

World Health Organization
http://www.who.int

CANADIAN RESOURCES:

Canadian Health Coalition
http://www.healthcoalition.ca/index.html

Health Canada
http://www.hc-sc.gc.ca

References:

Churg-Strauss syndrome. EBSCO Publishing DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated December 12, 2007. Accessed February 23, 2008.

Dawidowska K. Where’s the (safe) beef? Prevention . 2004;56:34.

Moo-ve over, beef burgers: EN finds many alternatives. Environmental Nutrition . 2004 Aug;27:5.

NINDS Creutzfeldt-Jakob disease information page. National Institute of Neurological Disorders and Strokes website. Available at: http://www.ninds.nih.gov/disorders/cjd/cjd_pr.htm .

Prusiner SB. Detecting mad cow disease. Scientific American . 2004;291:60-67.

Raloff J. Better protection from mad cow disease. Science News . 2004;165:93.

Smith-Bathgate B. Creutzfeldt-Jakob disease: diagnosis and nursing care issues. Nursing Times . 2005;101:52.

Zeidler M, Sellar R, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet . 2000;355:1412-1419.



Last reviewed February 2008 by J. Thomas Megerian, MD, PhD, FAAP

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


Your Health and Happiness


DiggDeliciousNewsvineRedditStumbleTechnoratiFacebook