Netherlands Finds Case of Variant Cruetzfeldt-Jakob Disease


By Peggy Peck, Senior Editor, MedPage Today
Reviewed by Zalman S. Agus, MD; Emeritus Professor at the University of Pennsylvania School of Medicine.
April 25, 2005
Also covered by: CNN, Newsday


MedPage Today Action Points

• A variant of Cruetzfeldt-Jakob Disease (CJD) is believed to be linked to consumption of meat from beef infected with bovine spongiform encephalitis (BSE). Explain to patients who are concerned about the risk of mad cow disease that thus far the U.S. beef supply has been protected.
• Explain that CJD is a rare, degenerative brain disease that usually affects people aged 60 or older. The disease is not spread by casual contact.

Review
UTRECHT, The Netherlands, April 25-The Dutch Health Ministry says a patient here has been diagnosed with the country's first known case of variant Cruetzfeldt-Jakob Disease (vCJD).



In an official announcement, the ministry said the case was reported by Rotterdam's Erasmus Medisch Centrum, the national surveillance center that monitors the disease. The Dutch government reported the case to other European nations. The Dutch National Health Inspectorate is "investigating whether the disease may have been transmitted to others."



Since the patient was "never a blood or tissue donor, and never received any blood transfusions or tissue transplants" the ministry said that it is "highly improbable that this patient infected others or contracted the disease from someone else."



Creutzfeldt-Jakob Disease (CJD), which was first described in 1920, is a rare, degenerative, fatal disease of the brain that is usually diagnosed in people age 60 or older. Approximately one case of CJD occurs per 1,000,000 population per year with a worldwide distribution. In recent years it has received more attention because of similarities to bovine spongiform encephalopathy (BSE), also known as mad cow disease.



There was an outbreak of BSE in cattle in the United Kingdom in the late 1980s, and eventually more than 200,000 cattle were infected. Almost immediately there was concern that humans eating infected beef might also be infected, and in 1990 a CJD surveillance program was established in Britain and was later extended to other European countries.

That surveillance initially identified 10 cases of vCJD, which, unlike traditional CJD, occurred in persons younger than 50. Since then the numbers have continued to increase, and a report last July by the FDA's Center for Food Safety and Nutrition stated that there were "155 confirmed and probable cases of vCJD worldwide" including one case in the U.S. -- a young woman who was infected while living in England.

Both BSE and CJD belong to a family of diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. Scrapie, which affects sheep and goats, and feline encephalopathy are other common TSEs.



According to the National Institutes of Neurological Disorders and Stroke (NINDS), non-variant CJD is divided into three major categories:

• Sporadic, the most common type of CJD, occurs in persons with no known risk factors. About 85% of CJD cases are classified as sporadic.
  
• Hereditary CJD accounts for 5% to 10% of cases in the U.S. Persons with hereditary CJD have a family history of disease or a genetic mutation associated with CJD.
  
• Acquired CJD, in which the disease is transmitted by exposure to infected brain or central nervous system tissue usually through medical procedures.

Persons with vCJD believed to be associated with mad cow disease usually present with psychiatric symptoms. The variant form typically has a longer time from symptom onset to death than standard CJD. Traditional CJD is characterized by rapidly progressive dementia as well as problems with muscle coordination, impaired vision and personality changes. There is no fever nor are there flu-like symptoms. As the disease progresses the patient's mental impairment becomes severe, often progressing to coma. Pneumonia is a frequent co-morbidity. Treatment for CJD is palliative.



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Primary source: National Institute of Neurological Disorders and Stroke
Source reference:

Creutzfeldt-Jakob Disease Fact Sheet

Additional source: Emerging Infectious Diseases
Source reference:

Brown P, Will RG, Bradley R et al. "Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease: Background, Evolution, and Current Concerns." Emer Infect Dis. 2001; 7: 6-16.

Additional source: U.S. Food and Drug Administration Center for Food Safety and Applied Nutrition

Source reference:
Commonly Asked Questions About BSE in Products Regulated by FDA's Center for Food Safety and Applied Nutrition (CFSAN).

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