alert: mad cow disease d utrecht (ho ho ho)

A case of variant Creutzfeldt-Jakob Disease (vCJD), the human form of mad cow disease (BSE), has been confirmed in a patient in Utrecht, the Netherlands -- the first known case in the country -- according to the Ministry of Health, Welfare and Sport.

Rotterdam’s Erasmus Medisch Centrum, the national surveillance center that monitors the disease in the Netherlands, initially reported the case. The European surveillance center for vCJD in Edinburgh confirmed the diagnosis based on brain x-rays and the course of the disease.

The Dutch National Health Inspectorate has launched an investigation to determine whether the disease may have been transmitted to others. The Dutch government also has notified European authorities of the case.

Other Infections from This Case Improbable

VCJD is a variant of Creutzfeldt-Jakob Disease. Characterized by a spongy degeneration of the brain, the disease is caused by special protein structures called prions. It can be transmitted by tissue transplants and contaminated hospital instruments, especially during neurosurgical procedures.

Recently, speculation has arisen that vCJD may be transmitted by blood, based on two cases in the United Kingdom where this may have occurred. However, no conclusive scientific evidence for that hypothesis has been found to date.

The patient concerned in the Netherlands' case was never a blood or tissue donor, and never received any blood transfusions or tissue transplants. In light of that, it is highly improbable that this patient infected others or contracted the disease from someone else, the Ministry says.

Tainted Beef Likely Cause

In recent years, the Netherlands has introduced various measures to minimize the risk of transmission by blood:

# As of September 1, 2001: removal of white blood cells from all blood products (General Leukocyte Depletion).
# As of November 1, 2001: exclusion of donors who lived in the United Kingdom for over six months between 1980 and 1996.
# As of February 1, 2005: exclusion of blood donors who personally received a blood transfusion after January 1, 1980.

Investigations into the contraction of vCJD are focusing on the consumption of tainted beef as a cause. To date, it is unclear whether this particular case is attributable to contaminated beef, the Ministry says, and further investigation will be needed to determine whether the cause is traceable.

Safety Precautions Taken

The Netherlands ensures the safety of its beef by testing all vulnerable cattle for BSE, the Ministry of Health notes. In addition, the brains and spinal cords of cattle are separated and destroyed during the slaughtering process, as they could be infectious.

Other cases of vCJD have emerged in the past in European countries, starting in England. Ireland, France, Italy, Japan, Canada and the United States also have witnessed the occurrence of the disease, the Ministry notes.