First case of human mad cow disease in the Netherlands

Main Category: CJD / vCJD / Mad Cow Disease
Article Date: 22 Apr 2005 - 11:00 PDT

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Authorities in the Netherlands have confirmed they have a patient in hospital with vCJD (the human form of Mad Cow Disease).

Authorities are trying to find out whether anybody else is also infected. Possible routes of contamination may be through donating blood or an organ. According to authorities, the patient is neither a blood nor an organ donor. Therefore, the chances of someone else being infected (from this patient) is remote.

What is Creutzfeldt-Jakob Disease (CJD)?

Creutzfeldt-Jakob Disease (CJD) is one of a small group of fatal diseases caused by infectious agents called prions. These attack the brain, killing cells and creating gaps in tissue. The disease is always fatal.

There are two types of CJD: classical and variant.

What are prions (in relation to variant CJD)?

The agent that causes variant CJD is believed to be an abnormal form of a protein, known as a prion. Prions are normal cellular proteins that are present in many organs and tissues, including the brain, spinal cord and eyes of healthy humans and animals. The abnormal form of prion causes surrounding proteins to change their shape. The abnormal protein collects in central nervous tissue and by an unknown mechanism causes the nerve cells to die. This results in the characteristic holes in the brain tissue that can be seen under the microscope.

How do people get variant CJD?

Scientific evidence has linked variant CJD to eating contaminated beef products from animals infected with bovine spongiform encephalopathy (BSE) or "mad-cow disease." Processed meat products made from cows infected with BSE carry a high risk for transmitting the disease.

What are the symptoms of variant CJD?

Variant CJD symptoms include early psychiatric symptoms such as anxiety, depression, withdrawal and behavioural changes. Persistent pain or odd sensations in the face or limbs often develop. The disease then progresses to motor difficulties, involuntary movements and mental deterioration, often ending in a persistent vegetative state. The patient may live on average for about one year after the onset of symptoms.

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