Ireland first case of vCJD, Human form of Mad Cow Disease, Confirmed

Main Category: CJD / vCJD / Mad Cow Disease
Article Date: 10 Nov 2004 - 11:00 PDT


Irish authorities have confirmed their first case of vCJD, the human form of mad cow disease. The patient, a man in his early twenties, is seriously ill in hospital in Dublin.

A spokesman said, "All of the necessary precautions are being taken and the relevant authorities have been updated. In the public interest, the hospital reiterates that the patient never received a blood transfusion or made a blood donation, and that the cause of infection is not linked to an operation."

This vCJD case did not originate from Britain, say authorities, because the man has never been to Britain. The variant CJD originated in the Republic of Ireland.

The only other case of vCJD in Ireland was a woman who had lived in England for a long time.

Doctors took a biopsy from the man's tonsils to make their diagnosis, said a hospital spokesman.

The Spokesman added "In the interests of patient confidentiality, further information on the patient's condition will not be issued and we ask that the privacy of the patient and family be respected during this difficult time."

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.

How is variant CJD diagnosed?

The diagnosis of variant CJD is very difficult, but brain scans, using magnetic resonance imaging (MRI), and tonsil biopsy are two types of tests used to establish a probable diagnosis. However, the final confirmation of variant CJD can only be done by examining brain tissue, generally after the patient has died.

What treatment is available for a person who has variant CJD?

At present, there is no cure or treatment to slow the progression of the disease.

Is there a test to screen for variant CJD?

No screening test is available for persons who may have been exposed to "mad-cow disease," hence there is no known method of detecting variant CJD before symptoms appear.

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