지난 호주의 고스포드 병원(Gosford Hospital)에서 퇴행성 뇌질환으로 사망한 여성의 사망원인이 전형적 크로이츠펠츠 야콥병(classic-CJD)이나 변형 크로이츠펠트 야콥병(인간광우병)일 가능성이 제기되고 있다는 [데일리 텔레그라프]지의 보도입니다.

사망한 여성은 호주의 유명한 텔레비전 저널리스트인 마이크 윌리씨(Mike Willesee)의 이혼한 전처(前妻)인 캐롤 윌리씨(Carol Willesee)라고 합니다.

호주에서는 지난 40년간 395명의 전형적 크로이츠펠츠 야콥병(classic-CJD=sCJD, iCJD, fCJD) 환자가 확정진단을 받았다고 보고되어 있으며,  변형 크로이츠펠트 야콥병(vCJD, 인간광우병) 환자는 발생한 사실이 없습니다.

이번에 사망한 여성의 경우 1990년대 이후 외국 여행의 경험도 없기 때문에 만일 인간광우병(vCJD) 진단이 나올 경우, 광우병에 감염된 호주산 쇠고기를 통해 인간광우병에 걸린 것으로 볼 수 있기 때문에 ‘광우병 청정국가’인 호주의 입장에서는 엄청난 파문이 일 것으로 예상됩니다.

호주의 보건당국은 아직까지 검사결과에 대해 알 수 없는 상태라고 밝히고 있습니다만, 바짝 긴장하고 있는 것으로 추정됩니다. 

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Mad cow disease death scare after woman dies

【Died from CJD in 2006 … Carol Willesee. Source: The Daily Telegraph】

Health officials are testing her body for the illness that sparked a global scare in the 1990s.

However, experts believe it is more likely that she died from a degenerative form of Creutzfeldt-Jakob disease (CJD) because she had not travelled overseas in several years.

North Sydney Central Coast Area Health confirmed yesterday that the remains of the woman, who died at Gosford Hospital last week, had been sent to Sydney University to test for CJD. There are two main types of the untreatable disease – classic CJD and variant CJD, commonly referred to as the human equivalent of the mad cow disease.

Because the woman had not travelled overseas in recent years, health officials believe it is more likely she died from classic CJD.

Classic CJD is a rare degenerative disease triggered by mutations within a person’s brain and is not spread by people. It can be genetic and in most countries, including Australia, descendants of CJD victims are prohibited from donating blood.

Variant CJD emerged in the UK in the 1990s and is linked to the consumption of meat products from cattle infected with bovine spongiform encephalopathy (BSE), more commonly known as mad cow disease.

It is a separate disease even though many of the symptoms, including the early and rapid onset of dementia, blindness, loss of balance and muscle spasm, are similar.

According to the National CJD Registry in Melbourne, there have been 395 confirmed cases of classic CJD in the past 40 years. There have been no confirmed cases of variant CJD or human mad cow in Australia.

“Central Coast Health Service has confirmed that testing for Creutzfeldt-Jakob Disease is being conducted on a patient who recently died in Gosford Hospital,” an area health spokeswoman said.

“The results of the tests are not yet known.”

The results are expected to take some time because of the process involved with removing the brain and preparing it for various tests.

Carol Willesee, the ex-wife of television journalist Mike Willesee, died from classic CJD in 2006 after medical staff at Nepean Hospital failed to diagnose the condition.

In four months the accomplished stage actress went from a healthy, active woman to not being able to walk unassisted. She died aged 59.

Daughters Amy and Jo Willesee told a special commission of inquiry into acute care services in NSW they suspected she had the disease after researching her symptoms on the internet. But it was not until weeks after she was admitted that the doctors said they were investigating the possibility of CJD – despite medical notes later revealing it had been initially raised in emergency.