Is an epidemic of CJD going undetected?

May 11, 2002 New Scientist by Debora MacKenzie
THE brain-wasting disease CJD may be much more common in young people than anyone had suspected.

Two young men from Michigan who died from the disease last autumn were only diagnosed because a specialist insisted on tests that wouldn't normally be done. He is now saying that when anyone, no matter how young, dies with suspicious neurological symptoms, there should be a post-mortem to check for CJD.

The men, aged 26 and 28, were brought to the University of Michigan hospital in Ann Arbor last summer with rapidly worsening neurological symptoms. One had severe epilepsy, the other had symptoms of dementia. Norman Foster, the attending neurologist and a CJD specialist, says neither man's symptoms fitted all the official criteria for the disease.

Normally they might have been diagnosed as suffering from brain inflammations of unknown cause, he says. "But we pushed to get a biopsy." It revealed that each had a different form of sporadic CJD.

The pattern of brain lesions and an analysis of the prion proteins that caused the disease showed it was not vCJD, the human form of BSE. Nor did it look like the chronic wasting disease of deer that occurs in neighbouring Wisconsin, which some fear might also be transmissible to humans.

Foster warns that if infection from deer led to an upsurge in CJD, it would go undetected because the disease is rarely tested for in young people. He thinks anyone who dies with dementia should be autopsied, and brain biopsies should be taken from young people who might have CJD, even if they do not have all the usual symptoms. He and other CJD specialists will meet in October to plan a surveillance programme for Michigan.

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