Creutzfeldt-Jakob Disease (http://more.abcnews.go.com/sections/living/yourhealt

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CJD: A Disease in Disguise?

LIVING

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The human equivalent of mad-cow disease may be more common than we

think

Autopsies suggest misdiagnosies 914 kb (avi)

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Only 5% of CJD cases worldwide occur in people younger

than 45

Correspondent McKenzie with neuropathologist Dr.

idis (ABC News)

ABCNEWS.com

Creutzfeldt-Jakob Disease is fatal. It eats away at the brain,

leaving it riddled with holes like a sponge.

It’s difficult to diagnose and may be much more widespread than

previously believed. What is worse, it’s infectious.

Health officials maintain that only about 250 new cases of CJD

are diagnosed in the United States each year. But autopsies suggest that between

1 and 13 percent of cases of Alzheimer’s disease and other brain disorders may

be mistakenly diagnosed CJD.

Those preliminary findings suggest that a public-health problem

may be going overlooked, ABCNEWS' McKenzie reports.

“It’s a very good idea to take a closer look at Alzheimer’s

patients and other people who are written off as having typical senile dementia,

because there may be a hidden group [of people with CJD] in those patients,”

says Dr. idis, a neuropathologist at Yale Medical School.

It’s Contagious

If even 1 percent of Alzheimer’s patients had CJD, that would be

40,000 cases a year. And each undetected case is significant because, unlike

Alzheimer’s, CJD is infectious.

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Even with standard sterilization practices, this lethal disease can

be transmitted from patient to patient through neurosurgical instruments, as

well as through transplants of corneas and certain brain tissues. At least 100

people have been thus infected.

Surveillance

The Centers for Disease Control and Prevention are conducting

surveillance for CJD around the United States. And if there has been an increase

in the number of CJD cases, the next step is identifying the sources of

infection.

Deadly for Man and Beast

Cows from infected English herds were destoyed

(AP library)

CJD is in a class of diseases called TSEs. The National

Institutes of Health defines TSEs— transmissible spongiform encephalopathies—as

degenerative diseases characterized by acute progressive dementia and abnormal

limb movements.

Death usually occurs in four to six months after

infection.

Other TSEs include kuru, a condition limited to

New Guinea and spread by cannibalism, and scrapie, a disease that affects sheep.

But it was the so-called mad cow disease that

brought widespread attention to TSEs. A small number of cases, allegedly caused

by consumption of British beef contaminated with bovine spongiform

encephalopathy, caused widespread panic and an international ban on British

beef.

Heightened precautions around the world may help

reduce the number of cases of BSE contracted from eating tainted beef.

— Clare Weiss, ABCNEWS.com