Press Release WHO/22
13 February 1998
NEW DIAGNOSTIC CRITERIA RECOMMENDED FOR CREUTZFELDT-JAKOB DISEASE
Advances have been made in the field of diagnosing Creutzfeldt-Jakob disease (CJD) and these should now be used routinely in the diagnosis of the most common form of the disease ("Sporadic CJD"), 50 experts from around the world concluded at the end of a three-day consultation convened at World Health Organization (WHO) headquarters from 11 to 13 February.
The most important advance has been the identification of a novel spinal fluid test, the 14-3-3 assay, which appears to accurately detect Sporadic CJD. New information also suggests that magnetic brain scanning may be used to diagnose Sporadic CJD. However, neither of these investigations is known to be useful as pre-symptomatic screening tests, and no pre-symptomatic screening tests exist at present.
WHO is currently promoting the global surveillance of CJD in response to the occurrence of a new variant of CJD (nvCJD) in the UK. Unlike Sporadic CJD, whose cause is unknown, nvCJD is thought to be most likely due to the bovine spongiform encephalopathy agent. The experts reviewed the clinical and investigative features of the 24 cases of nvCJD so far identified and agreed upon criteria to identify a suspect nvCJD case.
There are four known forms of CJD and all of them are currently incurable. The meeting reviewed the latest ideas relating to potential therapeutics and noted that, at present, no CJD therapy was available. As the possibility of a significant epidemic of nvCJD occurring within the next 10 to 15 years cannot be dismissed, the Consultation noted that the early identification of an effective therapy is of paramount importance. Such a treatment would also offer hope to those individuals who are known to be at risk of developing CJD, such as people who received potentially infected human growth hormone or have a family history of one of the rare hereditary forms of CJD.
The Consultation stressed the pressing need for further research into the molecular properties of the CJD agent to help identify effective means of treating the disease. In parallel, efforts should be made to develop pre-symptomatic diagnostic tests to enable any future therapy to be used as early as possible in the disease course.
For further information please contact Gregory Hartl, Health Communications and Public Relations, WHO, Geneva. Telephone (+41 22) 791 4458. Fax (+41 22) 791 4858. Email firstname.lastname@example.org
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