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Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative disorder belonging to the spongiform encephalopathies. A variant form (vCJD) is most likely the result of infection with the agent that causes bovine spongiform encephalopathy (BSE). Diagnostic information can be obtained by EEG, testing cerebrospinal fluid for the presence of the 14-3-3 protein, MRI, brain biopsy, tonsil biopsy, and postmortem brain examination. Some tests, such as MRI and postmortem brain examination, can be used to distinguish between CJD and vCJD. Pathological prions in a tonsil biopsy are only found with vCJD. In the Netherlands, there are four known cases of iatrogenic CJD. On the basis of certain exposure to BSE via the food chain, cases of vCJD are also to be expected. Chloropromazine and mepacrine are known to inhibit the formation of pathological prion conformations, but clinical trials have not yet been carried out.

Type

Journal article

Journal

Nederlands tijdschrift voor geneeskunde

Publication Date

04/2002

Volume

146

Pages

750 - 754

Addresses

Afd. Epidemiologie en Biostatistiek, Erasmus Universitair Medisch Centrum, Postbus 1738, 3000 DR Rotterdam.

Keywords

Animals, Cattle, Humans, Zoonoses, Creutzfeldt-Jakob Syndrome, Encephalopathy, Bovine Spongiform, Iatrogenic Disease, Incidence, Food Contamination, Netherlands