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A Dutch woman died at the age of 26 years, after a disease duration of 18 months, due to the new variant of Creutzfeldt-Jakob's disease (vCJD). She had never travelled to the United Kingdom and there was no history of potential iatrogenic exposure. However, she had worked in the catering and food production industry for the previous 6 years and had frequently consumed raw meat. The disease course showed the classical clinical picture of vCJD, which was confirmed by post-mortem examination of the brain. Contrary to classical sporadic CJD, patients with the variant disease are usually younger and present predominantly with psychiatric symptoms. Sensory complaints like pain and dysaesthesiae usually follow soon. Only later are these symptoms followed by rapidly progressive neurological symptoms and signs. All patients genotyped so far are homozygous for methionine on codon 129 of the prion protein gene. Recognition of the disease is of particular importance because of possible transmission via blood and tissues. In patients with rapidly progressive psychiatric symptoms and unexplained neurological signs, particularly sensory complaints, one must consider the possibility of vCJD.

Type

Journal article

Journal

Nederlands tijdschrift voor geneeskunde

Publication Date

12/2005

Volume

149

Pages

2949 - 2954

Addresses

Afd. Pathologie, Universitair Medisch Centrum Utrecht, Postbus 85.500, 3508 GA Utrecht. c.jansen@lab.azu.nl

Keywords

Brain, Humans, Creutzfeldt-Jakob Syndrome, Disease Progression, Diagnosis, Differential, Fatal Outcome, Adult, Netherlands, Female