A second probable case of variant Creutzfeldt-Jakob (vCJD) disease in a
patient in the Netherlands was reported on 22 June 2006, by the National
Institute of Public Health and the Environment (RIVM) .
The case was detected through national surveillance of CJD, which is coordinated
at the Erasmus Medical Centre in Rotterdam. The probable diagnosis is based
on magnetic resonance imaging and, clinical signs. The patient has no mutations
associated with inherited CJD, and is homozygous for methionine at codon
129 of the prion protein gene. The diagnosis was reviewed by the United
Kingdom’s National Creutzfeldt-Jakob Disease Surveillance Unit, which runs
the European and Allied Countries Collaborative Study Group of CJD (EUROCJD,
Patient data was collected by the national public health service together
with the CJD surveillance unit, and revealed no specific exposure risks.
The patient has never donated or received blood or tissue and has not undergone
surgical procedures with risk of infection with prions. The patient has
never lived in or travelled to the United Kingdom. Contaminated meat products
remain the only plausible source of infection. Bovine spongiform encephalopathy
(BSE) has been diagnosed in 82 cows in the Netherlands since 1997, according
to data from the Ministry of Agriculture, Nature and Food Quality. 
This report brings the total number of vCJD cases reported to EUROCJD
from outside the UK by June 2006 to 31: 17 in France, 4 in the republic
of Ireland, 2 each in the United States and the Netherlands, and 1 in each
of Italy, Canada, Saudi Arabia, Japan, Portugal and Spain. Six of these
31 cases were in people who lived in the UK for more than 6 months between
1980 and 1996 .