Centro Nacional de Epidemiología, Instituto de
Salud Carlos III, Madrid, Spain (http://cne.isciii.es/)
The Spanish Ministry of Health has reported the detection
of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain .
The patient was a 26 year old woman in Madrid who died on 10 July 2005.
She had no specific risk factors for vCJD (no prior blood transfusions or
visits to the United Kingdom).
The patient’s symptoms began in November 2004, with rapid progression to
dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal
fluid. Other neurological manifestations including ataxia, dysarthria, apraxia
and myoclonus appeared in early 2005. The first abnormal MRI was observed
in April 2005. The patient was methionine homozygous at codon 129 of the
prion protein gene (PRPN). There were no identified PRPN mutations or family
history of CJD.
The case was notified to the Spanish CJD state registry in May 2005 and
initially fitted the criteria for both probable sporadic CJD (sCJD) and
possible vCJD; it was registered as probable sCJD. Brain neuropathology
results of tests conducted at Fundación Hospital de Alcorcón
at Madrid subsequently confirmed vCJD. Materials have been sent to the European
reference centre at the United Kingdom’s National CJD Surveillance Unit
in Edinburgh for further study, and results are expected later in August.