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.