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clinical and genetic features of human prion diseases in catalonia: 1993-2002.we describe the clinical and genetic characteristics of the 85 definite or probable human prion diseases cases died between january 1993 and december 2002 in catalonia (an autonomous community of spain, 6 million population). seventy-three (86%) cases were sporadic creutzfeld-jakob diseases (scjd) (49 definite, 24 probable), with a median age at onset of 66 years. the clinical presentation was dementia in 29 cases, ataxia in 14 and visual symptoms in five. the median survival was 3 months. the 1 ...200415469448
the impact of the introduction of the 14-3-3 protein assay in the surveillance of sporadic creutzfeldt-jakob disease in catalonia.the 14-3-3 assay in the csf has a high predictive value for the diagnosis of sporadic creutzfeldt-jakob disease (cjd). our objective was to analyse the trends in mortality and incidence rates of cjd in catalonia, an autonomous community of spain, setting the cut off point the date when the 14-3-3 assay was incorporated to our cjd surveillance system. only definite and probable cases according to the criteria adapted from masters and colleagues were included for the analysis. familial cases were ...200111518001
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