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Brain Advance Access originally published online on September 10, 2004
Brain 2004 127(10):2348-2359; doi:10.1093/brain/awh249
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Brain Vol. 127 No. 10 © Guarantors of Brain 2004; all rights reserved

Predictors of survival in sporadic Creutzfeldt–Jakob disease and other human transmissible spongiform encephalopathies

M. Pocchiari1, M. Puopolo1, E. A. Croes2, H. Budka3, E. Gelpi3, S. Collins4, V. Lewis4, T. Sutcliffe5, A. Guilivi5, N. Delasnerie-Laupretre6, J.-P. Brandel6, A. Alperovitch6, I. Zerr7, S. Poser7, H. A. Kretzschmar8, A. Ladogana1, I. Rietvald2, E. Mitrova9, P. Martinez-Martin10, J. de Pedro-Cuesta10, M. Glatzel11, A. Aguzzi11, S. Cooper12, J. Mackenzie12, C. M. van Duijn2 and R. G. Will12

1 Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences Rome, Italy, 2 Department of Epidemiology and Biostatistics, Erasmus MC, Rotterdam, The Netherlands, 3 Austrian Reference Centre for Human Prion Diseases (OERPE) and Institute of Neurology Department of Cell Biology and Neuro Sciences, Vienna, Austria, 4 Department of Pathology, The University of Melbourne, Victoria, Australia, 5 Blood Safety Surveillance and Health Care Acquired Infections Division, Centre for Infectious Disease Prevention and Control, LCDC Building, Ontario, Canada, 6 U.360 INSERM, Hôpital de la Salpetriere, Paris, France, 7 Department of Neurology, Georg-August-Universität Göttingen, 8 Institute of Neuropathology, University of Munich, Munich, Germany, 9 Institute of Preventative and Clinical Medicine, National Reference Centre of Slow Virus Neuroinfections, Bratislava, Slovakia, 10 Instituto de Salud Carlos III, Centro Nacional de Epidemiologia, Departamento de Epidemiologia Aplicada, Madrid, Spain, 11 Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich, Zurich, Switzerland and 12 National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK

Correspondence to: Professor R. G. Will, National Creutzfeldt–Jakob disease Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU, UK E-mail: r.g.will{at}ed.ac.uk

A collaborative study of human transmissible spongiform encephalopathies has been carried out from 1993 to 2000 and includes data from 10 national registries, the majority in Western Europe. In this study, we present analyses of predictors of survival in sporadic (n = 2304), iatrogenic (n = 106) and variant Creutzfeldt–Jakob disease (n = 86) and in cases associated with mutations of the prion protein gene (n = 278), including Gerstmann–Sträussler–Scheinker syndrome (n = 24) and fatal familial insomnia (n = 41). Overall survival for each disease type was assessed by the Kaplan–Meier method and the multivariate analyses by the Cox proportional hazards model. In sporadic disease, longer survival was correlated with younger age at onset of illness, female gender, codon 129 heterozygosity, presence of CSF 14-3-3 protein and type 2a prion protein type. The ability to predict survival based on patient covariates is important for diagnosis and counselling, and the characterization of the survival distributions, in the absence of therapy, will be an important starting point for the assessment of potential therapeutic agents in the future.


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