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Abstract #0963

Improving strain diagnosis of prion disease by diffusion MRI and biophysical modelling

Marco Palombo1, Matteo Figini1, Riccardo Pascuzzo2, Paola Caroppo3, Mattia Verri2, Torben Schneider4, Claudia Angela Michela Gandini Wheeler-Kingshot5,6,7, Veronica Redaelli3, Daniel C Alexander1, Hui Zhang1, Giorgio Giaccone3, and Alberto Bizzi2

1Centre for Medical Image Computing, Department of Computer Science, University College London, London, United Kingdom, 2Neuroradiology, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milano, Italy, 3Neurology, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milano, Italy, 4Philips United Kingdom, Guildford, United Kingdom, 5Queen Square MS Centre, UCL Institute of Neurology, Faculty of Brain Sciences, University College London, London, United Kingdom, 6Department of Brain and Behavioural Sciences, University of Pavia, Pavia, Italy, 7Brain MRI 3T Research Center, IRCCS Mondino Foundation, Pavia, Italy

Sporadic Creutzfeldt–Jakob disease (sCJD) is the most common form of prion disease, characterized by five different strains, presenting intracellular vacuoles with different diameter/distribution. Unfortunately, no reliable non-invasive method for strain identification currently exists. Here we provide the first quantitative maps of MR-measured vacuolar diameter/density in five sCJD patients, using multishell diffusion MRI and biophysical modelling. Results show distribution of small and larger vacuoles in the brain lesions of each patient, presumably corresponding to different sCJD strains, and absence of vacuoles in five age-matched healthy controls. If validated, this method would be extremely valuable for non-invasive diagnosis of sCJD strain

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