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-Kingshot^5,6,7, 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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