Harpreet Hyare1,2, Enrico De Vita3,4, Chris Carswell1,2, Andrew Thompson1,2, Ana Lukic1,2, Tarek Yousry3,4, Peter Rudge1,2, Simon Mead1,2, John Collinge1,2, John Thornton3,4
1MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology, London, United Kingdom; 2National Prion Clinic, National Hospital for Neurology & Neurosurgery, UCLH NHS Trust, London, United Kingdom; 3Lysholm Department of Neuroradiology, National Hospital for Neurology & Neurosurgery, London, United Kingdom; 4Academic Neuroradiological Unit, Department of Brain Repair & Rehabilitation, UCL Institute of Neurology, London, United Kingdom
We present the application of voxel-based analysis of diffusion tensor imaging (DTI) in the largest cohort of patients with a range of human prion diseases and compared our findings with voxel-based morphometry. DTI measures of tisssue integrity revealed diffuse white matter (WM) alterations, indepependent of atrophy. The distribution of these changes varied between the different forms of prion disease. Our findings suggest that loss of WM integrity occurs early in prion disease and the distribution of frontal involvement supports a role of compromised cortical circuitry in cognitive impairment of these patients.