Enrico De Vita1,
2, Harpreet Hyare3, 4, Gerard R. Ridgway5,
Marie-Claire Porter3, 4, Andrew Thompson3,
4, Chris Carswell3, 4, Ana Lukic3,
4, Rolf H. Jager1, 2, Diana Caine3,
4, Peter Rudge3, 4, Tarek A. Yousry1,
2, John Collinge3, 4, Simon Mead3,
4, John Thornton1, 2
1Lysholm
Department of Neuroradiology, National Hospital for Neurology and
Neurosurgery, UCLH NHS Foundation Trust, London, United Kingdom; 2Academic
Neuroradiological Unit, Department of Brain Repair and Rehabilitation, UCL
Institute of Neurology, London, United Kingdom; 3MRC Prion Unit,
Department of Neurodegenerative Diseases, UCL Institute of Neurology, London,
United Kingdom; 4National Prion Clinic, National Hospital for
Neurology and Neurosurgery, UCLH NHS Foundation Trust, London, United
Kingdom; 5Wellcome Department of Cognitive Neurology, UCL
Institute of Neurology, London, United Kingdom
Human prion diseases (transmissible spongiform encephalopathies) are progressive and fatal neurodegenerative disorders. Voxel based morphometry (VBM) has only been used to study prion disease pathology in cross sectional studies. Only whole-brain ry measures have been employed to assess progressive degeneration longitudinally. Here we followed up a large numbr of prion disease patients with serial cerebral MRI and applied longitudinal VBM to characterize progressive structural change. We reveal significant regional changes in brain atrophy in prion patients consistent with known prion pathology and show correlation of rates of structural change with change in clinical assessment scores.