Objectives In this prospective, longitudinal, multiparametric MRI study, we investigated clinical as well as brain grey matter and white matter (WM) regional changes in patients with progressive supranuclear palsy-Richardson’s syndrome (PSP-RS).
Methods Twenty-one patients with PSP-RS were evaluated at baseline relative to 36 healthy controls and after a mean follow-up of 1.4 years with clinical rating scales, neuropsychological tests and MRI scans.
Results Relative to controls, patients with PSP-RS showed at baseline a typical pattern of brain damage, including midbrain atrophy, frontal cortical thinning and widespread WM involvement of the main infratentorial and supratentorial tracts that exceeded cortical damage. Longitudinal study showed that PSP-RS exhibited no further changes in cortical thinning, which remained relatively focal, while midbrain atrophy and WM damage significantly progressed. Corpus callosum and frontal WM tract changes correlated with the progression of both disease severity and behavioural dysfunction.
Conclusions This study demonstrated the feasibility of carrying out longitudinal diffusion tensor MRI in patients with PSP-RS and its sensitivity to identifying the progression of pathology. Longitudinal midbrain volume loss and WM changes are associated with PSP disease course.
- progressive supranuclear palsy
- richardson syndrome
- magnetic resonance imaging (MRI)
- diffusion tensor MRI
- white matter
- cortical thickness
- midbrain volume
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Contributors FA: study concept/design, analysis/interpretation of data, drafting the manuscript for content. FC, PV, AM and MC: analysis/interpretation of data, revising the manuscript for content. MJ-L and INP: acquisition of data, analysis/interpretation of data, revising the manuscript for content. VSK: study concept/design, interpretation of data, revising the manuscript for content, obtaining funding. MF: study concept/design, interpretation of data, revising the manuscript for content, study supervision and coordination, obtaining funding.
Funding This study was partially supported by the Ministry of Education, Science and Technological Development of the Republic of Serbia (project no. 175090).
Competing interests FA is Section Editor of NeuroImage: Clinical; has received speaker honoraria from EXCEMED–Excellence in Medical Education and Biogen Idec; and receives research support from the Italian Ministry of Health, AriSLA (Fondazione Italiana di Ricerca per la SLA), and the European Research Council. FC, MJ-L, INP and AM report no disclosures. INP has received speaker honoraria from Boehringer Ingelheim, GSK, El Pharma, Roche and Actavis. PV has received speaker honoraria from EXCEMED–Excellence in Medical Education. MC has received compensation for consulting and/or serving on advisory boards from Teva Pharmaceuticals and Biogen Idec. VSK has received research grants from the Ministry of Education and Science, Republic of Serbia and the Serbian Academy of Science and Arts; and speaker honoraria from Novartis. MF is Editor-in-Chief of the Journal of Neurology ; serves on a scientific advisory board for Teva Pharmaceutical Industries; has received compensation for consulting services and/or speaking activities from Biogen Idec, EXCEMED, Merck Serono and Teva Pharmaceutical Industries; and has received research support from Biogen Idec, Merck Serono, Teva Pharmaceutical Industries, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla, Cure PSP, Alzheimer’s and Drug Discovery Foundation, and the Jacques and Gloria Gossweiler Foundation (Switzerland).
Ethics approval Local ethical standards committee on human experimentation (Faculty of Medicine, University of Belgrade) approved the study protocol.
Provenance and peer review Not commissioned; externally peer reviewed.
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