Histologic tau lesions and magnetic resonance imaging biomarkers differ across two progressive supranuclear palsy variants.

Progressive supranuclear palsy is a neurodegenerative disease characterized by the deposition of four-repeat tau in neuronal and glial lesions in the brainstem, cerebellar, subcortical and cortical brain regions. There are varying clinical presentations of progressive supranuclear palsy with different neuroimaging signatures, presumed to be due to different topographical distributions and burden of tau. The classic Richardson syndrome presentation is considered a subcortical variant, whilst progressive supranuclear palsy with predominant speech and language impairment is considered a cortical variant, although the pathological underpinnings of these variants are unclear. In this case-control study, we aimed to determine whether patterns of regional tau pathology differed between these variants and whether tau burden correlated with neuroimaging. Thirty-three neuropathologically confirmed progressive supranuclear palsy patients with either the Richardson syndrome (

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© The Author(s) 2024. Published by Oxford University Press on behalf of the Guarantors of Brain.

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F.O., A.F.C., H.M.C., J.R.D., Y.C.S. and M.L.S. have nothing to disclose. J.L.W., K.A.J., C.R.J., F.Ali., H.B., M.M.M., C.G.S., D.W.D. and R.L.U. receive support from the US NIH. K.A.J. is an Associate Editor of Annals of Clinical and Translation Neurology. F.Agosta. is an Associate Editor of NeuroImage: Clinical, has received speaker honoraria from Biogen Idec and Roche and receives or has received research supports from the Italian Ministry of Health, AriSLA (Fondazione Italiana di Ricerca per la SLA), the European Research Council and Foundation Research on Alzheimer Disease. M.F. is Editor-in-Chief of the Journal of Neurology and Associate Editor of Human Brain Mapping, received compensation for consulting services and/or speaking activities from Almiral, Alexion, Bayer, Biogen, Celgene, Eli Lilly, Genzyme, Merck Serono, Novartis, Roche, Sanofi, Takeda and Teva Pharmaceutical Industries and receives research support from Biogen Idec, Merck Serono, Novartis, Roche, Teva Pharmaceutical Industries, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla and ARiSLA (Fondazione Italiana di Ricerca per la SLA).