Distribution patterns of tau pathology in progressive supranuclear palsy
| Autor: | Gabor G. Kovacs, Edward B. Lee, Ellen Gelpi, Corey T. McMillan, Claire Troakes, David G. Coughlin, Sharon X. Xie, John Q. Trojanowski, Carolin Kurz, Günter U. Höglinger, Gesine Respondek, Armin Giese, Murray Grossman, David J. Irwin, Yaroslau Compta, Thomas Arzberger, Laura Donker Laat, Virginia M.-Y. Lee, John L. Robinson, Safa Al-Sarraj, Milica Ječmenica Lukić, John C. van Swieten, Sigrun Roeber |
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| Přispěvatelé: | Neurology, Clinical Genetics |
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Male
Stage Cerebellum Aging Striatum Neurodegenerative Alzheimer's Disease Neurofibrillary tangle Cohort Studies Tufted astrocyte pathology [Brain] Supranuclear Palsy Propagation Richardson syndrome Brain Middle Aged analysis [tau Proteins] ddc Subthalamic nucleus Tauopathy Frontotemporal Dementia (FTD) Globus pallidus medicine.anatomical_structure Neurological Female Supranuclear Palsy Progressive pathology [Supranuclear Palsy Progressive] 1.1 Normal biological development and functioning Clinical Sciences tau Proteins Biology Pathology and Forensic Medicine Progressive supranuclear palsy Cellular and Molecular Neuroscience Rare Diseases Progressive Underpinning research medicine Acquired Cognitive Impairment Humans ddc:610 Aged Original Paper Neurology & Neurosurgery Neurosciences Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD) Sequential involvement medicine.disease Coiled body Brain Disorders Dentate nucleus Dementia Neurology (clinical) Tau Neuroscience |
| Zdroj: | Acta neuropathologica, vol 140, iss 2 Acta Neuropathologica Acta neuropathologica 140(2), 99-119 (2020). doi:10.1007/s00401-020-02158-2 Kovacs, G G, Lukic, M J, Irwin, D J, Arzberger, T, Respondek, G, Lee, E B, Coughlin, D, Giese, A, Grossman, M, Kurz, C, McMillan, C T, Gelpi, E, Compta, Y, van Swieten, J C, Laat, L D, Troakes, C, Al-Sarraj, S, Robinson, J L, Roeber, S, Xie, S X, Lee, V M Y, Trojanowski, J Q & Höglinger, G U 2020, ' Distribution patterns of tau pathology in progressive supranuclear palsy ', Acta Neuropathologica, vol. 140, no. 2, pp. 99-119 . https://doi.org/10.1007/s00401-020-02158-2 Acta Neuropathologica, 140(2), 99-119. Springer-Verlag |
| ISSN: | 0001-6322 |
| DOI: | 10.1007/s00401-020-02158-2 |
| Popis: | Progressive supranuclear palsy (PSP) is a 4R-tauopathy predominated by subcortical pathology in neurons, astrocytes, and oligodendroglia associated with various clinical phenotypes. In the present international study, we addressed the question of whether or not sequential distribution patterns can be recognized for PSP pathology. We evaluated heat maps and distribution patterns of neuronal, astroglial, and oligodendroglial tau pathologies and their combinations in different clinical subtypes of PSP in postmortem brains. We used conditional probability and logistic regression to model the sequential distribution of tau pathologies across different brain regions. Tau pathology uniformly predominates in the neurons of the pallido-nigro-luysian axis in different clinical subtypes. However, clinical subtypes are distinguished not only by total tau load but rather cell-type (neuronal versus glial) specific vulnerability patterns of brain regions suggesting distinct dynamics or circuit-specific segregation of propagation of tau pathologies. For Richardson syndrome (n = 81) we recognize six sequential steps of involvement of brain regions by the combination of cellular tau pathologies. This is translated to six stages for the practical neuropathological diagnosis by the evaluation of the subthalamic nucleus, globus pallidus, striatum, cerebellum with dentate nucleus, and frontal and occipital cortices. This system can be applied to further clinical subtypes by emphasizing whether they show caudal (cerebellum/dentate nucleus) or rostral (cortical) predominant, or both types of pattern. Defining cell-specific stages of tau pathology helps to identify preclinical or early-stage cases for the better understanding of early pathogenic events, has implications for understanding the clinical subtype-specific dynamics of disease-propagation, and informs tau-neuroimaging on distribution patterns. Electronic supplementary material The online version of this article (10.1007/s00401-020-02158-2) contains supplementary material, which is available to authorized users. |
| Databáze: | OpenAIRE |
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