Cited by 314Parkinson's Disease Mechanisms and TreatmentsDeep Brain Stimulation and Movement DisordersAlzheimer's disease research and treatments

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Progressive Supranuclear Palsy: Pathology and Genetics

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Dickson, Dennis W., Rademakers, Rosa, Hutton, Michael L.·2007·Brain Pathology

DOI 10.1111/j.1750-3639.2007.00054.xPMC PMC8095545

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Abstract

Progressive supranuclear palsy (PSP) is an atypical Parkinsonian disorder associated with progressive axial rigidity, vertical gaze palsy, dysarthria and dysphagia. Neuropathologically, the subthalamic nucleus and brainstem, especially the midbrain tectum and the superior cerebellar peduncle, show atrophy. The substantia nigra shows loss of pigment corresponding to nigrostriatal dopaminergic degeneration. Microscopic findings include neuronal loss, gliosis and neurofibrillary tangles in basal ganglia, diencephalon and brainstem. Characteristic tau pathology is also found in glia. The major genetic risk factor for sporadic PSP is a common variant in the gene encoding microtubule‐associated protein tau (MAPT) and recent studies have suggested that this may result in the altered expression of specific tau protein isoforms. Imaging studies suggest that there may be sensitive and specific means to differentiate PSP from other parkinsonian disorders, but identification of a diagnostic biomarker is still elusive.

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Authors: Dickson, D. W., Rademakers, R., & Hutton, M. L.
Affiliations: Mayo Clinic in Florida (US)
ORCID: Dickson, Dennis W.
Rademakers, Rosa
Venue: Brain Pathology
Publisher: Wiley
Published: 2007-01
Volume: 17
Issue: 1
Pages: 74-82
ISSN: 1015-6305 (ISSN-L)
1750-3639
DOI: 10.1111/j.1750-3639.2007.00054.x
PMC: PMC8095545
OpenAlex: W2014875179
Cited by: 314
References: 99
Citations by year: 2026: 20 · 2025: 24 · 2024: 25 · 2023: 25 · 2022: 15 · 2021: 17 · 2020: 11 · 2019: 18 · 2018: 7 · 2017: 25 · 2016: 19 · 2015: 11 · 2014: 17 · 2013: 14 · 2012: 10
FWCI: 1.58
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor, 56 d)
Locations: Brain Pathology (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: National Institute on Aging (R01AG015866, P01AG014449, P01AG003949, P50AG025711, P01AG017216, P50AG016574)
National Institute of Neurological Disorders and Stroke (P01NS040256, P50NS040256)
National Institute of Environmental Health Sciences (R01ES013941, R01-ES02941)
Topics: Health Sciences › Medicine › Clinical Neurology › Parkinson's Disease Mechanisms and Treatments
Health Sciences › Medicine › Clinical Neurology › Deep Brain Stimulation and Movement Disorders
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: progressive supranuclear palsy, tauopathy, tau pathology, MAPT gene, tau isoforms, atypical parkinsonism, subthalamic nucleus, midbrain tectum, superior cerebellar peduncle, nigrostriatal degeneration, neurofibrillary tangles, gliosis, brainstem atrophy, differential diagnosis, diagnostic biomarkers, neuroimaging
MeSH terms: Humans, Supranuclear Palsy, Progressive, tau Proteins

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Progressive Supranuclear Palsy: Pathology and Genetics

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