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Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches
Open accessAbstract
Progressive supranuclear palsy (PSP), previously believed to be a common cause of atypical parkinsonism, is now recognised as a range of motor and behavioural syndromes that are associated with a characteristic 4-repeat tau neuropathology. New research criteria that recognise early presentations of PSP and operationalise diagnosis of the full spectrum of clinical phenotypes have been reported. The Movement Disorders Society PSP diagnostic criteria include syndromes with few or mild symptoms that are suggestive of underlying PSP pathology and could provide an opportunity for earlier therapeutic interventions in the future. These criteria also include definitions for variant PSP syndromes with different patterns of movement, language, or behavioural features than have been conclusively associated with PSP pathology. Data from new diagnostic biomarkers can be combined with the clinical features of disease to increase the specificity of the new criteria for underlying PSP pathology. Because PSP is associated with tau protein abnormalities, there is growing interest in clinical trials of new tau-directed therapies. These therapies are hypothesised to have disease-modifying effects by reducing the concentration of toxic forms of tau in the brain or by compensating for loss of tau function. Since tau pathology is also central to Alzheimer's disease and chronic traumatic encephalopathy, a successful tau therapeutic for PSP might inform treatment of other neurodegenerative diseases.
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“Although the presence of an overt frontotemporal dementia syndrome can be defined on the basis of established criteria, there are no such criteria for the prodromal forms of fronto temporal lobar degeneration, except for progressive supranuclear See Online for appendix Review palsy.31”
From Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations (2022) · cites this paper as [31]
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“Neuropathologically, PSP is defined by the accumulation of pTau in both neurons and glial cells.369”
From Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets (2023) · cites this paper as [369]
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Self-citation
Metadata
- Authors
- Boxer, A. L., Yu, J.-T., Golbe, L. I., Litvan, I., Lang, A. E., & Höglinger, G. U.
- Affiliations
- University of California, San Francisco (US)
- Qingdao University (CN)
- Rutgers, The State University of New Jersey (US)
- University of California San Diego (US)
- University of Toronto (CA)
- German Center for Neurodegenerative Diseases (DE)
- Corresponding author
- Boxer, Adam L
- Venue
- The Lancet Neurology
- Publisher
- Elsevier BV
- Published
- 2017-07
- Volume
- 16
- Issue
- 7
- Pages
- 552-563
- ISSN
- 1474-4422 (ISSN-L)
- PMC
- PMC5802400
- OpenAlex
- W2625168757
- Cited by
- 485
- References
- 105
- Citations by year
- 2026: 45 · 2025: 52 · 2024: 59 · 2023: 42 · 2022: 57 · 2021: 56 · 2020: 62 · 2019: 61 · 2018: 43 · 2017: 7
- FWCI
- 6.28
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- The Lancet Neurology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- mediaTUM – the media and publications repository of the Technical University Munich (Technical University Munich) (submittedVersion)
- DZNE Pub (submittedVersion)
- Funders
- Deutsche Forschungsgemeinschaft (HO2402/6‐2)
- National Institutes of Health
- National Institute on Aging (P50AG005131, U01AG045390, R01AG038791, P01AG019724)
- National Heart, Lung, and Blood Institute (T35HL007491)
- National Institute of Neurological Disorders and Stroke (U01NS086659, U54NS092089)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Parkinson's Disease Mechanisms and Treatments
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Keywords
- progressive supranuclear palsy, diagnostic criteria, tau pathology, 4-repeat tau, biomarkers, atypical parkinsonism, disease-modifying therapy, neurodegenerative diseases, Alzheimer's disease, chronic traumatic encephalopathy
- MeSH terms
- Humans, Supranuclear Palsy, Progressive, Biomarkers
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