Journal Article

DZNE-2020-00337

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Progressive supranuclear palsy.

Giagkou, N. ; Höglinger, G. U.DZNE* ; Stamelou, M. (Corresponding author)

2019
Elsevier, Acad. Press Heidelberg [u.a.]

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Please use a persistent id in citations: doi:10.1016/bs.irn.2019.10.013

Abstract: Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized pathologically by 4 repeat tau deposition in various cell types and anatomical regions. Richardson's syndrome (RS) is the initially described and one of the clinical phenotypes associated with PSP pathology, characterized by vertical supranuclear gaze paly in particular downwards, postural instability with early falls and subcortical frontal dementia. PSP can manifest as several other clinical phenotypes, including PSP-parkinsonism, -pure akinesia with gait freezing, -frontotemporal dementia, - corticobasal syndrome, - speech/language impairment. RS can also have a pathologic diagnosis other than PSP, including corticobasal degeneration, FTD-TDP-43 and others. New clinical diagnostic criteria take into account this phenotypic variability in an attempt to diagnose the disease earlier, given the current lack of a validated biomarker. At present, therapeutic options for PSP are symptomatic and insufficient. Recent large neuroprotective trials have failed to provide a positive clinical outcome, however, have led to the design of better studies that are ongoing and hold promise for a neuroprotective treatment for PSP.

Keyword(s): Humans (MeSH) ; Supranuclear Palsy, Progressive: diagnosis (MeSH) ; Supranuclear Palsy, Progressive: drug therapy (MeSH) ; Supranuclear Palsy, Progressive: metabolism (MeSH) ; Supranuclear Palsy, Progressive: physiopathology (MeSH)

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Contributing Institute(s):

1. Translational Neurodegeneration (AG Höglinger 1 ; AG Höglinger)

Research Program(s):

1. 344 - Clinical and Health Care Research (POF3-344) (POF3-344)

Appears in the scientific report 2019

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Database coverage:
; BIOSIS Previews ; BIOSIS Reviews Reports And Meetings ; Clarivate Analytics Master Journal List ; NCBI Molecular Biology Database ; SCOPUS ; Science Citation Index ; Science Citation Index Expanded ; Web of Science Core Collection

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