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@ARTICLE{Giagkou:144973,
author = {Giagkou, Nikolaos and Höglinger, Günter U and Stamelou,
Maria},
title = {{P}rogressive supranuclear palsy.},
journal = {International review of neurobiology},
volume = {149},
issn = {0074-7742},
address = {Heidelberg [u.a.]},
publisher = {Elsevier, Acad. Press},
reportid = {DZNE-2020-00337},
pages = {49-86},
year = {2019},
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.},
keywords = {Humans / Supranuclear Palsy, Progressive: diagnosis /
Supranuclear Palsy, Progressive: drug therapy / Supranuclear
Palsy, Progressive: metabolism / Supranuclear Palsy,
Progressive: physiopathology},
cin = {AG Höglinger 1 ; AG Höglinger},
ddc = {610},
cid = {I:(DE-2719)1110002},
pnm = {344 - Clinical and Health Care Research (POF3-344)},
pid = {G:(DE-HGF)POF3-344},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:31779824},
doi = {10.1016/bs.irn.2019.10.013},
url = {https://pub.dzne.de/record/144973},
}