TY  - JOUR
AU  - Giagkou, Nikolaos
AU  - Höglinger, Günter U
AU  - Stamelou, Maria
TI  - Progressive supranuclear palsy.
JO  - International review of neurobiology
VL  - 149
SN  - 0074-7742
CY  - Heidelberg [u.a.]
PB  - Elsevier, Acad. Press
M1  - DZNE-2020-00337
SP  - 49-86
PY  - 2019
AB  - 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.
KW  - Humans
KW  - Supranuclear Palsy, Progressive: diagnosis
KW  - Supranuclear Palsy, Progressive: drug therapy
KW  - Supranuclear Palsy, Progressive: metabolism
KW  - Supranuclear Palsy, Progressive: physiopathology
LB  - PUB:(DE-HGF)16
C6  - pmid:31779824
DO  - DOI:10.1016/bs.irn.2019.10.013
UR  - https://pub.dzne.de/record/144973
ER  -