TY - JOUR
T1 - The diagnosis of progressive supranuclear palsy
T2 - current opinions and challenges
AU - Ali, Farwa
AU - Josephs, Keith
N1 - Publisher Copyright:
© 2018, © 2018 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2018/7/3
Y1 - 2018/7/3
N2 - Introduction: Progressive supranuclear palsy (PSP) is associated with microtubule-associated protein tau dysfunction. Originally thought to result in a syndrome of atypical Parkinsonism, vertical supranuclear gaze palsy, and cognitive impairment, several additional phenotypic manifestations of PSP pathology have been described over the last 20 years. Furthermore, prototypical PSP features may develop late, making early diagnosis challenging. Areas covered: An in-depth view of emerging knowledge in the field of PSP. Advances in clinicopathologic correlation, blood, cerebrospinal, and more importantly neuroimaging biomarkers are discussed in light of the 2017 PSP diagnostic criteria by the Movement Disorders Society Study Group. Discoveries related to molecular pathogenesis have enabled development of disease-modifying therapies for PSP, many of which are currently under investigation. Expert commentary: Despite remarkable growth in our knowledge of tauopathies like PSP, early and accurate clinical prediction of PSP neuropathology remains challenging. Clinical phenotypes overlap, and biomarkers are nonspecific. There is a pressing need for disease-specific biomarkers that enable timely identification of patients and biomarker-driven investigation of disease-modifying therapies.
AB - Introduction: Progressive supranuclear palsy (PSP) is associated with microtubule-associated protein tau dysfunction. Originally thought to result in a syndrome of atypical Parkinsonism, vertical supranuclear gaze palsy, and cognitive impairment, several additional phenotypic manifestations of PSP pathology have been described over the last 20 years. Furthermore, prototypical PSP features may develop late, making early diagnosis challenging. Areas covered: An in-depth view of emerging knowledge in the field of PSP. Advances in clinicopathologic correlation, blood, cerebrospinal, and more importantly neuroimaging biomarkers are discussed in light of the 2017 PSP diagnostic criteria by the Movement Disorders Society Study Group. Discoveries related to molecular pathogenesis have enabled development of disease-modifying therapies for PSP, many of which are currently under investigation. Expert commentary: Despite remarkable growth in our knowledge of tauopathies like PSP, early and accurate clinical prediction of PSP neuropathology remains challenging. Clinical phenotypes overlap, and biomarkers are nonspecific. There is a pressing need for disease-specific biomarkers that enable timely identification of patients and biomarker-driven investigation of disease-modifying therapies.
KW - AV-1451
KW - PSP
KW - Progressive supranuclear palsy
KW - tau PET
UR - http://www.scopus.com/inward/record.url?scp=85050202810&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85050202810&partnerID=8YFLogxK
U2 - 10.1080/14737175.2018.1489241
DO - 10.1080/14737175.2018.1489241
M3 - Review article
C2 - 29902389
AN - SCOPUS:85050202810
SN - 1473-7175
VL - 18
SP - 603
EP - 616
JO - Expert review of neurotherapeutics
JF - Expert review of neurotherapeutics
IS - 7
ER -