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Degrees of diagnostic certainty for progressive supranuclear palsy (PSP), obtained by combinations of clinical features and clinical clues

Degrees of diagnostic certainty for progressive supranuclear palsy (PSP), obtained by combinations of clinical features and clinical clues
Diagnostic certainty Definition Combinations of clinical features and clinical clues Predominance type Abbreviation
Definite PSP Gold standard defining the disease entity. Neuropathological diagnosis Any clinical presentation Definite PSP
Probable PSP

Highly specific, but not very sensitive for PSP.

Suitable for therapeutic and biological studies.
(O1 or O2) + (P1 or P2) PSP with Richardson's syndrome (PSP-RS) Probable PSP-RS
(O1 or O2) + A1 PSP with progressive gait freezing (PSP-PGF) Probable PSP-PGF
(O1 or O2) + (A2 or A3) PSP with predominant parkinsonism (PSP-P) Probable PSP-P
(O1 or O2) + C2 PSP with predominant frontal presentation (PSP-F) Probable PSP-F
Possible PSP

Substantially more sensitive, but less specific for PSP.

Suitable for descriptive epidemiological studies and clinical care.
O1 PSP with predominant ocular motor dysfunction (PSP-OM) Possible PSP-OM
O2 + P3 PSP-RS Possible PSP-RS
A1 PSP-PGF Possible PSP-PGF
(O1 or O2) + C1 PSP with predominant speech/language disorder (PSP-SL)* Possible PSP-SL
(O1 or O2) + C3 PSP with predominant corticobasal syndrome (PSP-CBS)* Possible PSP-CBS
Suggestive of PSP

Suggestive of PSP, but not passing the threshold for possible or probable PSP.

Suitable for early identification.
O2 or O3 PSP-OM Suggestive of PSP-OM
P1 or P2 PSP with predominant postural instability (PSP-PI) Suggestive of PSP-PI
O3 + (P2 or P3) PSP-RS Suggestive of PSP-RS
(A2 or A3) + (O3, P1, P2, C1, C2, CC1, CC2, CC3, or CC4) PSP-P Suggestive of PSP-P
C1 PSP-SL Suggestive of PSP-SL
C2 + (O3 or P3) PSP-F Suggestive of PSP-F
C3 PSP-CBS Suggestive of PSP-CBS
The basic features (mandatory inclusion and exclusion criteria, and context-dependent exclusion criteria; refer to UpToDate text for details) apply for all probable, possible, and suggestive degrees of certainty. Core clinical features are defined by their functional domain (ocular motor dysfunction [O], postural instability [P], akinesia [A], and cognitive dysfunction [C]), and stratified by presumed levels of certainty (1 = highest, 2 = middle, 3 = lowest) that contribute to the diagnosis of PSP. Supportive clinical clues are described in the UpToDate text. Operationalized definitions of clinical features and clinical clues are described in a separate UpToDate table.
CC1: levodopa resistance; CC2: hypokinetic, spastic dysarthria; CC3: dysphagia; CC4: photophobia.
* Probable 4R-tauopathy (ie, either PSP or corticobasal degeneration).
From: Höglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: The Movement Disorder Society criteria. Mov Disord 2017; 32:853. http://onlinelibrary.wiley.com/wol1/doi/10.1002/mds.26987/abstract. Copyright © 2017 International Parkinson and Movement Disorder Society. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (http://onlinelibrary.wiley.com).
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