Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Sep;268(9):3400-3408.
doi: 10.1007/s00415-021-10511-y. Epub 2021 Mar 11.

Cognitive and behavioral profile of progressive supranuclear palsy and its phenotypes

Andrea Horta-Barba  1   2   3   4   5 , Javier Pagonabarraga  6   7   8   9 , Saül Martínez-Horta  1   2   3   4 , Laura Busteed  1 , Berta Pascual-Sedano  1   2   3   4   5 , Ignacio Illán-Gala  3   4   10 , Juan Marin-Lahoz  1   2   3 , Ignacio Aracil-Bolaños  1   2   3   4 , Jesús Pérez-Pérez  1   2   3   4 , Frederic Sampedro  1   3   4 , Helena Bejr-Kasem  1   2   3   4   5 , Jaime Kulisevsky  11   12   13   14   15
Affiliations

Cognitive and behavioral profile of progressive supranuclear palsy and its phenotypes

Andrea Horta-Barba et al. J Neurol. 2021 Sep.

Abstract

Background: Although several progressive supranuclear palsy (PSP) phenotypes have recently been described, studies identifying cognitive and neuropsychiatric differences between them are lacking.

Methods: An extensive battery of cognitive and behavioural assessments was administered to 63 PSP patients, 25 PD patients with similar sociodemographic characteristics, and 25 healthy controls. We analysed differences in phenomenology, frequency and severity of cognitive and neuropsychiatric symptoms between PSP, PD and HC, and between PSP subtypes.

Results: Regarding phenotypes, 64.6% met criteria for Richardson's syndrome (PSP-RS), 10.7% PSP with predominant Parkinsonism (PSP-P), 10.7% with PSP progressive gait freezing (PSP-PGF), and 10.7% PSP with predominant speech/language disorder (PSP-SL). Impairment was more severe in the PSP group than in the PD and HC groups regarding motor scores, cognitive testing and neuropsychiatric scales. Cognitive testing did not clearly differentiate between PSP phenotypes, but PSP-RS and PSP-SL appeared to have more cognitive impairment than PSP-PGF and PSP-P, mainly due to an increased impairment in frontal executive domains. Regarding neuropsychiatric disturbances, no specific behavior was more common in any of the PSP subtypes.

Conclusion: Motor deficits delineate the phenotypes included in currently accepted MDS-PSP criteria. Cognition and behavioural disturbances are common in PSP and allow us to distinguish this disorder from other neurological diseases, but they do not differentiate between PSP phenotypes.

Keywords: Behavior; Cognition; Motor features; PSP phenotypes; Progressive supranuclear palsy.

PubMed Disclaimer

References

    1. Nath U (2001) The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain 124(7):1438–1449. https://doi.org/10.1093/brain/124.7.1438 - DOI - PubMed
    1. Coughlin D, Irwin DJ (2017) Emerging diagnostic and therapeutic strategies for tauopathies. Curr Neurol Neurosci Rep 17:9. https://doi.org/10.1007/s11910-017-0779-1 - DOI
    1. Boxer AL, Yu JT, Golbe LI, Litvan I, Lang AE, Höglinger GU (2017) Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches. Lancet Neurol 16(7):552–563. https://doi.org/10.1016/S1474-4422(17)30157-6 - DOI - PubMed - PMC
    1. Cordato NJ, Halliday GM, Caine D, Morris JGL (2006) Comparison of motor, cognitive, and behavioral features in progressive supranuclear palsy and Parkinson’s disease. Mov Disord 21(5):632–638. https://doi.org/10.1002/mds.20779 - DOI - PubMed
    1. Williams DR, Lees AJ (2009) Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 8(3):270–279. https://doi.org/10.1016/S1474-4422(09)70042-0 - DOI - PubMed

LinkOut - more resources