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1 "David R Williams"
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Original Article
Prospective Characterization of Cognitive Function in Typical and ‘Brainstem Predominant’Progressive Supranuclear Palsy Phenotypes
Young-Eun C Lee, David R Williams, Jacqueline F I Anderson
J Mov Disord. 2018;11(2):72-77.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.17067
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  • 7 Web of Science
  • 7 Crossref
AbstractAbstract PDF
Objective
Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson’s syndrome (PSP-RS) and two atypical ‘brainstem predominant’ PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery.
Methods
Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests.
Results
The typical PSP-RS subgroup demonstrated greater impairments in processing speed [t(19) = -4.10, p = 0.001 (d =1.66)] and executive function [t(19) = -2.63, p = 0.02 (d = 1.20)] compared to the ‘brainstem predominant’ PSP phenotype.
Conclusion
This is the first prospective study to demonstrate that PSP-RS and ‘brainstem predominant’ PSP phenotypes can be differentiated on cognitive grounds. These differences correspond with variations in pathological profiles reported in the literature.

Citations

Citations to this article as recorded by  
  • Pathomechanisms of cognitive impairment in progressive supranuclear palsy
    Kurt A. Jellinger
    Journal of Neural Transmission.2023; 130(4): 481.     CrossRef
  • Differential Diagnosis of Rare Subtypes of Progressive Supranuclear Palsy and PSP-Like Syndromes—Infrequent Manifestations of the Most Common Form of Atypical Parkinsonism
    Patrycja Krzosek, Natalia Madetko, Anna Migda, Bartosz Migda, Dominika Jaguś, Piotr Alster
    Frontiers in Aging Neuroscience.2022;[Epub]     CrossRef
  • Clinical Spectrum of Tauopathies
    Nahid Olfati, Ali Shoeibi, Irene Litvan
    Frontiers in Neurology.2022;[Epub]     CrossRef
  • Neuropsychological assessment could distinguish among different clinical phenotypes of progressive supranuclear palsy: A Machine Learning approach
    Maria Grazia Vaccaro, Alessia Sarica, Andrea Quattrone, Carmelina Chiriaco, Maria Salsone, Maurizio Morelli, Aldo Quattrone
    Journal of Neuropsychology.2021; 15(3): 301.     CrossRef
  • “Parkinson’s disease” on the way to progressive supranuclear palsy: a review on PSP-parkinsonism
    Ján Necpál, Miroslav Borsek, Bibiána Jeleňová
    Neurological Sciences.2021; 42(12): 4927.     CrossRef
  • The Progressive Supranuclear Palsy: Past and Present Aspects
    Theodore P. Parthimos, Kleopatra H. Schulpis
    Clinical Gerontologist.2020; 43(2): 155.     CrossRef
  • Progressive Supranuclear Palsy—Parkinsonism Predominant (PSP-P)—A Clinical Challenge at the Boundaries of PSP and Parkinson's Disease (PD)
    Piotr Alster, Natalia Madetko, Dariusz Koziorowski, Andrzej Friedman
    Frontiers in Neurology.2020;[Epub]     CrossRef

JMD : Journal of Movement Disorders