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    Prospective Characterization of Cognitive Function in Typical and 'Brainstem Predominant' Progressive Supranuclear Palsy Phenotypes

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    Author
    Lee, Y-EC; Williams, DR; Anderson, JFI
    Date
    2018-05-01
    Source Title
    Journal of Movement Disorders
    Publisher
    KOREAN MOVEMENT DISORDERS SOC
    University of Melbourne Author/s
    Anderson, Jacqueline; Lee, Young-Eun
    Affiliation
    Melbourne School of Psychological Sciences
    Melbourne Conservatorium of Music
    Metadata
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    Document Type
    Journal Article
    Citations
    Lee, Y. -E. C., Williams, D. R. & Anderson, J. F. I. (2018). Prospective Characterization of Cognitive Function in Typical and 'Brainstem Predominant' Progressive Supranuclear Palsy Phenotypes. JOURNAL OF MOVEMENT DISORDERS, 11 (2), pp.72-77. https://doi.org/10.14802/jmd.17067.
    Access Status
    Open Access
    URI
    http://hdl.handle.net/11343/255240
    DOI
    10.14802/jmd.17067
    Abstract
    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.

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