Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: A neuropsychological and neuroimaging analysis

Authors

  • Jonathan D. Rohrer MRCP,

    1. Department of Neurodegenerative Disease, Dementia Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
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    • The first two authors contributed equally to this work

  • Dominic Paviour PhD, MRCP,

    1. Department of Neurodegenerative Disease, Dementia Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Reta Lila Weston Institute, UCL Institute of Neurology, University College London, London, United Kingdom
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    • The first two authors contributed equally to this work

  • Adolfo M. Bronstein MD, FRCP,

    1. Department of Clinical Neuroscience, Charing Cross Hospital, Imperial College, London, United Kingdom
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  • Sean S. O'Sullivan MRCP,

    1. Reta Lila Weston Institute, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Andrew Lees MD, FRCP,

    1. Reta Lila Weston Institute, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Jason D. Warren PhD, FRACP

    Corresponding author
    1. Department of Neurodegenerative Disease, Dementia Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    • Dementia Research Centre, Institute of Neurology, Queen Square, London WC1N 3BG, UK
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  • Potential conflict of interest: The authors have no other financial disclosures or any conflicts of interest

Abstract

There is currently considerable interest in the clinical spectrum of progressive nonfluent aphasia (PNFA) and progressive supranuclear palsy (PSP) and the intersection of these two entities. Here, we undertook a detailed prospective clinical, neuropsychological, and neuroimaging analysis of 14 consecutive patients presenting with PNFA to identify cases meeting clinical criteria for PSP. These patients had further detailed assessment of extrapyramidal and oculomotor functions. All patients had high-resolution MR brain volumetry and a cortical thickness analysis was undertaken on the brain images. Four patients presenting with PNFA subsequently developed features of a PSP syndrome, including a typical oculomotor palsy. The neuropsychological profile in these cases was similar to other patients with PNFA, however, with more marked reduction in propositional speech, fewer speech errors, less marked impairment of literacy skills but more severe associated deficits of episodic memory and praxis. These PSP-PNFA cases had less prominent midbrain atrophy but more marked prefrontal atrophy than a comparison group of five patients with pathologically confirmed PSP without PNFA and more prominent midbrain atrophy but less marked perisylvian atrophy than other PNFA cases. In summary, although the PSP-PNFA syndrome overlaps with PNFA without PSP, certain neuropsychological and neuroanatomical differences may help predict the development of a PSP syndrome. © 2010 Movement Disorder Society

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