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Cognitive–behavioural features of progressive supranuclear palsy syndrome overlap with frontotemporal dementia

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Abstract

Cognitive impairment is common in patients with the neurodegenerative tauopathy progressive supranuclear palsy (PSP). Although a pattern of ‘subcortical’ cognitive impairment is considered prototypical in PSP, pathological and clinical observations suggest an overlap with frontotemporal dementia (FTD). Our objective was to evaluate behavioural and cognitive symptoms in a retrospective study of patients with PSP syndrome (PSPS) and their relationship to features seen in behavioural variant FTD. We reviewed the records of 62 patients (29 male, 33 female, median age 65.5 years) evaluated at a tertiary cognitive clinic who met NINDS–SPSP criteria for probable or possible PSP, and collected clinical details of their presenting history, cognitive and behavioural features. We also evaluated the proportion of patients fulfilling FTD Consensus criteria. Cognitive and behavioural symptoms were a predominant presenting feature in 58 % of patients evaluated. Cognitive slowing, executive impairments, and inefficient memory recall, consistent with ‘subcortical’ impairment, were identified in the majority of patients. Twenty patients (32 %) fulfilled cognitive and behavioural criteria for possible FTD at initial assessment, whereas behavioural changes not meeting formal diagnostic criteria were present in a greater proportion of the patients. Our findings support the existence of a spectrum of cognitive–behavioural features in PSPS, with significant clinical overlap with behavioural variant FTD. Cognitive and behavioural profiling should be an integral part of the assessment of patients with PSPS.

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Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

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Authors and Affiliations

  1. Cerebral Function Unit, Greater Manchester Neurosciences Centre, Salford Royal NHS Foundation Trust, Stott Lane, Salford, M6 8HD, UK

    Christopher Kobylecki, Matthew Jones, Jennifer C. Thompson, Anna M. Richardson, David Neary, David M. A. Mann, Julie S. Snowden & Alexander Gerhard

  2. Centre for Clinical and Cognitive Neuroscience, Institute of Brain, Behaviour and Mental Health, University of Manchester, Manchester, UK

    Christopher Kobylecki, Matthew Jones, Jennifer C. Thompson, David Neary, David M. A. Mann, Julie S. Snowden & Alexander Gerhard

  3. Manchester Medical School, University of Manchester, Manchester, UK

    Anna M. Richardson

Authors
  1. Christopher Kobylecki
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  2. Matthew Jones
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  3. Jennifer C. Thompson
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  4. Anna M. Richardson
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  5. David Neary
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Correspondence to Christopher Kobylecki.

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Kobylecki, C., Jones, M., Thompson, J.C. et al. Cognitive–behavioural features of progressive supranuclear palsy syndrome overlap with frontotemporal dementia. J Neurol 262, 916–922 (2015). https://doi.org/10.1007/s00415-015-7657-z

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  • DOI: https://doi.org/10.1007/s00415-015-7657-z

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