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Characteristics and progression of cognitive deficits in progressive supranuclear palsy vs. multiple system atrophy and Parkinson’s disease

  • Neurology and Preclinical Neurological Studies - Original Article
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Abstract

Cognitive impairment is frequent in progressive supranuclear palsy (PSP) and less common in multiple system atrophy (MSA), but characteristics and progression compared with Parkinson’s disease (PD) need to be properly defined. We evaluated 35 PSP with Richardson’s syndrome (PSP-RS), 30 MSA as well as 65 age-, sex-, and education-matched PD with an extensive clinical and neuropsychological assessment, allowing Level II cognitive diagnosis. Eighteen PSP, 12 MSA and 30 PD had a second evaluation between 12 and 18 months (mean 15 months) after the first assessment. PSP performance at Montreal Cognitive Assessment (MoCA), verbal fluencies (phonemic and semantic tasks), Stroop test (Error and Time), Digit Span Sequencing (DSS), incomplete letters of Visual Object and Space Perception (VOSP) and Benton’s Judgment of Line Orientation (JLO) performance were significantly poorer at baseline compared to PD and MSA. Executive, language and visuospatial abilities declined longitudinally in PSP, but not in PD and MSA. After 1.5 year, 16% of PSP converted to dementia. Our study provides evidence that cognitive progression is more severe and rapid in PSP-RS than PD and MSA. Further, we observed that MoCA, verbal fluency (particularly semantic), DSS and Benton’s JLO are valuable tests to detect cognitive progression in PSP-RS and may be proposed as possible biomarker to assess efficacy of disease modification strategies.

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Acknowledgements

We would like to thank the patients for participating in this study.

Funding

The work of the authors was supported by the Ministry of Health under Grant number GR-2016-02361986.

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Authors and Affiliations

  1. IRCCS San Camillo Hospital, Via Alberoni, 70, 30126, Venice, Italy

    Eleonora Fiorenzato, Valeria Camparini, Luca Weis, Carlo Semenza & Roberta Biundo

  2. Department of Neurosciences, Padova Neuroscience Center, University of Padua, Via Giustiniani, 5, 35128, Padua, Italy

    Angelo Antonini & Carlo Semenza

Authors
  1. Eleonora Fiorenzato
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  2. Angelo Antonini
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  3. Valeria Camparini
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  4. Luca Weis
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  5. Carlo Semenza
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  6. Roberta Biundo
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Correspondence to Eleonora Fiorenzato.

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This study was approved by the Venice Research Ethics Committee of Venice, Italy.

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Fiorenzato, E., Antonini, A., Camparini, V. et al. Characteristics and progression of cognitive deficits in progressive supranuclear palsy vs. multiple system atrophy and Parkinson’s disease. J Neural Transm 126, 1437–1445 (2019). https://doi.org/10.1007/s00702-019-02065-1

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  • DOI: https://doi.org/10.1007/s00702-019-02065-1

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