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Motor, cognitive and behavioral differences in MDS PSP phenotypes

  • Marina PicilloEmail author
  • Sofia Cuoco
  • Maria Francesca Tepedino
  • Arianna Cappiello
  • Giampiero Volpe
  • Roberto Erro
  • Gabriella Santangelo
  • Maria Teresa Pellecchia
  • Paolo Barone
  • The PSP Salerno study group
Original Communication

Abstract

Introduction

Movement Disorder Society (MDS) new diagnostic criteria for Progressive Supranuclear palsy (PSP) identifying different disease phenotypes were recently released. The aim of the present study is to report on the cognitive and behavioral features of the different phenotypes diagnosed according to the MDS criteria.

Methods

Forty-nine PSP patients underwent an extensive battery of clinical assessments. Differences between PSP subtypes were computed with χ2 or ANOVA tests. Using the z scores, subjects were classified as having normal cognition, mild cognitive impairment, single or multiple domain, and dementia. A logistic regression model was implemented to investigate the major determinants of PSP non-Richardson’s syndrome phenotype.

Results

Half of the cohort presented Richardson’s syndrome (46.9%), followed by PSP with parkinsonism and corticobasal syndrome (22.4% and 14.2%, respectively). Richardson’s syndrome and PSP with corticobasal syndrome presented a similar burden of disease. The only cognitive testing differentiating the phenotypes were semantic fluency and ideomotor apraxia. The majority of our cohort was either affected by dementia or presented normal cognition. Richardson’s syndrome presented the highest rate of dementia. The only marker of PSP non-Richardson’s syndrome phenotype was better performance in visuo-spatial testing, implying worse visuo-spatial abilities in PSP Richardson’s syndrome.

Conclusion

Available clinical assessments hardly capture differences between PSP phenotypes. The cognitive testing differentiating the PSP phenotypes were semantic fluency and ideomotor apraxia. In PSP, mild cognitive impairment likely represents an intermediate step from normal cognition to dementia. The only marker of PSP non-Richardson’s syndrome phenotype was better performance in visuo-spatial testing.

Keywords

Progressive supranuclear palsy Diagnostic criteria MDS Subtype 

Abbreviations

15-RAWLT

Rey’s auditory 15-word learning test

AES

Apathy evaluation scale

ANOVA

Analysis of variance

BDI-II

Beck depression inventory II

BJLO

Benton’s judgment of line orientation

CDT

Clock drawing test

DSM-5th

Statistical Diagnostic Manual of Psychiatry-5th edition

ENPA

Neuropsychological examination of aphasia battery

IADL

Instrumental activities of daily life

MCI

Mild cognitive impairment

MoCA

Montreal cognitive assessment battery

MRI

Magnetic resonance imaging

MDS

Movement disorders society

NINDS-PSP criteria

National Institute of Neurodegenerative Disorders and Stroke-progressive supranuclear palsy criteria

NNIPPS

Natural history and neuroprotection in Parkinson plus syndrome

PSP

Progressive supranuclear palsy

PSP-CBS

Progressive supranuclear palsy with predominant corticobasal syndrome

PSP-D

Progressive supranuclear palsy with dementia

PSP-F

Progressive supranuclear palsy with predominant frontal presentation

PSP-MCImd

Progressive supranuclear palsy with mild cognitive impairment-multiple domain

PSP-MCI-sd

Progressive supranuclear palsy with mild cognitive impairment-single domain

PSP-NC

Progressive supranuclear palsy with normal cognition

PSP-P

Progressive supranuclear palsy with predominant parkinsonism

PSP-PGF

Progressive supranuclear palsy with predominant progressive gait freezing

PSP-rs

Progressive supranuclear palsy-rating scale

PSP-RS

Progressive supranuclear palsy with predominant Richardson’s syndrome subtype

PSP-SL

Progressive supranuclear palsy with predominant speech/language disorder

SPSS

Statistical package for social science

SVP

Semantic verbal fluency

TMT

Trial making test

VAS

Visual analog scale

Notes

Acknowledgements

The PSP Salerno Study Group: Marina Picillo, Roberto Erro, Sofia Cuoco, Maria Francesca Tepedino, Renzo Manara, Maria Teresa Pellecchia, Paolo Barone, Marianna Amboni, Arianna Cappiello, Immacolata Carotenuto, Giovanna Dati, Gabriella Santangelo, Pietro Siano, Annamaria Vallelunga, Giampiero Volpe.

Funding

There is no specific source of funding for this manuscript.

Compliance with ethical standards

Conflicts of interest

MP is supported by the Michael J Fox Foundation for Parkinson’s research; PB received consultancies as a member of the advisory board for Zambon, Lundbeck, UCB, Chiesi, Abbvie and Acorda; RE received consultancies from Zambon and honoraria from TEVA; the other authors report no financial disclosures.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Marina Picillo
    • 1
    Email author
  • Sofia Cuoco
    • 1
  • Maria Francesca Tepedino
    • 1
  • Arianna Cappiello
    • 1
  • Giampiero Volpe
    • 1
  • Roberto Erro
    • 1
  • Gabriella Santangelo
    • 2
  • Maria Teresa Pellecchia
    • 1
  • Paolo Barone
    • 1
  • The PSP Salerno study group
  1. 1.Department of Medicine, Surgery and Dentistry, Neuroscience SectionCenter for Neurodegenerative Diseases (CEMAND), University of SalernoBaronissi (Salerno)Italy
  2. 2.Department of PsychologyUniversity of Campania “Luigi Vanvitelli”CasertaItaly

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