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



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.


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.


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.


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.


Progressive supranuclear palsy Diagnostic criteria MDS Subtype 



Rey’s auditory 15-word learning test


Apathy evaluation scale


Analysis of variance


Beck depression inventory II


Benton’s judgment of line orientation


Clock drawing test


Statistical Diagnostic Manual of Psychiatry-5th edition


Neuropsychological examination of aphasia battery


Instrumental activities of daily life


Mild cognitive impairment


Montreal cognitive assessment battery


Magnetic resonance imaging


Movement disorders society

NINDS-PSP criteria

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


Natural history and neuroprotection in Parkinson plus syndrome


Progressive supranuclear palsy


Progressive supranuclear palsy with predominant corticobasal syndrome


Progressive supranuclear palsy with dementia


Progressive supranuclear palsy with predominant frontal presentation


Progressive supranuclear palsy with mild cognitive impairment-multiple domain


Progressive supranuclear palsy with mild cognitive impairment-single domain


Progressive supranuclear palsy with normal cognition


Progressive supranuclear palsy with predominant parkinsonism


Progressive supranuclear palsy with predominant progressive gait freezing


Progressive supranuclear palsy-rating scale


Progressive supranuclear palsy with predominant Richardson’s syndrome subtype


Progressive supranuclear palsy with predominant speech/language disorder


Statistical package for social science


Semantic verbal fluency


Trial making test


Visual analog scale



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.


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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