Motor, cognitive and behavioral differences in MDS PSP phenotypes
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.
KeywordsProgressive 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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