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Motor, cognitive and behavioral differences in MDS PSP phenotypes.

Marina Picillo1, Sofia Cuoco2, Maria Francesca Tepedino2

  • 1Department of Medicine, Surgery and Dentistry, Neuroscience Section, Center for Neurodegenerative Diseases (CEMAND), University of Salerno, 84131, Baronissi (Salerno), Italy. mpicillo@unisa.it.

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Summary

New criteria for Progressive Supranuclear Palsy (PSP) identify subtypes, but cognitive tests like semantic fluency and ideomotor apraxia are key differentiators. Visuospatial testing may distinguish PSP subtypes, with Richardson's syndrome showing deficits.

Keywords:
Diagnostic criteriaMDSProgressive supranuclear palsySubtype

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Area of Science:

  • Neurology
  • Neuroscience
  • Clinical Diagnostics

Background:

  • The Movement Disorder Society (MDS) recently released new diagnostic criteria for Progressive Supranuclear Palsy (PSP), enabling the identification of distinct disease phenotypes.
  • These criteria aim to refine the classification and understanding of PSP, a rare neurodegenerative disorder.

Purpose of the Study:

  • To characterize the cognitive and behavioral features of different PSP phenotypes as defined by the latest MDS criteria.
  • To investigate the clinical utility of existing assessments in differentiating these PSP subtypes.

Main Methods:

  • A cohort of 49 PSP patients was evaluated using a comprehensive battery of clinical assessments.
  • Statistical analyses (χ², ANOVA) were employed to compare differences between PSP subtypes.
  • Cognitive status was categorized into normal cognition, mild cognitive impairment, single or multiple domain impairment, and dementia using z scores.
  • Logistic regression identified key determinants of the PSP non-Richardson's syndrome phenotype.

Main Results:

  • Richardson's syndrome was the most prevalent phenotype (46.9%), followed by PSP with parkinsonism (22.4%) and corticobasal syndrome (14.2%).
  • Semantic fluency and ideomotor apraxia were the only cognitive tests significantly differentiating the phenotypes.
  • The majority of patients presented with either dementia or normal cognition; Richardson's syndrome had the highest dementia rate.
  • Better visuospatial testing performance was the sole marker for the PSP non-Richardson's syndrome phenotype, indicating poorer visuospatial abilities in Richardson's syndrome.

Conclusions:

  • Current clinical assessments have limited ability to distinguish between PSP phenotypes.
  • Semantic fluency and ideomotor apraxia are crucial cognitive markers for differentiating PSP subtypes.
  • Mild cognitive impairment in PSP appears to be a transitional stage between normal cognition and dementia.
  • Enhanced visuospatial testing performance is a potential indicator for non-Richardson's syndrome PSP.