The Progressive Supranuclear Palsy: Past and Present Aspects

Clin Gerontol. 2020 Mar-Apr;43(2):155-180. doi: 10.1080/07317115.2019.1694115. Epub 2019 Nov 22.

Abstract

Objectives: Recently, new criteria for sensitive and specific clinical diagnosis of progressive supranuclear palsy (PSP) have been addressed while distinct clinical phenotypes of the disorder have been increasingly described in the literature. This study aimed to describe past and present aspects of the disease as well as to highlight the cognitive and behavioral profile of PSP patients in relation to the underlying pathology, genetics and treatment procedures.Methods: A Medline and Scopus search was performed to identify articles published on this topic. Articles published solely in English were considered.Results: The most common clinical characteristics of PSP included early postural instability and falls, vertical supranuclear gaze palsy, parkinsonism with poor response to levodopa and pseudobulbar palsy. Frontal dysfunction and verbal fluency deficits were the most distinct cognitive impairments in PSP while memory, visuospatial and social cognition could also be affected. Apathy and impulsivity were also present in PSP patients and had significant impact on relatives and caregivers.Conclusions: PSP is a neurodegenerative disorder with prominent tau neuropathology. Movement, motivation and communication impairments in patients with PSP may limit participation in everyday living activities. Comprehensive neuropsychological assessments are of significant importance for PSP cognitive evaluation. Pharmacologic and non-pharmacologic approaches could be applied in order to relieve patients and improve quality of life.Clinical Implications: Executive dysfunction is the most notable cognitive impairment and dominates the neuropsychological profile of patients with PSP.

Keywords: Cognition; MAPT; executive functions; neurodegeneration; parkinsonism.

Publication types

  • Systematic Review

MeSH terms

  • Activities of Daily Living
  • Cognitive Dysfunction / etiology
  • Cognitive Dysfunction / genetics
  • Humans
  • Neuropsychological Tests
  • Phenotype
  • Quality of Life*
  • Supranuclear Palsy, Progressive / complications
  • Supranuclear Palsy, Progressive / diagnosis
  • Supranuclear Palsy, Progressive / physiopathology*
  • Supranuclear Palsy, Progressive / therapy