<p>Progressive supranuclear palsy (PSP) is an adult-onset neurodegenerative disorder characterized by postural instability, ocular motor dysfunction, akinesia, and cognitive impairment. The accumulation of tau protein disrupts microtubule dynamics and axonal transport, leading to neurodegeneration in subcortical structures such as the basal ganglia and brainstem. These effects result in the clinical features of motor dysfunction and cognitive impairment associated with PSP. Dementia in patients with PSP is common but may be difficult to diagnose. This narrative review describes the cognitive decline and dementia associated with PSP. The pathological hallmarks of PSP, clinical features, and diagnostic criteria are also discussed to provide additional context for the diagnosis of PSP dementia.</p>

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Dementia in Progressive Supranuclear Palsy: A Narrative Review

  • Kathleen S. Moore,
  • Yara S. Stipho,
  • Nicki Niemann,
  • Holly A. Shill,
  • Justine Chan,
  • Crystal J. Abrego,
  • Danielle Eagan,
  • Boris Decourt,
  • Marwan N. Sabbagh

摘要

Progressive supranuclear palsy (PSP) is an adult-onset neurodegenerative disorder characterized by postural instability, ocular motor dysfunction, akinesia, and cognitive impairment. The accumulation of tau protein disrupts microtubule dynamics and axonal transport, leading to neurodegeneration in subcortical structures such as the basal ganglia and brainstem. These effects result in the clinical features of motor dysfunction and cognitive impairment associated with PSP. Dementia in patients with PSP is common but may be difficult to diagnose. This narrative review describes the cognitive decline and dementia associated with PSP. The pathological hallmarks of PSP, clinical features, and diagnostic criteria are also discussed to provide additional context for the diagnosis of PSP dementia.