Progressive supranuclear palsy

Introduction
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By Robert Fekete MD

Progressive supranuclear palsy (PSP) is also known as or subsumes Richardson disease. -ed.

Progressive supranuclear palsy remains an intractable and under-recognized neurodegenerative illness with a median survival of only 7 years. However, patients will benefit from accurate, early diagnosis that allows them to understand the prognosis, to avoid unnecessary tests and useless treatments, and to avail themselves of disease-specific support services. Advances in understanding the tau protein aggregation and mitochondrial dysfunction of progressive supranuclear palsy and the toxic and the genetic insults underlying them are accruing swiftly. In this clinical article, Dr. Robert Fekete of Baylor College of Medicine describes the subtypes of progressive supranuclear palsy as well as advances in pathology, imaging, and genetics of the disorder.

Key points

  • Ophthalmoparesis, especially downgaze impairment, is a classic clinical feature of progressive supranuclear palsy that may appear later in the course of the disorder.
  • Slowing of downard saccades appears earlier in the clinical course of progressive supranuclear palsy.
  • Multiple subtypes of progressive supranuclear palsy have been identified, including the classic Richardson syndrome, progressive supranuclear palsy-parkinsonism (PSP-P), or pure-akinesia-with-gait-freezing (PAGF).

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Pregnancy
Anesthesia
References cited
Contributors