Progressive Supranuclear Palsy

Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disorder affecting movement, vision, speech, and cognition. Symptoms typically emerge in the 60s, with most individuals surviving 5-9 years post-diagnosis, though some live longer.[1] First identified in 1964 as distinct from Parkinson’s disease, PSP—sometimes called Steele-Richardson-Olszewski syndrome—has no cure, though some symptoms can be managed with medication or therapy.[2]

PSP affects 3-6 per 100,000 people worldwide (about 20,000 Americans), making it far less common than Parkinson’s.[1] It involves tau protein buildup in the brain, leading to progressive deterioration. While its cause remains unknown, research continues to explore genetic and environmental factors.

Quick Facts

  • No known cause or cure[3]
  • Affects balance, walking, coordination, eye movement, speech, swallowing, and thinking[1]
  • Prevalence: 5-6 per 100,000[1]
  • Onset typically in early 60s, sometimes 40s[1]
  • Slightly more common in men, no racial or occupational bias[1]

Symptoms

Early signs often include loss of balance and frequent falls, progressing to slow movements (bradykinesia), clumsiness, and trunk stiffness, eventually requiring wheelchair use.[1] PSP is marked by abnormal eye movements, particularly vertical gaze palsy, causing blurred vision, light sensitivity, and a staring gaze.[4] Other symptoms include slurred speech (dysarthria), difficulty swallowing (dysphagia), and personality changes like apathy, alongside cognitive declines in attention and planning.[5]

  • Loss of balance and falls
  • Personality changes (e.g., apathy, disinhibition)
  • Weak eye movements, especially downward
  • Slurred speech and swallowing issues
  • Slowed movement and neck stiffness

Cause and Pathophysiology

The cause of PSP is unclear, with less than 1% of cases familial. A genetic variant, the H1 haplotype on chromosome 17, is linked to PSP but isn’t sufficient alone.[6] Environmental toxins and mitochondrial dysfunction are also under investigation.[7] PSP involves tau protein tangles in neurons and glial cells, primarily affecting the basal ganglia, brainstem, cerebral cortex, and cerebellum.[8]

Diagnosis

PSP is often mistaken for Parkinson’s or Alzheimer’s due to overlapping symptoms.[9] Key diagnostic features include poor levodopa response, symmetrical onset, and eye movement issues (e.g., downgaze palsy).[10] MRI may show midbrain atrophy (the "hummingbird" sign), aiding diagnosis.[11]

Management

No cure exists, but supportive care includes medications like levodopa or amantadine for some symptoms,[1] Botox for dystonia,[12] and therapies (occupational, speech, physical) to manage balance, speech, and mobility.[13] Experimental tau-targeting treatments are under research.[14]

Prognosis and History

Survival averages 7 years from onset, with pneumonia a common cause of death due to swallowing difficulties.[1] PSP was first detailed in 1963 by Steele, Richardson, and Olszewski,[2] with earlier cases noted from 1877 and cognitive features highlighted in 1974.[15]

For more PSP resources, visit our Resource Links!

Donorbox Logo Donate