Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder of the brain – a so-called tauopathy – leading to progressive gait, eye movement and cognitive problems with recurrent falls.

Signs and Symptoms

First symptoms of PSP include loss of balance with recurrent falls, and changes of personality. The symptoms can vary between patients. With progression of the disease blurring of vision and problems controlling eye movement arise, including amongst others slowing of vertical saccades, where the downgaze is typically affected before the upgaze, and supranuclear ophthalmoplegia, difficulties opening or closing the eye lids.

Diagnosis

Early diagnosis is often difficult due to the vague signs and symptoms, which could also be associated with other neurodegenerative diseases such as Parkinson’s disease or Alzheimer’s disease. Difficulties moving the eyes are a hallmark of PSP, which makes the examination of eye movement a vital test for diagnosis.

Observable ocular motor disorders

  • Vertical supranuclear gaze palsy, initially vertically, first affecting saccades, then smooth pursuit (vertical/horizontal)
  • Combined vertical and horizontal gaze palsy with normal eye movements induced by the VOR

Reference

Litvan I, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology, 1996 Jul;47(1):1-9. PubMed