Progressive Supranuclear Palsy

Excerpt

Progressive supranuclear palsy (PSP) is an uncommon neurological disorder that affects movement, gait, balance, speech, swallowing, vision, eye movements, mood, behavior, and cognition. Steele, Richardson, and Olszewski described the syndrome in 1964 as an unusual constellation of supranuclear gaze palsy, progressive axial rigidity, pseudobulbar palsy, and mild dementia. This disease is now a well-recognized atypical parkinsonian syndrome (or Parkinson-plus disorder).

In 1972, Steele predicted clinical variants of the syndrome were likely to occur as the disease affected different brainstem nuclei at different times and to different degrees. Since then, different phenotypes have been characterized and linked to the severity of abnormal tau accumulation and neuronal loss in various brain regions. Different progressive supranuclear palsies, regardless of clinical characteristics, share similar neuropathologic features.