Progressive supranuclear palsy

Progressive supranuclear palsy

• aka Steele-Richardson-Olszewski syndrome
• a 'Parkinson Plus' syndrome

• postural instability and falls
  • patients tend to have a stiff, broad-based gait
• impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs)
• parkinsonism
  • bradykinesia is prominent
• cognitive impairment
  • primarily frontal lobe dysfunction

• MRI is the investigation of choice
  Progressive supranuclear palsy: MRI is the investigation of choice
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  Importance: 64
  • midbrain atrophy with relative preservation of the pons
  • characteristic 'hummingbird sign' (sagittal view)
  • dilated third ventricle
• DAT scan
  • may show reduced uptake but does not reliably distinguish PSP from Parkinson’s disease

• poor response to L-dopa