Walsh & Hoyt: Steele-Richardson-Olszewski Syndrome (Progressive Supranuclear Palsy)

In 1964, Steele, Richardson, and Olszewski reported a group of patients with a late-onset neurodegenerative disease characterized by supranuclear gaze palsy, pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and trunk, and dementia. At postmortem examination, there was neurofibrillary degeneration with loss of neurons and gliosis affecting the striatum, pallidum, subthalamic nucleus, substantia nigra, locus ceruleus, tectum, tegmentum, basis pontis, and certain cranial nerve nuclei. Progressive supranuclear palsy (PSP) is now recognized to be part of a spectrum of neurodegenerative conditions including corticobasal degeneration and frontotemporal dementia associated with the abnormal aggregation of tau protein, the so called tauopathies.