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Collaborative Hypertext of Radiology

Nervous system



progressive supranuclear palsy (Steele-Richardson-Olszewski syndome)

  • about 8% of patients with clinical diagnosis of Parkinson disease
  • median age of onset = 64 yrs (range 50-77)

  • core clinical syndrome
    • supranuclear ophthalmoplegia
    • akinesia (presents as frequent falls)
    • rigidity
    • nuchal dystonia
    • pseudobulbar palsy (dysarthria, dysphagia)
    • cognitive abnormality leading to dementia

  • imaging
    • atrophy of midbrain (substantia nigra) and pontine tegmentum with abnormally enlarged prepontine cistern
    • variable atrophy of globus pallidus
    • some cases show atrophy of fronto-temporal regions with enlarged lateral ventricles and third ventricle
    • some cases show atrophy of motor cortex

  • neuropathological changes
    • neuronal loss and gliosis with neurofibrillary tangles
      • oculomotor nucleus
      • substantia nigra
      • basal ganglia

  • DDx
    • cortico-basal degeneration
    • multiple system atrophy
      • striatonigral degeneration
      • Shy-Drager syndrome
      • olivo-ponto-cerebellar atrophy
    • arteriosclerotic disease with lacunar infarction
    • idiopathic Parkinson disease

James Lowe, DM BM BS MRCPath  -  13 November 1997
Last updated:  1 October 2013


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reflex sympathetic dystrophy


Copyright © 2013, Charles E. Kahn, Jr.