CHORUS
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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
Copyright © 2013, Charles E. Kahn, Jr.
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