Chapter

Progressive supranuclear palsy (or Steele–Richardson–Olszewski disease)

Ivan Donaldson, C. David Marsden, Susanne A. Schneider and Kailash P. Bhatia

in Marsden's Book of Movement Disorders

Published on behalf of Oxford University Press

ISBN: 9780192619112
Published online January 2012 | e-ISBN: 9780199698103 | DOI: http://dx.doi.org/10.1093/med/9780192619112.003.0359
Progressive supranuclear palsy (or Steele–Richardson–Olszewski disease)

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PSP or Steele–Richardson–Olszewski disease (SROD) is an idiopathic, sporadic disease, with onset in middle or late life. It is characterized clinically by a supranuclear gaze palsy, especially for vertical gaze and particularly downgaze, neck rigidity, and dystonia, a predominantly axial akinetic–rigid parkinsonian syndrome, a pseudobulbar palsy, loss of equilibrium with frequent falls, and a frontal lobe-type dementia. The condition is progressive and leads to death on average about 6 years from the onset.

Neuropathologically, the condition is characterized by widespread neuronal loss, glial changes, and the presence of characteristic NFT, mainly in diencephalic and brainstem nuclei and tau-positive filamentous inclusions in both neurons and glia.

Chapter.  26649 words.  Illustrated.

Subjects: Neurology

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