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

Progressive supranuclear palsy (or Steele–Richardson–Olszewski disease)
Chapter:
Progressive supranuclear palsy (or Steele–Richardson–Olszewski disease)
Author(s):

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

DOI:
10.1093/med/9780192619112.003.0359
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date: 29 May 2020

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.

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