Review
Classic diseases revisited
Progressive supranuclear palsy
(Steele-Richardson-Olszewski disease)
Huw R Morris, Nicholas W Wood, Andrew J Lees
National Hospital
for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
Correspondence to: Dr AJ Lees
Accepted 14 May 1999
Progressive supranuclear palsy is a neurodegenerative disease
which affects the brainstem and basal ganglia. Patients present with
disturbance of balance, a disorder of downward gaze and
L-DOPA-unresponsive parkinsonism and usually develop progressive
dysphagia and dysarthria leading to death from the complications of
immobility and aspiration. Treatment remains largely supportive but,
potentially, treatments based on cholinergic therapy may be useful. As
in Alzheimer's disease, the neuronal degeneration is associated with
the deposition of hyperphosphorylated tau protein as neurofibrillary
tangles but there are important distinctions between the two diseases. Evidence from familial fronto-temporal dementia with parkinsonism linked to chromosome 17 suggests that tau protein deposition is a
primary pathogenic event in some neurodegenerative diseases. The
understanding of the mechanism of tau deposition in progressive supranuclear palsy is likely to be of importance in unravelling its aetiology.
Keywords: progressive supranuclear palsy; Steele-Richardson-Olszewski disease; tau protein
© 1999 by The Fellowship of Postgraduate Medicine
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