(Steele-Richardson-Olszewski Syndrome) and Clinical Predictors of Survival: a Clinicopathological Study
3'ournal ofNeurology, Neurosurgery, and Psychiatry 1996;61:615-620 615 Natural history of progressive supranuclear palsy J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.60.6.615 on 1 June 1996. Downloaded from (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study I Litvan, C A Mangone, A McKee, M Veiny, A Parsa, K Jellinger, L D'Olhaberriague, K Ray Chaudhuri, R K B Pearce Abstract Keywords: progressive supranuclear palsy; natural his- Objective-To analyse the natural history tory; survival ofprogressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) Progressive supranuclear palsy (PSP or Steele- and clinical predictors of survival in 24 Richardson-Olszewski syndrome) causes patients with PSP confirmed by necropsy, postural instability, supranuclear vertical oph- who fulfilled the NINDS criteria for a thalmoplegia, parkinsonism unresponsive to neuropathological diagnosis of typical levodopa, pseudobulbar palsy, and mild PSP. dementia.' 2 However, patients with PSP with- Methods-Patients were selected from the out ophthalmoplegia or dementia, or present- research and clinical files of seven med- ing only with dementia or akinesia, have also ical centres involving tertiary centres of been reported.3-7 Austria, England, France, and the United In the absence of laboratory markers for the States. Clinical features were analysed in diagnosis of PSP, neuropathological examina- Neuroepidemiology detail. The patients' mean age at onset of tion remains the "gold standard"
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