Search tips
Search criteria 


Logo of jnnpsycJournal of Neurology, Neurosurgery and PsychiatryVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
J Neurol Neurosurg Psychiatry. 1996 June; 60(6): 615–620.
PMCID: PMC1073943

Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study.


OBJECTIVE--To analyse the natural history of progressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) and clinical predictors of survival in 24 patients with PSP confirmed by necropsy, who fulfilled the NINDS criteria for a neuropathological diagnosis of typical PSP. METHODS--Patients were selected from the research and clinical files of seven medical centres involving tertiary centres of Austria, England, France, and the United States. Clinical features were analysed in detail. The patients' mean age at onset of PSP was 63 (range 45-73) years. RESULTS--The most frequent clinical features (occurring in at least 75% of the patients) were early postural instability and falls, vertical supranuclear palsy, akinetic-rigid predominant parkinsonian disorder characterised by symmetric bradykinesia and axial rigidity unrelieved by levodopa, pseudobulbar palsy, and frontal release signs. Occasionally, segmental dystonia or myoclonus were described, but neither aphasia nor alien limb syndrome was reported. Fractures occurred in 25% of the patients but were unrelated to the severity of the gait or to the presence of falls. Median survival time was 5.6 (range 2-16.6) years. Onset of falls during the first year, early dysphagia, and incontinence predicted a shorter survival time. Age at onset, sex, early onset of dementia, vertical supranuclear palsy, or axial rigidity had no effect on prognosis of survival. Pneumonia was the most common immediate cause of death. PSP was most often clinically misdiagnosed as Parkinson's disease. Errors in diagnosis suggest that PSP is underdiagnosed. CONCLUSION--Progressive onset of early postural instability with falls or supranuclear vertical palsy in the fifth decade, should suggest the diagnosis of PSP. Onset of falls during the first year are emphasised, as they could lead to an early diagnosis and influence the prognosis of patients with PSP. Whether appropriate treatment of the dysphagia could prolong the survival of PSP patients needs to be explored.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (1.2M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Nuwer MR. Progressive supranuclear palsy despite normal eye movements. Arch Neurol. 1981 Dec;38(12):784–784. [PubMed]
  • Dubas F, Gray F, Escourolle R. Maladie de Steele-Richardson-Olszewski sans ophtalmoplégie. Six cas anatomo-cliniques. Rev Neurol (Paris) 1983;139(6-7):407–416. [PubMed]
  • Masliah E, Hansen LA, Quijada S, DeTeresa R, Alford M, Kauss J, Terry R. Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy? Ann Neurol. 1991 Apr;29(4):389–396. [PubMed]
  • Matsuo H, Takashima H, Kishikawa M, Kinoshita I, Mori M, Tsujihata M, Nagataki S. Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry. 1991 May;54(5):397–400. [PMC free article] [PubMed]
  • Mizusawa H. ["Pure akinesia" and progressive supranuclear palsy]. No To Shinkei. 1993 Feb;45(2):113–118. [PubMed]
  • Dubinsky RM, Jankovic J. Progressive supranuclear palsy and a multi-infarct state. Neurology. 1987 Apr;37(4):570–576. [PubMed]
  • Will RG, Lees AJ, Gibb W, Barnard RO. A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome. J Neurol Neurosurg Psychiatry. 1988 Sep;51(9):1224–1227. [PMC free article] [PubMed]
  • Gibb WR, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain. 1989 Oct;112(Pt 5):1171–1192. [PubMed]
  • Foster NL, Gilman S, Berent S, Sima AA, D'Amato C, Koeppe RA, Hicks SP. Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities. J Neurol Neurosurg Psychiatry. 1992 Aug;55(8):707–713. [PMC free article] [PubMed]
  • Jankovic J, Rajput AH, Golbe LI, Goodman JC. What is it? Case 1, 1993: parkinsonism, dysautonomia, and ophthalmoparesis. Mov Disord. 1993 Oct;8(4):525–532. [PubMed]
  • Winikates J, Jankovic J. Vascular progressive supranuclear palsy. J Neural Transm Suppl. 1994;42:189–201. [PubMed]
  • Revesz T, Daniel SE, Lees AJ, Will RG. A case of progressive subcortical gliosis associated with deposition of abnormal prion protein (PrP) J Neurol Neurosurg Psychiatry. 1995 Jun;58(6):759–760. [PMC free article] [PubMed]
  • Daniel SE, de Bruin VM, Lees AJ. The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. Brain. 1995 Jun;118(Pt 3):759–770. [PubMed]
  • Gearing M, Olson DA, Watts RL, Mirra SS. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology. 1994 Jun;44(6):1015–1024. [PubMed]
  • Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, McKee A, Tabaton M, Litvan I. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1994 Nov;44(11):2015–2019. [PubMed]
  • Brusa A, Mancardi GL, Bugiani O. Progressive supranuclear palsy 1979: an overview. Ital J Neurol Sci. 1980 Oct;1(4):205–222. [PubMed]
  • De Bruin VM, Lees AJ. Subcortical neurofibrillary degeneration presenting as Steele-Richardson-Olszewski and other related syndromes: a review of 90 pathologically verified cases. Mov Disord. 1994 Jul;9(4):381–389. [PubMed]
  • Collins SJ, Ahlskog JE, Parisi JE, Maraganore DM. Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria. J Neurol Neurosurg Psychiatry. 1995 Feb;58(2):167–173. [PMC free article] [PubMed]
  • Colosimo C, Albanese A, Hughes AJ, de Bruin VM, Lees AJ. Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson's disease. Arch Neurol. 1995 Mar;52(3):294–298. [PubMed]
  • Litvan I, Hauw JJ, Bartko JJ, Lantos PL, Daniel SE, Horoupian DS, McKee A, Dickson D, Bancher C, Tabaton M, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol. 1996 Jan;55(1):97–105. [PubMed]
  • Tinetti ME, Speechley M. Prevention of falls among the elderly. N Engl J Med. 1989 Apr 20;320(16):1055–1059. [PubMed]
  • Tinetti ME, Inouye SK, Gill TM, Doucette JT. Shared risk factors for falls, incontinence, and functional dependence. Unifying the approach to geriatric syndromes. JAMA. 1995 May 3;273(17):1348–1353. [PubMed]
  • Pfaffenbach DD, Layton DD, Jr, Kearns TP. Ocular manifestations in progressive supranuclear palsy. Am J Ophthalmol. 1972 Dec;74(6):1179–1184. [PubMed]
  • Davis PH, Bergeron C, McLachlan DR. Atypical presentation of progressive supranuclear palsy. Ann Neurol. 1985 Apr;17(4):337–343. [PubMed]
  • Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1986 Jul;36(7):1005–1008. [PubMed]
  • Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy. Neurology. 1988 Jul;38(7):1031–1034. [PubMed]
  • de Bruin VM, Lees AJ, Daniel SE. Diffuse Lewy body disease presenting with supranuclear gaze palsy, parkinsonism, and dementia: a case report. Mov Disord. 1992 Oct;7(4):355–358. [PubMed]
  • Rinne JO, Lee MS, Thompson PD, Marsden CD. Corticobasal degeneration. A clinical study of 36 cases. Brain. 1994 Oct;117(Pt 5):1183–1196. [PubMed]
  • Wenning GK, Ben Shlomo Y, Magalhães M, Daniel SE, Quinn NP. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain. 1994 Aug;117(Pt 4):835–845. [PubMed]
  • Amarenco P, Roullet E, Hannoun L, Marteau R. Progressive supranuclear palsy as the sole manifestation of systemic Whipple's disease treated with pefloxacine. J Neurol Neurosurg Psychiatry. 1991 Dec;54(12):1121–1122. [PMC free article] [PubMed]
  • Quinn N. Parkinsonism--recognition and differential diagnosis. BMJ. 1995 Feb 18;310(6977):447–452. [PMC free article] [PubMed]
  • Lang AE, Bergeron C, Pollanen MS, Ashby P. Parietal Pick's disease mimicking cortical-basal ganglionic degeneration. Neurology. 1994 Aug;44(8):1436–1440. [PubMed]
  • Feinberg MJ, Knebl J, Tully J, Segall L. Aspiration and the elderly. Dysphagia. 1990;5(2):61–71. [PubMed]
  • Splaingard ML, Hutchins B, Sulton LD, Chaudhuri G. Aspiration in rehabilitation patients: videofluoroscopy vs bedside clinical assessment. Arch Phys Med Rehabil. 1988 Aug;69(8):637–640. [PubMed]
  • Ben-Shlomo Y, Marmot MG. Survival and cause of death in a cohort of patients with parkinsonism: possible clues to aetiology? J Neurol Neurosurg Psychiatry. 1995 Mar;58(3):293–299. [PMC free article] [PubMed]
  • Kircher T, Nelson J, Burdo H. The autopsy as a measure of accuracy of the death certificate. N Engl J Med. 1985 Nov 14;313(20):1263–1269. [PubMed]
  • Schonfeld SM, Golbe LI, Sage JI, Safer JN, Duvoisin RC. Computed tomographic findings in progressive supranuclear palsy: correlation with clinical grade. Mov Disord. 1987;2(4):263–278. [PubMed]
  • Savoiardo M, Strada L, Girotti F, D'Incerti L, Sberna M, Soliveri P, Balzarini L. MR imaging in progressive supranuclear palsy and Shy-Drager syndrome. J Comput Assist Tomogr. 1989 Jul-Aug;13(4):555–560. [PubMed]
  • Savoiardo M, Girotti F, Strada L, Ciceri E. Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders. J Neural Transm Suppl. 1994;42:93–110. [PubMed]
  • Burn DJ, Sawle GV, Brooks DJ. Differential diagnosis of Parkinson's disease, multiple system atrophy, and Steele-Richardson-Olszewski syndrome: discriminant analysis of striatal 18F-dopa PET data. J Neurol Neurosurg Psychiatry. 1994 Mar;57(3):278–284. [PMC free article] [PubMed]
  • Brooks DJ. PET studies in progressive supranuclear palsy. J Neural Transm Suppl. 1994;42:119–134. [PubMed]
  • Foster NL, Gilman S, Berent S, Sima AA, D'Amato C, Koeppe RA, Hicks SP. Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities. J Neurol Neurosurg Psychiatry. 1992 Aug;55(8):707–713. [PMC free article] [PubMed]
  • Ghika J, Tennis M, Growdon J, Hoffman E, Johnson K. Environment-driven responses in progressive supranuclear palsy. J Neurol Sci. 1995 May;130(1):104–111. [PubMed]
  • van Royen E, Verhoeff NF, Speelman JD, Wolters EC, Kuiper MA, Janssen AG. Multiple system atrophy and progressive supranuclear palsy. Diminished striatal D2 dopamine receptor activity demonstrated by 123I-IBZM single photon emission computed tomography. Arch Neurol. 1993 May;50(5):513–516. [PubMed]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group