Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

  • E. Tolosa
  • F. Valldeoriola
  • M. J. Marti
Part of the Journal of Neural Transmission. Supplementa book series (NEURAL SUPPL, volume 42)


Progressive supranuclear palsy (PSP) is characterized clinically by supranuclear gaze palsy, neck dystonia, parkinsonism, pseudobulbar palsy, gait imbalance with frequent falls and frontal lobe-type dementia. In the advanced typical case, when supranuclear gaze palsy and other main features are present diagnosis is relatively easy. Diagnostic problems, though, are frequent in the early stages due to the variable clinical presentation and in those atypical cases in which gaze palsy does not develop or that present as a severe dementig disorder or as an isolated akinetic-rigid syndrome. In this review we summarize the clinical features of PSP and emphasize those aspects helpful in the differential diagnosis with Parkinsnon’s disease and other motor and cognitive disorders that can pose difficult diagnostic problems. Clinical diagnostic criteria are also discussed and modifications of those currently in used are proposed.


Alzheimer Disease Parkinson Disease Progressive Supranuclear Palsy Progressive Supranuclear Palsy Cervical Dystonia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Agid Y, Javoy-Agid F, Rubert M, et al (1986) Progressive supranuclear palsy: anatomo-clinical and biochemical considerations. Adv Neurol 45: 191–206.Google Scholar
  2. Albert ML, Feldman RG, Willis AL (1974) The “subcortical dementia” of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 37: 121–130.PubMedCrossRefGoogle Scholar
  3. Al-Din ASN, Al-Kurdi A, Al-Salem MK (1990) Autosomal recessive ataxias, slow eye movements, dementia and extrapyramidal disturbances. J Neurol Sci 96: 191–205.PubMedCrossRefGoogle Scholar
  4. Anzil AP (1969) Progressive supranuclear palsy: case report with pathological findings. Acta Neuropathol (Berl) 14: 72–76.CrossRefGoogle Scholar
  5. Behrman S, Carroll JD, Janota I, Matthews WB (1969) Progressive supranuclear palsy. Clinico-pathological study of four cases. Brain 92: 663–678.PubMedCrossRefGoogle Scholar
  6. Blin J, Baron JC, Dubois B, Pillon B, Cambon H, Cambier J, Agid Y (1990) Positron emission tomography study in progressive supranuclear palsy: brain hypometabolic pattern and clinicometabolic correlations. Arch Neurol 47: 747–752.PubMedCrossRefGoogle Scholar
  7. Brusa A, Mancardi GL, Bugiani O (1979) Progressive supranuclear palsy 1979: an overview. Italian J Neurol Sci i: 205–222.CrossRefGoogle Scholar
  8. Cambier J, Masson M, Viader F, Limodin J, Strube A (1985) Le syndrome frontal de la paralysie supranucléaire progressive. Rev Neurol (Paris) 141: 528–536.Google Scholar
  9. Cruz-Sánchez FF, Rossi ML, Cardozo A, Deacon P, Tolosa E (1992) Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer’s changes. Antigenic determinants that distinguish cortical and sub-cortical neurofibrillary tangles. Neurosci Lett 136: 43–46.PubMedCrossRefGoogle Scholar
  10. Davis PH, Bergeron C, McLachlan DR (1985) Atypical presentation of progressive supranuclear palsy. Ann Neurol 17: 337–343.PubMedCrossRefGoogle Scholar
  11. De Bruin VMS, Lees AJ, Daniel SE (1992) Diffuse Lewy body disease presenting with supranuclear gaze palsy, parkinsonism and dementia: a case report. Mov Disord 7: 335–358.CrossRefGoogle Scholar
  12. Dubas F, Gray F, Escourelle R (1983) Maladie de Steele-Richardson-Olszewski sans ophtalmoplégie: six cas anatomo-cliniques. Rev Neurol (Paris) 139: 407–416.Google Scholar
  13. Duvoisin RC (1987) The olivopontocerebellar atrophies. In: Marsden DC, Fahn S (eds) Movement disorders, vol 2. Butterworth, London, pp 249–271.Google Scholar
  14. Duvoisin RC, Golbe LI, Lepore FE (1987) Progressive supranuclear palsy. Can J Neurol Sci 14: 544–547.Google Scholar
  15. Fearnley JM, Revesz T, Brooks DJ, Frackowiak RSJ, Lees AJ (1991) Diffuse Lewy body disease presenting with a supranuclear gaze palsy. J Neurol Neurosurg Psychiatry 54: 159–161.PubMedCrossRefGoogle Scholar
  16. Fisk JD, Goodale MA, Burkhart G, Barnett HJM (1982) Progressive supranuclear palsy: the relationship between ocular motor dysfunction and psychological test performance. Neurology 32: 698–705.PubMedCrossRefGoogle Scholar
  17. Foster NL, Gilman S, Berent S, Sima AAF, D’Amato C, Koeppe RA, Hicks SP (1992) Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities. J Neurol Neurosurg Psychiatry 55: 707–713.PubMedCrossRefGoogle Scholar
  18. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112: 1171–1192.PubMedCrossRefGoogle Scholar
  19. Goffinet AM, De Voider AG, Gillain C, Rectem D, Bol A, Michel C, Cogneau M, Labar D, Laterre C (1989) Positron tomography demonstrates frontal lobe hypometabolism in progressive supranuclear palsy. Ann Neurol 25: 131–139.PubMedCrossRefGoogle Scholar
  20. Golbe LI, Davis PH (1993) Progressive supranuclear palsy. In: Jankovic J, Tolosa E (eds) Parkinson’s disease and movement disorders. William & Wilkins, Baltimore, pp 145–161.Google Scholar
  21. Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC (1988) Prevalence and natural history of progressive supranuclear palsy. Neurology 38: 1031–1034.PubMedCrossRefGoogle Scholar
  22. Grafman J, Litvan I, Gómez C, Chase TN (1990) Frontal lobe function in progressive supranuclear palsy. Arch Neurol 47: 553–558.PubMedCrossRefGoogle Scholar
  23. Hughes AJ, Lees AJ, Sterm GM (1991) Challenge tests to predict the dopaminergic response in untreated Parkinson’s disease. Neurology 41: 1723–1725.PubMedCrossRefGoogle Scholar
  24. Hughes AJ, Daniel SE, Kilford L, Lees AJ (1992) Accuracy of diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry 55: 181–184.PubMedCrossRefGoogle Scholar
  25. Jackson JA, Jankovic J, Ford J (1983) Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Ann Neurol 13: 273–278.PubMedCrossRefGoogle Scholar
  26. Jellinger K, Riederer P, Tomonaga M (1980) Progressive supranuclear palsy: clinico-pathological and biochemical studies. J Neural Transm [Suppl] 16: 111–128.Google Scholar
  27. Kish SJ, Chang LJ, Mirchandani L, Shannak K, Hornykiewicz O (1985) Progressive supranuclear palsy: relationship between extrapyramidal disturbances, dementia and brain neurotransmitter markers. Ann Neurol 18: 530–536.PubMedCrossRefGoogle Scholar
  28. Koepper A, Hans M (1976) Supranuclear ophtalmoplegia in olivopontocerebellar degeneration. Neurology 26: 764–768.CrossRefGoogle Scholar
  29. Kristensen MO (1985) Progressive supranuclear palsy — 20 years later. Acta Neurol Scand 71: 177–189.PubMedCrossRefGoogle Scholar
  30. Lees AJ (1987) The Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). In: Marsden DC, Fahn S (eds) Movement disorders, vol 2. Butterworth, London, pp 272–287.Google Scholar
  31. Léger JM, Girault JA, Bolgert F (1987) Deux cas de dystonie isolée d’un membre supérieur inauguant une maladie de Steele-Richardson-Olszewski. Rev Neurol (Paris) 143: 140–142.Google Scholar
  32. Lepore FE, Steele JC, Tilson G, Calne DB, Duvoisin RC, Lavine L, McDarby JV (1988) Supranuclear disturbances of ocular motility in Lytico-Bodig. Neurology 38: 1849–1853.PubMedCrossRefGoogle Scholar
  33. Lewis AJ, Gawel MJ (1990) Diffuse Lewy body disease with dementia and oculomotor dysfunction. Mov Disord 5: 143–147.PubMedCrossRefGoogle Scholar
  34. Lezak MD (1983) Neuropsychological assessment. Oxford University Press, New York.Google Scholar
  35. Litvan I, Grafman J, Gómez G, Chase TN (1989) Memory impairment in patients with progressive supranuclear palsy. Arch Neurol 46: 765–767.PubMedCrossRefGoogle Scholar
  36. Maher ER, Smith EM, Lees AJ (1985) Cognitive deficits in the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). J Neurol Neurosurg Psychiatry 48: 1234–1239.PubMedCrossRefGoogle Scholar
  37. Maher ER, Lees AJ (1986) The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 36: 1005–1008.PubMedCrossRefGoogle Scholar
  38. Masliah E, Hansen LA, Quijada S, DeTeresa R, Alford M, Kauss J, Terry R (1991) Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy. Ann Neurol 29: 389–396.PubMedCrossRefGoogle Scholar
  39. Mastaglia FL, Grainger KMR (1975) Internuclear ophtalmoplegia in progressive supranuclear palsy. J Neurol Sci 25: 303–308.PubMedCrossRefGoogle Scholar
  40. Matsuo H, Takashima H, Kishinawa M, Kinoshita I, Mori M, Tsujihata M, Nagataki S (1991) Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 54: 397–400.PubMedCrossRefGoogle Scholar
  41. Messert B, Van Nuis C (1966) A syndrome of paralysis of downward gaze, dysarthria, pseudobulbar palsy, rigidity of neck and trunk and dementia. J Nerv Ment Dis 143: 47–54.PubMedCrossRefGoogle Scholar
  42. Milder DG, Elliott CF, Evans WA (1989) Neuropathological findings in a case of coexistent progressive supranuclear palsy and Alzheimer’s disease. Clin Exp Neurol 20: 181–187.Google Scholar
  43. Morariu MA (1979) Progressive supranuclear palsy and normal-pressure hydrocephalus. Neurology 29: 1544–1546.PubMedCrossRefGoogle Scholar
  44. Mori H, Yoshimura M, Tomonaga M, Yamanouchi H (1986) Progressive supranuclear palsy with Lewy bodies. Acta Neuropathol 71: 344–346.PubMedCrossRefGoogle Scholar
  45. Neville BRG, Lake BD, Stephens R, Sanders MD (1973) A neurovisceral storage disease with vertical supranuclear ophtalmoplegia, and its relationship to Niemann-Pick disease. Brain 96: 97–120.PubMedCrossRefGoogle Scholar
  46. Olson DA, Gearing M, Watts RL, Mirra SS (1992) Clinical-pathological heterogeneity in progressive supranuclear palsy. Ann Neurol 32: 244 (abstract).Google Scholar
  47. Perkin GD, Lees AJ, Stern GM, Kocek RS (1978) Problems in the diagnosis of progressive supranuclear palsy. Can J Neurol Sci 5: 167–173.Google Scholar
  48. Pfaffenbach DD, Layton OD, Kearns TP (1972) Ocular manifestations in progressive supranuclear palsy. Am J Ophtal 74: 1174–1184.Google Scholar
  49. Pillon B, Dubois B, Lhermitte F, Agid Y (1986) Heterogeneity of cognitive impairment in progressive supranuclear palsy, Parkinson’s disease and Alzheimer’s disease. Neurology 36: 1179–1185.PubMedCrossRefGoogle Scholar
  50. Pillon B, Dubois B (1992) Cognitive and behavioural impairments. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy. Clinical and research approaches. Oxford University Press, New York, pp 223–239.Google Scholar
  51. Pirozzolo FJ, Jankovic J, Levy JK (1986) Progressive supranuclear palsy: are cognitive and motor deficits related. Neurology 36 [Suppl]1: 308.Google Scholar
  52. Probst A (1977) Dégénérescence neurofibrillaire sous-corticale sénile avec présence de tubules contournés et de filaments droits: form atypique de la paralysie supranu-cléaire progressive. Rev Neurol (Paris) 133: 417–428.Google Scholar
  53. Rafal RD, Friedman JH (1987) Limb dystonia in progressive supranuclear palsy. Neurology 37: 1546–1549.PubMedCrossRefGoogle Scholar
  54. Rafal RD, Posner MI, Friedman JH, Inhoff AW, Bernstein E (1988) Orienting of visual attention in progressive supranuclear palsy. Brain 111: 267–280.PubMedCrossRefGoogle Scholar
  55. Rascol O, Clanet M, Monstastruc JL, Simonetta M, Soulier-Esteve MJ, Doyon B, Rascol A (1989) Abnormal ocular movements in Parkinson’s disease. Brain 112: 1193–1214.PubMedCrossRefGoogle Scholar
  56. Oberts AH (1969) Brain damage in boxers: a study of prevalence of traumatic encephalopathy among ex-professional boxers. London, Pitman.Google Scholar
  57. Rosenberg B, Nyhan WL, Day C, Shore P (1976) Autosomal dominance strionigral degeneration. Neurology 26: 703–714.PubMedCrossRefGoogle Scholar
  58. Ross-Russell R (1980) Supranuclear palsy of eyelid closure. Brain 103: 71–82.CrossRefGoogle Scholar
  59. Sasaki S, Maruyama S, Toyoda Ch (1991) A case of progressive supranuclear palsy with widespread senile plaques. J Neurol 238: 345–348.PubMedCrossRefGoogle Scholar
  60. Schwarz J, Tatsch K, Arnold G, Gasser T, Trenkwalder C, Kirsch CM, Oertel WH (1992) 123-Iodobenzamide-SPECT predicts dopaminergic responsiveness in patients with de novo parkinsonism. Neurology 42: 556–561.PubMedCrossRefGoogle Scholar
  61. Sonies BC (1992) Swallowing and speech disturbances. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy. Clinical and research approaches. Oxford University Press, New York, pp 240–253.Google Scholar
  62. Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. A heterogeneous degeneration involving the brainstem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10: 333–358.PubMedCrossRefGoogle Scholar
  63. Steele JC (1975) Progressive supranuclear palsy. In: Vinken PJ, Bruyn GW, deJong JMB (eds) Handbook of clinical neurology, vol 22. System disorders and atrophies, part II. North-Holland, Amsterdam, pp 217–229.Google Scholar
  64. Steele JC, Guzman T (1987) Observations about amyotrophic lateral sclerosis and the Parkinsonism-Dementia complex of Guam with regard to epidemiology and etiology. Can J Neurol Sci 14: 358–362.PubMedGoogle Scholar
  65. Tripp JH, Lake BD, Young E, et al (1977) Juvenile Gaucher’s disease with horizontal gaze palsy in 3 siblings. J Neurol Neurosurg Psychiatry 40: 470–478.PubMedCrossRefGoogle Scholar
  66. Troost BT, Daroff RB (1977) The ocular motor defects in progressive supranuclear palsy. Ann Neurol 2: 297–403.CrossRefGoogle Scholar
  67. Yamamoto T, Kawamura J, Hashimoto S, Nakamura M, Iwamoto H, Kobashi Y, Ichijima K (1990) Pallido-nigro-luysian atrophy, progressive supranuclear palsy and adult onset Hallervorden-Spatz disease: a case of akinesia as a predominant feature of parkinsonism. J Neurol Sci 101: 98–106.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 1994

Authors and Affiliations

  • E. Tolosa
    • 1
    • 2
  • F. Valldeoriola
    • 1
  • M. J. Marti
    • 1
  1. 1.Neurology Department, Hospital Clinic, Faculty of MedicineUniversity of BarcelonaSpain
  2. 2.Neurology DepartmentHospital ClinicBarcelonaSpain

Personalised recommendations