Skip to main content
AAN.com
Views And Reviews
July 1, 1996

Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)
Report of the NINDS-SPSP International Workshop*

July 1996 issue
47 (1) 1-9

Abstract

To improve the specificity and sensitivity of the clinical diagnosis of progressive supranuclear palsy (PSP, Steele-Richardson-Olszewski syndrome), the National Institute of Neurological Disorders and Stroke (NINDS) and the Society for PSP, Inc. (SPSP) sponsored an international workshop to develop an accurate and universally accepted set of criteria for this disorder. The NINDS-SPSP criteria, which were formulated from an extensive review of the literature, comparison with other previously published sets of criteria, and the consensus of experts, were validated on a clinical data set from autopsy-confirmed cases of PSP. The criteria specify three degrees of diagnostic certainty: possible PSP, probable PSP, and definite PSP. Possible PSP requires the presence of a gradually progressive disorder with onset at age 40 or later, either vertical supranuclear gaze palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of onset, as well as no evidence of other diseases that could explain these features. Probable PSP requires vertical supranuclear gaze palsy, prominent postural instability, and falls in the first year of onset, as well as the other features of possible PSP. Definite PSP requires a history of probable or possible PSP and histopathologic evidence of typical PSP. Criteria that support the diagnosis of PSP, and that exclude diseases often confused with PSP, are presented. The criteria for probable PSP are highly specific, making them suitable for therapeutic, analytic epidemiologic, and biologic studies, but not very sensitive. The criteria for possible PSP are substantially sensitive, making them suitable for descriptive epidemiologic studies, but less specific. An appendix provides guidelines for diagnosing and monitoring clinical disability in PSP.
NEUROLOGY 1996;47: 1-9

Get full access to this article

View all available purchase options and get full access to this article.

REFERENCES

1.
Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. Arch Neurol 1964;10:333-359.
2.
Steele JC. Progressive supranuclear palsy. Brain 1972;95:693-704.
3.
Steele JC. Introduction. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:3-14.
4.
Posey WC. Analysis of the upward movements of the eyes. Ann Ophthalmol 1904;13:523-529.
5.
Spiller WG. The importance in clinical diagnosis of paralysis of associated movements of the eyeballs (Blick-Laehmung), especially of upward and downward associated movements. J Nerv Ment Dis 1905;32:417-531.
6.
Lees AJ. Foreward. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:vi-x.
7.
Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy. Neurology 1988;38:1031-1034.
8.
Golbe LI. The epidemiology of PSP. J Neural Transm Suppl 1994;42:263-273.
9.
Golbe LI. Epidemiology. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:33-43.
10.
Hauw J-J, Daniel SE, Dickson D, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1994;44:2015-2019.
11.
Litvan I, Hauw J-J, Bartko JJ, et al. Validity and reliability of the neuropathologic preliminary criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol 1996;55:97-105.
12.
Probst A, Langui D, Lautenschlager C, Ulrich J, Brion JP, Anderton BH. Progressive supranuclear palsy: extensive neuropil threads in addition to neurofibrillary tangles. Very similar antigenicity of subcortical neuronal pathology in progressive supranuclear palsy and Alzheimer's disease. Acta Neuropathol (Berl) 1988;77:61-68.
13.
Jellinger KA, Bancher C. Neuropathology. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:44-88.
14.
Lantos PL. The neuropathology of progressive supranuclear palsy. J Neural Transm Suppl 1994;42:137-152.
15.
Hauw J-J, Verny M, Mokhtari K, et al. Neuropathologic diagnostic criteria of Steele-Richardson-Olszewski disease (progressive supranuclear palsy) for brain banking purposes. In: Cruz-Sanchez FF, Ravid R, Cuzner ML, eds. Neuropathological diagnostic criteria for brain banking. Amsterdam: IOS Press, 1995:80-88.
16.
Gearing M, Olson DA, Watts RL, Mirra SS. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology 1994;44:1015-1024.
17.
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;51:1224-1227.
18.
Gibb WR, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989;112:1171-1192.
19.
Dubinsky RM, Jankovic J. Progressive supranuclear palsy and a multi-infarct state. Neurology 1987;37:570-576.
20.
Winikates J, Jankovic J. Vascular progressive supranuclear palsy. J Neural Transm Suppl 1994;42:189-201.
21.
Fearnley JM, Revesz T, Brooks DJ, Frackowiak RS, Lees AJ. Diffuse Lewy body disease presenting with a supranuclear gaze palsy. J Neurol Neurosurg Psychiatry 1991;54:159-161.
22.
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;7:355-358.
23.
Foster NL, Gilman S, Berent S, et al. Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities. J Neurol Neurosurg Psychiatry 1992;55:707-713.
24.
Jankovic J, Rajput AH, Golbe LI, Goodman JC. What is it? Case 1, 1993: parkinsonism, dysautonomia, and ophthalmoparesis. Mov Disord 1993;8:525-532.
25.
Revesz T, Daniel SE, Lees AJ, Will RG. A case of progressive subcortical gliosis associated with deposition of abnormal prion protein. J Neurol Neurosurg Psychiatry 1995; in press.
26.
Litvan I, Agid Y, Jankovic J, et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology 1996;46:922-930.
27.
Jackson JA, Jankovic J, Ford J. Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Ann Neurol 1983;13:273-278.
28.
Boller F, Lopez OL, Moossy J. Diagnosis of dementia: clinicopathologic correlations. Neurology 1989;39:76-79.
29.
Rajput AH, Rozdilsky B, Rajput A. Accuracy of clinical diagnosis in parkinsonism-a prospective study. Can J Neurol Sci 1991;18:275-278.
30.
Hughes AJ, Daniel SE, Kilford L, Lees AJ. Accuracy of clinical diagnosis of idiopathic Parkinson's disease: a clinicopathological study of 100 cases. J Neurol Neurosurg Psychiatry 1992;55:181-184.
31.
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 46-1993. A 75-year-old man with right-sided rigidity, dysarthria, and abnormal gait. N Engl J Med 1993;329:1560-1567.
32.
Litvan I, Chase TN. Traditional and experimental therapeutic approaches. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:254-269.
33.
Lees AJ. The Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). In: Marsden CD, Fahn S, eds. Movement disorders 2. London: Butterworths, 1987:272-287.
34.
Blin J, Baron JC, Dubois B, et al. Positron emission tomography study in progressive supranuclear palsy. Brain hypometabolic pattern and clinicometabolic correlations. Arch Neurol 1990;47:747-752.
35.
Golbe LI, Davis PH. Progressive supranuclear palsy. In: Jankovic J, Tolosa E, eds. Parkinson's disease and movement disorders. Baltimore: Williams and Wilkins, 1993:145-161.
36.
Duvoisin RC. Clinical diagnosis. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:15-33.
37.
Tolosa E, Valldeoriola F, Marti MJ. Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). J Neural Transm Suppl 1994;42:15-31.
38.
Collins SJ, Ahlskog JE, Parisi JE, Maraganore DM. Progressive supranuclear palsy: neuropathologically based diagnostic criteria. J Neurol Neurosurg Psychiatry 1995;58:167-173.
39.
Brusa A, Mancardi GL, Bugiani O. Progressive supranuclear palsy 1979: an overview. Ital J Neurol Sci 1980;4:205-222.
40.
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;9:381-389.
41.
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;52:294-298.
42.
Litvan I, Mangone CA, McKee A, et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathologic study. J Neurol Neurosurg Psychiatry 1996 (in press).
43.
Troost B. Neuro-ophthalmological aspects. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:44-88.
44.
Troost B, Daroff R. The ocular motor defects in progressive supranuclear palsy. Ann Neurol 1977;2:397-403.
45.
Cardoso FEC, Jankovic J. Progressive supranuclear palsy. In: Calne DB, ed. Neurodegenerative disorders. Philadelphia: W.B. Saunders, 1994:769-786.
46.
Brown J, Lantos P, Stratton M, Roques P, Rossor M. Familial progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1993;56:473-476.
47.
Tetrud JW, Golbe LI, Forno LS, Farmer PM. Autopsy-proven progressive supranuclear palsy in two siblings. Neurology 1996;46:931-934.
48.
Masliah E, Hansen L, Quijada S, et al. Late onset dementia with argyrophylic grains and subcortical tangles or atypical progressive supranuclear palsy? Ann Neurol 1991;29:389-396.
49.
Nuwer MR. Progressive supranuclear palsy despite normal eye movements. Arch Neurol 1981;38:784.
50.
Dubas F, Gray F, Escourolle R. Steele-Richardson-Olszewski disease without ophthalmoplegia. Six clinico-anatomic cases. Rev Neurol (Paris) 1983;139:407-416.
51.
Pfaffenbach DD, Layton DDJ, Kearns TP. Ocular manifestations in progressive supranuclear palsy. Am J Ophthalmol 1972;74:1179-1184.
52.
Davis PH, Bergeron C, McLachlan DR. Atypical presentation of progressive supranuclear palsy. Ann Neurol 1985;17:337-343.
53.
Matsuo H, Takashima H, Kishikawa M, et al. Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1991;54:397-400.
54.
Mizusawa H, Mochizuki A, Ohkoshi N, Yoshizawa K, Kanazawa I, Imai H. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993;60:618-621.
55.
Riley DE, Fogt N, Leigh RJ. The syndrome of `pure akinesia' and its relationship to progressive supranuclear palsy. Neurology 1994;44:1025-1029.
56.
Daniel SE, De Bruin VMS, Lees AJ. The clinical and pathological spcctrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy)-a reappraisal. Brain 1995; in press.
57.
Verny M, Jellinger KA, Hauw J-J, Bancher C, Litvan I, Agid Y. Progressive supranuclear palsy. A clinicopathological study of 21 cases. Acta Neuropathol, in press.
58.
Albert ML, Feldman RG, Willis AL. The `subcortical dementia' of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1974;37:121-130.
59.
Cambier J, Masson M, Viader F, Limodin J, Strube A. Frontal syndrome of progressive supranuclear palsy. Rev Neurol (Paris) 1985;141:528-536.
60.
Grafman J, Litvan I, Gomez C, Chase TN. Frontal lobe function in progressive supranuclear palsy. Arch Neurol 1990;47:553-558.
61.
Pillon B, Dubois B. Cognitive and behavioral impairments. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:223-240.
62.
Litvan I. Cognitive disturbances in progressive supranuclear palsy. J Neural Transm Suppl 1994;42:69-78.
63.
Ghika J, Tennis M, Growdon J, Hoffman E, Johnson K. Environment-driven responses in progressive supranuclear palsy. J Neurol Sci 1995;130:104-111.
64.
D'Antona R, Baron JC, Samson Y, et al. Subcortical dementia. Frontal cortex hypometabolism detected by positron tomography in patients with progressive supranuclear palsy. Brain 1985;108:785-799.
65.
Baron JC, Maziere B, Loch C, et al. Loss of striatal [sup 76 Br] bromospiperone binding sites demonstrated by positron tomography in progressive supranuclear palsy. J Cereb Blood Flow Metab 1986;6:131-136.
66.
Foster NL, Gilman S, Berent S, Morin EM, Brown MB, Koeppe RA. Cerebral hypometabolism in progressive supranuclear palsy studied with positron emission tomography. Ann Neurol 1988;24:399-406.
67.
Leenders KL, Frackowiak RS, Lees AJ. Steele-Richardson-Olszewski syndrome. Brain energy metabolism, blood flow and fluorodopa uptake measured by positron emission tomography. Brain 1988;111:615-630.
68.
Goffinet AM, De Volder AG, Gillain C, et al. Positron tomography demonstrates frontal lobe hypometabolism in progressive supranuclear palsy. Ann Neurol 1989;25:131-139.
69.
Brooks DJ, Ibanez V, Sawle GV, et al. Differing patterns of striatal sup 18 F-dopa uptake in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy. Ann Neurol 1990;28:547-555.
70.
Brooks DJ, Ibanez V, Sawle GV, et al. Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with sup 11 C-raclopride and positron emission tomography. Ann Neurol 1992;31:184-192.
71.
Burn DJ, Sawle GV, Brooks DJ. Differential diagnosis of Parkinson's disease, multiple system atrophy, and Steele-Richardson-Olszewski syndrome: discriminant analysis of striatal sup 18 F-dopa PET data. J Neurol Neurosurg Psychiatry 1994;57:278-284.
72.
Blin J, Horwitz B, Baron J, Agid Y. Does frontal cortex hypometabolism in progressive supranuclear palsy result from subcortical dysfunction? Eur J Neurol 1995;1:221-228.
73.
Pierrot-Deseilligny C, Turell E, Penet C, et al. Increased wave P300 latency in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1989;52:656-658.
74.
Johnson RJ, Litvan I, Grafman J. Progressive supranuclear palsy: altered sensory processing leads to degraded cognition. Neurology 1991;41:1257-1262.
75.
Johnson RJ. Event-related brain potentials. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:122-154.
76.
Vidailhet M, Rothwell JC, Thompson PD, Lees AJ, Marsden CD. The auditory startle response in the Steele-Richardson-Olszewski syndrome and Parkinson's disease. Brain 1992;115:1181-1192.
77.
Rothwell JC, Vidailhet M, Thompson PD, Lees AJ, Marsden CD. The auditory startle response in progressive supranuclear palsy. J Neural Transm Suppl 1994;42:43-50.
78.
Vidailhet M, Rivaud S, Gouider-Khouja N, et al. Eye movements in parkinsonian syndromes. Ann Neurol 1994;35:420-426.
79.
Masucci EF, Borts FT, Smirniotopoulos JG, Kurtzke JF, Schellinger D. Thin-section CT of midbrain abnormalities in progressive supranuclear palsy. AJNR Am J Neuroradiol 1985;6:767-772.
80.
Schonfeld SM, Golbe LI, Sage JI, Safer JN, Duvoisin RC. Computed tomographic findings in progressive supranuclear palsy: correlation with clinical grade. Mov Disord 1987;2:263-278.
81.
Savoiardo M, Girotti F, Strada L, Cieri E. Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders. J Neural Transm Suppl 1994;42:93-110.
82.
Laffont F, Autret A, Minz M, et al. Polygraphic study of nocturnal sleep in three degenerative diseases: ALS, oligo-ponto-cerebellar atrophy, and progressive supranuclear palsy. Waking Sleeping 1979;3:17-30.
83.
Perret JL, Jouvet M. Sleep study of progressive supranuclear paralysis. Electroencephalogr Clin Neurophysiol 1980;49:323-329.
84.
Aldrich MS, Foster NL, White RF, Bluemlein L, Prokopowicz G. Sleep abnormalities in progressive supranuclear palsy. Ann Neurol 1989;25:577-581.
85.
Aldrich MS. Sleep disturbances. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy: clinical and research approaches. New York: Oxford University Press, 1992:169-183.
86.
Fahn S, Elton RL, members of the UPDRS Development Committee. Unified Parkinson's disease rating scale. In: Fahn S, Marsden CD, Calne DB, Lieberman A, eds. Recent developments in Parkinson' disease, Vol. II. Florham Park, NJ: Macmillan Health Care Information, 1987:153-163.
87.
Grafman J, Litvan I, Stark M. Neuropsychological features of progressive supranuclear palsy. Brain Cogn 1995;2:311-320.
88.
Lezak MD. Neuropsychological assessment. 3rd ed. New York: Oxford University Press, 1995.

Information & Authors

Information

Published In

Neurology®
Volume 47Number 1July 1996
Pages: 1-9
PubMed: 8710059

Publication History

Published online: July 1, 1996
Published in print: July 1996

Permissions

Request permissions for this article.

Authors

Affiliations & Disclosures

*For Workshop participants and affiliations, see page 7.
Received November 27, 1995. Accepted in final form December 8, 1995.
Address correspondence and reprint requests to Dr. Irene Litvan, Federal Building, Room 714, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-9130.

Metrics & Citations

Metrics

Citations

Download Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.

Cited By
  1. Cerebrospinal fluid level of proNGF as potential diagnostic biomarker in patients with frontotemporal dementia, Frontiers in Aging Neuroscience, 15, (2024).https://doi.org/10.3389/fnagi.2023.1298307
    Crossref
  2. Visual fixation suppression of caloric nystagmus in progressive supranuclear palsy – A comparison with Parkinson’s disease, Journal of Vestibular Research, 33, 6, (385-401), (2024).https://doi.org/10.3233/VES-210147
    Crossref
  3. Neuropathological changes associated with aberrant cerebrospinal fluid p-tau181 and Aβ42 in Alzheimer’s disease and other neurodegenerative diseases, Acta Neuropathologica Communications, 12, 1, (2024).https://doi.org/10.1186/s40478-024-01758-3
    Crossref
  4. Low Dose Amantadine and Escitalopram in Progressive Supranuclear Palsy and Multiple System Atrophy, Annals of Neurosciences, (2024).https://doi.org/10.1177/09727531231222700
    Crossref
  5. Primitive reflexes and dementia in older adults: a meta‐analysis of observational and cohort studies, Psychogeriatrics, (2024).https://doi.org/10.1111/psyg.13098
    Crossref
  6. Staging of progressive supranuclear palsy-Richardson syndrome using MRI brain charts for the human lifespan, Brain Communications, 6, 2, (2024).https://doi.org/10.1093/braincomms/fcae055
    Crossref
  7. Neuroinflammation following anti-parkinsonian drugs in early Parkinson’s disease: a longitudinal PET study, Scientific Reports, 14, 1, (2024).https://doi.org/10.1038/s41598-024-55233-z
    Crossref
  8. Patients with Neurodegenerative Proteinopathies Exhibit Altered Tryptophan Metabolism in the Serum and Cerebrospinal Fluid, ACS Chemical Neuroscience, 15, 3, (582-592), (2024).https://doi.org/10.1021/acschemneuro.3c00611
    Crossref
  9. Correlation between clinical and neuropathological subtypes of progressive supranuclear palsy, Parkinsonism & Related Disorders, (106076), (2024).https://doi.org/10.1016/j.parkreldis.2024.106076
    Crossref
  10. Putaminal T1/T2-weighted ratio is increased in PSP compared to PD and healthy controls, a multi-cohort study, Parkinsonism & Related Disorders, 121, (106047), (2024).https://doi.org/10.1016/j.parkreldis.2024.106047
    Crossref
  11. See more
Loading...

View Options

Get Access

Login options

Check if you have access through your login credentials or your institution to get full access on this article.

Personal login Institutional Login
Purchase Options

Purchase this article to get full access to it.

Purchase Access, $39 for 24hr of access

View options

Full Text

View Full Text

Full Text HTML

View Full Text HTML

Media

Figures

Other

Tables

Share

Share

Share article link

Share