Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

Report of the NINDS-SPSP International Workshop

I. Litvan*, Y. Agid, D. Calne, G. Campbell, B. Dubois, R. C. Duvoisin, C. G. Goetz, L. I. Golbe, J. Grafman, J. H. Growdon, M. Hallett, J. Jankovic, N. P. Quinn, E. Tolosa, D. S. Zee

*Corresponding author for this work

Research output: Contribution to journalReview article

1728 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1-9
Number of pages9
JournalNeurology
Volume47
Issue number1
DOIs
StatePublished - Jan 1 1996

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National Institute of Neurological Disorders and Stroke
Progressive Supranuclear Palsy
Education
Research
Primary Spontaneous Pneumothorax
Paralysis
Epidemiologic Studies

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Litvan, I. ; Agid, Y. ; Calne, D. ; Campbell, G. ; Dubois, B. ; Duvoisin, R. C. ; Goetz, C. G. ; Golbe, L. I. ; Grafman, J. ; Growdon, J. H. ; Hallett, M. ; Jankovic, J. ; Quinn, N. P. ; Tolosa, E. ; Zee, D. S. / Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) : Report of the NINDS-SPSP International Workshop. In: Neurology. 1996 ; Vol. 47, No. 1. pp. 1-9.
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title = "Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of the NINDS-SPSP International Workshop",
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.",
author = "I. Litvan and Y. Agid and D. Calne and G. Campbell and B. Dubois and Duvoisin, {R. C.} and Goetz, {C. G.} and Golbe, {L. I.} and J. Grafman and Growdon, {J. H.} and M. Hallett and J. Jankovic and Quinn, {N. P.} and E. Tolosa and Zee, {D. S.}",
year = "1996",
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Litvan, I, Agid, Y, Calne, D, Campbell, G, Dubois, B, Duvoisin, RC, Goetz, CG, Golbe, LI, Grafman, J, Growdon, JH, Hallett, M, Jankovic, J, Quinn, NP, Tolosa, E & Zee, DS 1996, 'Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of the NINDS-SPSP International Workshop', Neurology, vol. 47, no. 1, pp. 1-9. https://doi.org/10.1212/WNL.47.1.1

Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) : Report of the NINDS-SPSP International Workshop. / Litvan, I.; Agid, Y.; Calne, D.; Campbell, G.; Dubois, B.; Duvoisin, R. C.; Goetz, C. G.; Golbe, L. I.; Grafman, J.; Growdon, J. H.; Hallett, M.; Jankovic, J.; Quinn, N. P.; Tolosa, E.; Zee, D. S.

In: Neurology, Vol. 47, No. 1, 01.01.1996, p. 1-9.

Research output: Contribution to journalReview article

TY - JOUR

T1 - Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

T2 - Report of the NINDS-SPSP International Workshop

AU - Litvan, I.

AU - Agid, Y.

AU - Calne, D.

AU - Campbell, G.

AU - Dubois, B.

AU - Duvoisin, R. C.

AU - Goetz, C. G.

AU - Golbe, L. I.

AU - Grafman, J.

AU - Growdon, J. H.

AU - Hallett, M.

AU - Jankovic, J.

AU - Quinn, N. P.

AU - Tolosa, E.

AU - Zee, D. S.

PY - 1996/1/1

Y1 - 1996/1/1

N2 - 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.

AB - 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.

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U2 - 10.1212/WNL.47.1.1

DO - 10.1212/WNL.47.1.1

M3 - Review article

VL - 47

SP - 1

EP - 9

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 1

ER -