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.
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The final diagnosis of PD, MSA, and PSP was determined by two neurologists according to updated clinical guidelines.
“The final diagnosis of PD, MSA, and PSP was determined by two neurologists according to the updated clinical guidelines[37-40].”
From QEEG Signatures are Associated with Nonmotor Dysfunctions in Parkinson's Disease and Atypical Parkinsonism: An Integrative Analysis (2023) · cites this paper as [37]
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Cerebrospinal fluid biomarkers predict frontotemporal dementia trajectory
Ljubenkov, P. A., Staffaroni, A. M., Rojas, J. C., Allen, I. E., Wang, P., Heuer, H., Karydas, A., Kornak, J., Cobigo, Y., Seeley, W. W., et al. (2018) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.643 · Cited by 49 · see citing works
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Elevated in vivo [18F]‐AV‐1451 uptake in a patient with progressive supranuclear palsy
Hammes, J., Bischof, G. N., Giehl, K., Faber, J., Drzezga, A., Klockgether, T., & van Eimeren, T. (2017) · Movement Disorders · doi:10.1002/mds.26727 · Cited by 49 · see citing works
Magdalinou, N. K., Noyce, A. J., Pinto, R., Lindstrom, E., Holmén-Larsson, J., Holtta, M., Blennow, K., Morris, H. R., Skillbäck, T., Warner, T. T., et al. (2017) · Parkinsonism & Related Disorders · doi:10.1016/j.parkreldis.2017.01.016 · Cited by 49 · see citing works
Bharti, K., Bologna, M., Upadhyay, N., Piattella, M. C., Suppa, A., Petsas, N., Giannì, C., Tona, F., Berardelli, A., & Pantano, P. (2017) · Frontiers in Neurology · doi:10.3389/fneur.2017.00248 · Cited by 44 · see citing works
Liew, T. M. (2021) · Age and Ageing · doi:10.1093/ageing/afab044 · Cited by 38 · see citing works
Carandini, T., Arighi, A., Sacchi, L., Fumagalli, G. G., Pietroboni, A. M., Ghezzi, L., Colombi, A., Scarioni, M., Fenoglio, C., De Riz, M. A., et al. (2019) · Alzheimer's Research & Therapy · doi:10.1186/s13195-019-0543-7 · Cited by 36 · see citing works
The interrelationship between non-motor symptoms in Atypical Parkinsonism
Lee, C.-N., Kim, M., Lee, H. M., Jang, J.-W., Lee, S.-M., Kwon, D.-Y., Park, K.-W., & Koh, S.-B. (2013) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2013.01.034 · Cited by 32 · see citing works
Identifying bvFTD within the wide spectrum of late onset frontal lobe syndrome: a clinical approach
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Looking for measures of disease severity in the frontotemporal dementia continuum
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“…(FTD) [2], primary progressive aphasia (PPA) of the logopenic (lv-PPA), semantic (SD) or non-fluent/agrammatic (nf-PPA) [22] variant, corticobasal syndrome (CBD) [4], progressive supranuclear palsy (PSP) [23], posterior cortical atrophy (PCA) [24], Lewy body dementia (LBD) [25], and depression [26].”
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The Use of Profanity During Letter Fluency Tasks in Frontotemporal Dementia and Alzheimer Disease
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Diagnosing progressive supranuclear palsy: Role of biological and neuroimaging markers
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- Authors
- 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.
- Affiliations
- National Institutes of Health (US)
- ORCID
- Venue
- Neurology
- Published
- 1996-07
- Volume
- 47
- Issue
- 1
- Pages
- 1-9
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2136557657
- Cited by
- 2,538
- References
- 0
- Citations by year
- 2026: 34 · 2025: 66 · 2024: 61 · 2023: 78 · 2022: 103 · 2021: 112 · 2020: 126 · 2019: 123 · 2018: 118 · 2017: 153 · 2016: 140 · 2015: 105 · 2014: 106 · 2013: 116 · 2012: 105
- FWCI
- 13.14
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neurology (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Health Sciences › Medicine › Neurology › Neurological disorders and treatments
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Keywords
- progressive supranuclear palsy, diagnostic criteria, supranuclear gaze palsy, postural instability, falls, sensitivity and specificity, autopsy confirmation
- MeSH terms
- Humans, Supranuclear Palsy, Progressive
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