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*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.
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
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N. J. Cordato, A. J. Duggins, G. M. Halliday, J. G. L. Morris, and C. Pantelis Clinical deficits correlate with regional cerebral atrophy in progressive supranuclear palsy Brain, June 1, 2005; 128(6): 1259 - 1266. [Abstract] [Full Text] [PDF]
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U Nath, R Thomson, R Wood, Y Ben-Shlomo, A Lees, C Rooney, and D Burn Population based mortality and quality of death certification in progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) J. Neurol. Neurosurg. Psychiatry, April 1, 2005; 76(4): 498 - 502. [Abstract] [Full Text] [PDF]
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T H Bak, T T Rogers, L M Crawford, V C Hearn, P S Mathuranath, and J R Hodges Cognitive bedside assessment in atypical parkinsonian syndromes J. Neurol. Neurosurg. Psychiatry, March 1, 2005; 76(3): 420 - 422. [Abstract] [Full Text] [PDF]
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P. Soliveri, S. Piacentini, and F. Girotti Limb apraxia in corticobasal degeneration and progressive supranuclear palsy Neurology, February 8, 2005; 64(3): 448 - 453. [Abstract] [Full Text] [PDF]
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N. M. Warren, M. A. Piggott, E. K. Perry, and D. J. Burn Cholinergic systems in progressive supranuclear palsy Brain, February 1, 2005; 128(2): 239 - 249. [Abstract] [Full Text] [PDF]
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S Garbutt, D E Riley, A N Kumar, Y Han, M R Harwood, and R J Leigh Abnormalities of optokinetic nystagmus in progressive supranuclear palsy J. Neurol. Neurosurg. Psychiatry, October 1, 2004; 75(10): 1386 - 1394. [Abstract] [Full Text] [PDF]
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U. Walter, D. Dressler, A. Wolters, T. Probst, A. Grossmann, and R. Benecke Sonographic discrimination of corticobasal degeneration vs progressive supranuclear palsy Neurology, August 10, 2004; 63(3): 504 - 509. [Abstract] [Full Text] [PDF]
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L. C.S. Tan, N. Venketasubramanian, C. Y. Hong, S. Sahadevan, J. J. Chin, E. S. Krishnamoorthy, A. K.Y. Tan, and S. M. Saw Prevalence of Parkinson disease in Singapore: Chinese vs Malays vs Indians Neurology, June 8, 2004; 62(11): 1999 - 2004. [Abstract] [Full Text] [PDF]
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A. Righini, A. Antonini, R. De Notaris, E. Bianchini, N. Meucci, G. Sacilotto, M. Canesi, D. De Gaspari, F. Triulzi, and G. Pezzoli MR Imaging of the Superior Profile of the Midbrain: Differential Diagnosis between Progressive Supranuclear Palsy and Parkinson Disease AJNR Am. J. Neuroradiol., June 1, 2004; 25(6): 927 - 932. [Abstract] [Full Text] [PDF]
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S. Yamada, K. Matsuo, M. Hirayama, and G. Sobue The effects of levodopa on apraxia of lid opening: A case report Neurology, March 9, 2004; 62(5): 830 - 831. [Full Text] [PDF]
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K Yoshikawa, Y Nakata, K Yamada, and M Nakagawa Early pathological changes in the parkinsonian brain demonstrated by diffusion tensor MRI J. Neurol. Neurosurg. Psychiatry, March 1, 2004; 75(3): 481 - 484. [Abstract] [Full Text] [PDF]
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M Ezquerra, J Campdelacreu, E Munoz, R Oliva, and E Tolosa Sequence analysis of tau 3'untranslated region and saitohin gene in sporadic progressive supranuclear palsy J. Neurol. Neurosurg. Psychiatry, January 1, 2004; 75(1): 155 - 157. [Abstract] [Full Text] [PDF]
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D Aarsland, I Litvan, D Salmon, D Galasko, T Wentzel-Larsen, and J P Larsen Performance on the dementia rating scale in Parkinson's disease with dementia and dementia with Lewy bodies: comparison with progressive supranuclear palsy and Alzheimer's disease J. Neurol. Neurosurg. Psychiatry, September 1, 2003; 74(9): 1215 - 1220. [Abstract] [Full Text] [PDF]
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H.R. Morris, Y. Osaki, J. Holton, A.J. Lees, N.W. Wood, T. Revesz, and N. Quinn Tau exon 10 +16 mutation FTDP-17 presenting clinically as sporadic young onset PSP Neurology, July 8, 2003; 61(1): 102 - 104. [Abstract] [Full Text] [PDF]
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Y. Tsuboi, J. Slowinski, K. A. Josephs, W. G. Honer, Z. K. Wszolek, and D. W. Dickson Atrophy of superior cerebellar peduncle in progressive supranuclear palsy Neurology, June 10, 2003; 60(11): 1766 - 1769. [Abstract] [Full Text] [PDF]
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U. Nath, Y. Ben-Shlomo, R.G. Thomson, A.J. Lees, and D.J. Burn Clinical features and natural history of progressive supranuclear palsy: A clinical cohort study Neurology, March 25, 2003; 60(6): 910 - 916. [Abstract] [Full Text] [PDF]
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C. G. Goetz, S. Leurgans, A. E. Lang, and I. Litvan Progression of gait, speech and swallowing deficits in progressive supranuclear palsy Neurology, March 25, 2003; 60(6): 917 - 922. [Abstract] [Full Text] [PDF]
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