Student or educator? Sign up with your school email and get 6 months of Pro free.
CoChat
Sign Up
Cited by 667

The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.

Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism

Williams, David R., de Silva, Rohan, Paviour, Dominic C., Pittman, Alan, Watt, Hilary C., Kilford, Linda, Holton, Janice L., Revesz, Tamas, Lees, Andrew J.·2005·Brain
DOI 10.1093/brain/awh488
Open this paper in CoChat — free account

Abstract

The clinical diagnosis of progressive supranuclear palsy (PSP) relies on the identification of characteristic signs and symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagnose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and to compare the biochemical and genetic characteristics of these groups. In 103 consecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson's syndrome (RS) and PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32%) were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson's disease (PSP-P). Fourteen cases (14%) could not be separated according to these criteria. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 years, P < 0.001) and age at death earlier (72.1 versus 75.5 years, P = 0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basal pons also differed significantly. In RS, the mean four-repeat:three-re…

Overview

Integrity signals

Registry

Crossref · 10.1093/brain/awh488

Cites

4 of 4 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Verification

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Citations

Cites

Show all 4 references

Research works printed but not linked: 63

  1. Albert ML, Feldman RG, Willis AL. The ‘subcortical dementia’ of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry1974; 37: 121–30. · DOI · Paper page
  2. Arai T, Ikeda K, Akiyama H, et al. Distinct isoforms of tau aggregated in neurons and glial cells in brains of patients with Pick's disease, corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol (Berl)2001; 101: 167–73. · DOI · Paper page
  3. Arnold G, Tatsch K, Oertel WH, et al. Clinical progressive supranuclear palsy: differential diagnosis by IBZM-SPECT and MRI. J Neural Transm Suppl1994; 42: 111–8. · doi:10.1007/978-3-7091-6641-3_9
  4. Avila J, Lucas JJ, Perez M, Hernandez F. Role of tau protein in both physiological and pathological conditions. Physiol Rev2004; 84: 361–84. · DOI · Paper page
  5. Bergeron C, Pollanen MS, Weyer L, Lang AE. Cortical degeneration in progressive supranuclear palsy. A comparison with cortical-basal ganglionic degeneration. J Neuropathol Exp Neurol1997; 56: 726–34. · DOI
  6. Bigio EH, Brown DF, White CL. Progressive supranuclear palsy with dementia: cortical pathology. J Neuropathol Exp Neurol1999; 58: 359–64. · DOI
  7. Birdi S, Rajput AH, Fenton M, et al. Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases. Mov Disord2002; 17: 1255–64. · DOI
  8. Braak H, Jellinger K, Braak E, Bohl J. Allocortical neurofibrillary changes in progressive supranuclear palsy. Acta Neuropathol (Berl)1992; 84: 478–83. · DOI
  9. Brion JP, Couck AM, Passareiro E, Flament-Durand J. Neurofibrillary tangles of Alzheimer's disease: an immunohistochemical study. J Submicrosc Cytol1985; 17: 89–96.
  10. Brion JP, Couck AM, Robertson J, Loviny TL, Anderton BH. Neurofilament monoclonal antibodies RT97 and 8D8 recognize different modified epitopes in paired helical filament-tau in Alzheimer's disease. J Neurochem1993; 60: 1372–82. · DOI
  11. Buee L, Delacourte A. Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease. Brain Pathol1999; 9: 681–93. · DOI · Paper page
  12. Buee L, Delacourte A, Wilhelmsen KC, Miller B, Geschwind D. Neuronal subpopulations and genetic background in tauopathies: a catch 22 story? Neurobiol Aging2001; 22: 115–21. · DOI
  13. Chambers CB, Lee JM, Troncoso JC, Reich S, Muma NA. Overexpression of four-repeat tau mRNA isoforms in progressive supranuclear palsy but not in Alzheimer's disease. Ann Neurol1999; 46: 325–32. · doi:10.1002/1531-8249(199909)46:3<325::aid-ana8>3.0.co;2-v
  14. Conrad C, Andreadis A, Trojanowski JQ, et al. Genetic evidence for the involvement of tau in progressive supranuclear palsy. Ann Neurol1997; 41: 277–81. · DOI · Paper page
  15. Daniel SE, De Bruin V, Lees AJ. The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. Brain1995; 118: 759–70. · DOI · Paper page
  16. Davis PH, Bergeron C, McLachlan DR. Atypical presentation of progressive supranuclear palsy. Ann Neurol1985; 17: 337–43. · DOI · Paper page
  17. de Silva R, Hope A, Pittman A, et al. Strong association of the Saitohin gene Q7 variant with progressive supranuclear palsy. Neurology2003; 61: 407–9. · DOI
  18. de Silva R, Lashley T, Gibb G, et al. Pathological inclusion bodies in tauopathies contain distinct complements of tau with three or four microtubule-binding repeat domains as demonstrated by new specific monoclonal antibodies. Neuropathol Appl Neurobiol2003; 29: 288–302. · DOI · Paper page
  19. Delacourte A, Robitaille Y, Sergeant N, et al. Specific pathological Tau protein variants characterize Pick's disease. J Neuropathol Exp Neurol1996; 55: 159–68. · DOI · Paper page
  20. Dubas F, Gray F, Escourolle R. [Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases]. Rev Neurol (Paris)1983; 139: 407–16.
  21. Flament S, Delacourte A, Hemon B, Defossez A. Characterization of two pathological tau protein, variants in Alzheimer brain cortices. J Neurol Sci1989; 92: 133–41. · DOI
  22. Forman MS. Genotype-phenotype correlations in FTDP-17: does form follow function? Exp Neurol2004; 187: 229–34. · DOI
  23. Gibb GM, de Silva R, Revesz T, Lees AJ, Anderton BH, Hanger DP. Differential involvement and heterogeneous phosphorylation of tau isoforms in progressive supranuclear palsy. Brain Res Mol Brain Res2004; 121: 95–101. · DOI
  24. Goedert M, Jakes R, Crowther RA, Hasegawa M, Smith MJ, Spillantini MG. Intraneuronal filamentous tau protein and alpha-synuclein deposits in neurodegenerative diseases. Biochem Soc Trans1998; 26: 463–71. · DOI
  25. Grundke-Iqbal I, Iqbal K, Quinlan M, Tung YC, Zaidi MS, Wisniewski HM. Microtubule-associated protein tau. A component of Alzheimer paired helical filaments. J Biol Chem1986; 261: 6084–9. · DOI
  26. Hanger DP, Brion JP, Gallo JM, Cairns NJ, Luthert PJ, Anderton BH. Tau in Alzheimer's disease and Down's syndrome is insoluble and abnormally phosphorylated. Biochem J1991; 275: 99–104. · DOI
  27. Hanger DP, Betts JC, Loviny TL, Blackstock WP, Anderton BH. New phosphorylation sites identified in hyperphosphorylated tau (paired helical filament-tau) from Alzheimer's disease brain using nanoelectrospray mass spectrometry. J Neurochem1998; 71: 2465–76. · DOI · Paper page
  28. Hanger DP, Gibb GM, de Silva R, et al. The complex relationship between soluble and insoluble tau in tauopathies revealed by efficient dephosphorylation and specific antibodies. FEBS Lett2002; 531: 538–42. · DOI
  29. Hauw JJ, Verny M, Delaere P, Cervera P, He Y, Duyckaerts C. Constant neurofibrillary changes in the neocortex in progressive supranuclear palsy. Basic differences with Alzheimer's disease and aging. Neurosci Lett1990; 119: 182–6. · DOI · Paper page
  30. Hughes AJ, Daniel SE, Lees AJ. Improved accuracy of clinical diagnosis of Lewy body Parkinson's disease. Neurology2001; 57: 1497–9. · DOI · Paper page
  31. Hughes AJ, Daniel SE, Ben-Shlomo Y, Lees AJ. The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain2002; 125: 861–70. · DOI · Paper page
  32. Jellinger K. Progressive supranuclear palsy (subcortical argyrophilic dystrophy). Acta Neuropathol (Berl)1971; 19: 347–52. · DOI
  33. Juncos JL, Hirsch EC, Malessa S, Duyckaerts C, Hersh LB, Agid Y. Mesencephalic cholinergic nuclei in progressive supranuclear palsy. Neurology1991; 41: 25–30. · DOI
  34. Ksiezak-Reding H, Morgan K, Mattiace LA, et al. Ultrastructure and biochemical composition of paired helical filaments in corticobasal degeneration. Am J Pathol1994; 145: 1496–508.
  35. Landwehrmeyer B, Palacios JM. Alterations of neurotransmitter receptors and neurotransmitter transporters in progressive supranuclear palsy. J Neural Transm Suppl1994; 42: 229–46. · DOI
  36. Lieberman AP, Trojanowski JQ, Lee VM, et al. Cognitive, neuroimaging, and pathological studies in a patient with Pick's disease. Ann Neurol1998; 43: 259–65. · DOI · Paper page
  37. Litvan I, Hauw JJ, Bartko JJ, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol1996; 55: 97–105. · DOI · Paper page
  38. Litvan I, Grimes DA, Lang AE, et al. Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration. J Neurol1999; 246 Suppl 2: II1NII5. · DOI
  39. Litvan I, Bhatia KP, Burn DJ, et al. Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Disord2003; 18: 467–86. · DOI · Paper page
  40. Liu WK, Le TV, Adamson J, et al. Relationship of the extended tau haplotype to tau biochemistry and neuropathology in progressive supranuclear palsy. Ann Neurol2001; 50: 494–502. · DOI
  41. Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology1986; 36: 1005–8. · DOI · Paper page
  42. Mailliot C, Sergeant N, Bussiere T, Caillet-Boudin ML, Delacourte A, Buee L. Phosphorylation of specific sets of tau isoforms reflects different neurofibrillary degeneration processes. FEBS Lett1998; 433: 201–4. · DOI
  43. Maraganore DM, Anderson DW, Bower JH, McDonnell SK, Rocca WA. Autopsy patterns for Parkinson's disease and related disorders in Olmsted County, Minnesota. Neurology1999; 53: 1342–4. · DOI
  44. Masliah E, Hansen LA, Quijada S, et al. Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy? Ann Neurol1991; 29: 389–96. · DOI
  45. Matsuo H, Takashima H, Kishikawa M, et al. Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry1991; 54: 397–400. · DOI
  46. Morris HR, Gibb G, Katzenschlager R, et al. Pathological, clinical and genetic heterogeneity in progressive supranuclear palsy. Brain2002; 125:969–75. · DOI
  47. Motoi Y, Takanashi M, Itaya M, Ikeda K, Mizuno Y, Mori H. Glial localization of four-repeat tau in atypical progressive supranuclear palsy. Neuropathology2004; 24: 60–5. · DOI
  48. Mulot SF, Hughes K, Woodgett JR, Anderton BH, Hanger DP. PHF-tau from Alzheimer's brain comprises four species on SDS-PAGE which can be mimicked by in vitro phosphorylation of human brain tau by glycogen synthase kinase-3 beta. FEBS Lett1994; 349: 359–64. · DOI
  49. Nath U, Ben-Shlomo Y, Thomson RG, Lees AJ, Burn DJ. Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study. Neurology2003; 60: 910–6. · DOI · Paper page
  50. Parvizi J, Van Hoesen GW, Damasio A. The selective vulnerability of brainstem nuclei to Alzheimer's disease. Ann Neurol2001; 49: 53–66. · DOI · Paper page
  51. Pierot L, Desnos C, Blin J, et al. D1 and D2-type dopamine receptors in patients with Parkinson's disease and progressive supranuclear palsy. J Neurol Sci1988; 86: 291–306. · DOI
  52. Pittman AM, Myers AJ, Duckworth J, et al. The structure of the tau haplotype in controls and in progressive supranuclear palsy. Hum Mol Genet2004; 13: 1267–74. · DOI
  53. Revesz T, Sangha H, Daniel SE. The nucleus raphe interpositus in the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Brain1996; 119: 1137–43. · DOI
  54. Ruberg M, Javoy-Agid F, Hirsch E, et al. Dopaminergic and cholinergic lesions in progressive supranuclear palsy. Ann Neurol1985; 18:523–9. · DOI · Paper page
  55. Schraen-Maschke S, Dhaenens CM, Delacourte A, Sablonniere B. Microtubule-associated protein tau gene: a risk factor in human neurodegenerative diseases. Neurobiol Dis2004; 15: 449–60. · DOI
  56. Sham PC, Curtis D. Monte Carlo tests for associations between disease and alleles at highly polymorphic loci. Ann Hum Genet1995; 59: 97–105. · DOI · Paper page
  57. Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogeneous degeneration involving the brainstem, basal ganglia and cerebellum with vertical supranuclear gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol1964; 10: 333–59. · DOI · Paper page
  58. Suzuki M, Desmond TJ, Albin RL, Frey KA. Cholinergic vesicular transporters in progressive supranuclear palsy. Neurology2002; 58: 1013–8. · DOI
  59. Takanashi M, Mori H, Arima K, Mizuno Y, Hattori N. Expression patterns of tau mRNA isoforms correlate with susceptible lesions in progressive supranuclear palsy and corticobasal degeneration. Brain Res Mol Brain Res2002; 104: 210–9. · DOI
  60. Vermersch P, Robitaille Y, Bernier L, Wattez A, Gauvreau D, Delacourte A. Biochemical mapping of neurofibrillary degeneration in a case of progressive supranuclear palsy: evidence for general cortical involvement. Acta Neuropathol (Berl)1994; 87: 572–7. · DOI
  61. Verny M, Duyckaerts C, Agid Y, Hauw JJ. The significance of cortical pathology in progressive supranuclear palsy. Clinico-pathological data in 10 cases. Brain1996; 119: 1123–36. · DOI
  62. Verny M, Jellinger KA, Hauw JJ, Bancher C, Litvan I, Agid Y. Progressive supranuclear palsy: a clinicopathological study of 21 cases. Acta Neuropathol (Berl)1996; 91: 427–31. · DOI
  63. Wenham PR, Price WH, Blandell G. Apolipoprotein E genotyping by one-stage PCR. Lancet1991; 337: 1158–9. · DOI · Paper page

Cited by

See all 667

Metadata

Authors
Williams, D. R., de Silva, R., Paviour, D. C., Pittman, A., Watt, H. C., Kilford, L., Holton, J. L., Revesz, T., & Lees, A. J.
Venue
Brain
Publisher
Oxford University Press (OUP)
Published
2005-03-23
Volume
128
Issue
6
Pages
1247-1258
ISSN
  • 1460-2156
  • 0006-8950
DOI
10.1093/brain/awh488
Cited by
667
References
4
Language
en

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

How to cite

Cite CoChat for the checks on this page, and the paper for its own findings.

This page
CoChat. (2026). Citation check: Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. https://app.cochat.ai/papers/10.1093/brain/awh488
The paper
Williams, D. R., de Silva, R., Paviour, D. C., Pittman, A., Watt, H. C., Kilford, L., Holton, J. L., Revesz, T., & Lees, A. J. (2005). Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. Brain. https://doi.org/10.1093/brain/awh488

Data sources

Bibliographic data: Semantic Scholar, OpenAlex, Crossref.

Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.