Skip to main content
SpringerLink
Log in

The neuropathology of progressive supranuclear palsy

  • Conference paper
Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy

Part of the book series: Journal of Neural Transmission. Supplementa ((NEURAL SUPPL,volume 42))

Summary

The macroscopical, histological, ultrastructural and immuno-cytochemical features of progressive supranuclear palsy (PSP) are reviewed. Recent investigations have revealed important differences in the distribution, ultrastructure and immunocytochemical profile of neurofibrillary tangles in PSP and in Alzheimer’s disease. Cortical involvement, as demonstrated by the presence of tangles and neuropil threads, has extended the neuropathological spectrum of PSP. Quantitative assessments of neuronal populations show neuronal loss, not only in various nuclei of the brainstem, diencephalon and cerebellum, but also in other areas, including the nucleus basalis of Meynert, substantia nigra and neostriatum.

A new classification, based on neuropathological criteria, is suggested in order to take into consideration the phenotypic heterogeneity of PSP. This new classification distinguishes three types: typical, atypical and combined cases. Typical (Type 1) cases conform to the original definition of PSP. Type 2, atypical cases are variants of the histological changes characteristic of PSP: either the severity or the distribution of abnormalities, or both of these deviate from the typical pattern. Cases with combined pathology belong to type 3 group: in these the typical pathology of PSP is accompanied by lesions characteristic of another neurodegenerative or vascular disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Akashi T, Arima K, Maruyama N, Ando S, Inose T (1989) Severe cerebral atrophy in progressive supranuclear palsy: a case report. Clin Neuropathol 8: 195–199.

    PubMed  CAS  Google Scholar 

  • Arai N (1989) “Grumose degeneration” of the dentate nucleus. A light and electron microscopic study in progressive supranuclear palsy and dentatorubropallidoluysial atrophy. J Neurol Sci 90: 131–145.

    Article  PubMed  CAS  Google Scholar 

  • Bancher C, Lassmann H, Budka H, et al (1987) Neurofibrillary tangles in Alzheimer’s disease and progressive supranuclear palsy: antigenic similarities and differences. Acta Neuropathol 74: 39–46.

    Article  PubMed  CAS  Google Scholar 

  • Barr AN (1986) Progressive supranuclear palsy. In: Vinken PJ, Bruyn GW, Klawans HJ (eds) Handbook of clinical neurology, vol 5(49). Extrapyramidal disorders. Elsevier, Amsterdam, pp 239–254.

    Google Scholar 

  • Brown J, Lantos P, Stratton M, Roques P, Rossor M (1993) A familial dementia with progressive supranuclear palsy pathology. J Neurol Neurosurg Psychiatry 56: 473–476.

    Article  PubMed  CAS  Google Scholar 

  • Cruz-Sanchez FF, Rossi ML, Cardozo A, Deacon P, Tolosa E (1992) Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer’s changes. Antigenic determinants that distinguish cortical and subcortical neurofibrillary tangles. Neurosci Lett 136: 43–46.

    Article  PubMed  CAS  Google Scholar 

  • Dubas F, Gray F, Escourolle R (1983) Maladie de Steele-Richardson-Olszewski sans ophtalmoplĂ©gie. Six cas anatomo-cliniques. Rev Neurol 139: 407–416.

    PubMed  CAS  Google Scholar 

  • Fearnley JM, Lees AJ (1991) Ageing and Parkinson’s disease: substantia nigra regional selectivity. Brain 114: 2283–2301.

    Article  PubMed  Google Scholar 

  • Flament S, Delacourte A, Veray M, Hauw J-J, Javoy-Agid F (1991) Abnormal tau protein in progressive supranuclear palsy. Similarities and differences with the neurofibrillary degeneration of the Alzheimer type. Acta Neuropathol 81: 591–596.

    Article  PubMed  CAS  Google Scholar 

  • Giaccone G, Tagliavini F, Street JS, Ghetti B, Bugiani O (1988) Progressive supranuclear palsy with hypertrophy of the olives. An immunocytochemical study of the cytoskeleton of argyrophilic neurons. Acta Neuropathol 77: 14–20.

    Article  PubMed  CAS  Google Scholar 

  • Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC (1988) Prevalence and natural history of progressive supranuclear palsy. Neurology 38: 1031–1034.

    Article  PubMed  CAS  Google Scholar 

  • Hauw J-J, Verny M, Delaère P, Cervera P, He Y, Duyckaerts C (1990) Constant neurofibrillary changes in the neocortex in progressive supranuclear palsy. Basic differences with Alzheimer’s disease and aging. Neurosci Lett 119: 182–186.

    Article  PubMed  CAS  Google Scholar 

  • Hirsch EC, Graybiel AM, Duyckaerts C, Javoy-Agid F (1987) Neuronal loss in the pedunculopontine tegmental nucleus in Parkinson disease and in progressive supra-nuclear palsy. Proc Natl Acad Sci USA 84: 5976–5980.

    Article  PubMed  CAS  Google Scholar 

  • Hynd EW, Pirozzolo FJ, Maletta GJ (1982) Progressive supranuclear palsy. Int J Neurosci 16: 87–98.

    Article  PubMed  CAS  Google Scholar 

  • Jackson JA, Jankovic J, Ford J (1983) Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Ann Neurol 13: 273–278.

    Article  PubMed  CAS  Google Scholar 

  • Jellinger K, Riederer P, Tomonaga M (1980) Progressive supranuclear palsy: clinico-pathological and biochemical studies. J Neural Transm [Suppl] 16: 111–128.

    Google Scholar 

  • Jellinger K (1971) Progressive supranuclear palsy (subcortical argyrophilic dystrophy). Acta Neuropathol 19: 347–352.

    Article  PubMed  CAS  Google Scholar 

  • Jellinger K (1988) The pedunculopontine nucleus in Parkinson’s disease, progressive supranuclear palsy and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 52: 540–543.

    Article  Google Scholar 

  • Kato T, Hirano A, Weinberg MN, Jacobs AK (1986) Spinal cord lesions in progressive supranuclear palsy: some new observations. Acta Neuropathol 71: 11–14.

    Article  PubMed  CAS  Google Scholar 

  • Kleinschmidt-DeMasters BK (1989) Early progressive supranuclear palsy: pathology and clinical presentation. Clin Neuropathol 8: 79–84.

    PubMed  CAS  Google Scholar 

  • Kristensen MO (1985) Progressive supranuclear palsy—20 years later. Acta Neurol Scand 71: 177–189.

    Article  PubMed  CAS  Google Scholar 

  • Leigh PN, Probst A, Dale GE, et al (1989) New aspects of pathology of neuro-degenerative disorders as revealed by ubiquitin antibodies. Acta Neuropathol 79: 61–72.

    Article  PubMed  CAS  Google Scholar 

  • Lennox G, Lowe J, Morrell K, Landon M, Mayer RJ (1988) Ubiquitin is a component of neurofibrillary tangles in a variety of neurodegenerative diseases. Neurosci Lett 94: 211–217.

    Article  PubMed  CAS  Google Scholar 

  • Love S, Saitoh T, Quijada S, Cole GM, Terry RD (1988) Alz-50, ubiquitin and tau immunoreactivity of neurofibrillary tangles, Pick bodies and Lewy bodies. J Neuropathol Exp Neurol 47: 393–405.

    Article  PubMed  CAS  Google Scholar 

  • Maher ER, Lees AJ (1986) The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 36: 1005–1008.

    Article  PubMed  CAS  Google Scholar 

  • Malessa S, Hirsch EC, Cervera P, et al (1991) Progressive supranuclear palsy: loss of choline acetyltransferase-like immunoreactive neurons in the pontine reticular formation. Neurology 41: 1593–1597.

    Article  PubMed  CAS  Google Scholar 

  • Manetto V, Perry G, Tabaton M, et al (1988) Ubiquitin is associated with abnormal cytoplasmic filaments characteristic of neurodegenerative disease. Proc Natl Acad Sci USA 85: 4501–4505.

    Article  PubMed  CAS  Google Scholar 

  • Mann DMA, Yates PO, Hawkes J (1983) The pathology of the human locus coeruleus. Clin Neuropathol 2: 1–7.

    PubMed  CAS  Google Scholar 

  • Mann DMA, Jones D (1990) Deposition of amyloid (A4) protein within the brains of persons with dementing disorders other than Alzheimer’s disease and Down’s syndrome. Neurosci Lett 109: 68–75.

    Article  PubMed  CAS  Google Scholar 

  • Mastaglia FL, Grainger K, Kee F, Sadka M, Lefroy R (1973) Progressive supranuclear palsy (the Steele-Richardson-Olszewski syndrome): clinical and electrophysiological observations in eleven cases. Proc Aust Assoc Neurol 10: 35–44.

    PubMed  CAS  Google Scholar 

  • Milder DG, Elliott CF, Evans WA (1984) Neuropathological findings in a case of coexistent progressive supranuclear palsy and Alzheimer’s disease. Clin Exp Neurol 20: 181–187.

    PubMed  CAS  Google Scholar 

  • Mizusawa H, Yen S-H, Hirano A, Llena JF (1989) Pathology of the dentate nucleus in progressive supranuclear palsy: a histological, immunohistochemical and ultra-structural study. Acta Neuropathol 78: 419–428.

    Article  PubMed  CAS  Google Scholar 

  • Montpetit V, Clapin DF, Guberman A (1985) Substructure of 20 nm filaments of progressive supranuclear palsy. Acta Neuropathol 68: 311–318.

    Article  PubMed  CAS  Google Scholar 

  • Mori H, Yoshimura M, Tomonaga M, Yamanouchi H (1986) Progressive supranuclear palsy with Lewy bodies. Acta Neuropathol 71: 344–346.

    Article  PubMed  CAS  Google Scholar 

  • Oyanagi K, Takahashi H, Wakabayashi K, Ikuta F (1988) Selective decrease of large neurons in the neostriatum in progressive supranuclear palsy. Brain Res 458: 218–223.

    Article  PubMed  CAS  Google Scholar 

  • Probst A, Langui D, Lautenschlager C, Ulrich J, Brion JP, Anderton BH (1988) Progressive supranuclear palsy: extensive neuropil threads in addition to neuro-fibrillary tangles. Very similar antigenicity of subcortical neuronal pathology in progressive supranuclear palsy and Alzheimer’s disease. Acta Neuropathol 77: 61–68.

    Article  PubMed  CAS  Google Scholar 

  • Pollock NJ, Mirra SS, Binder LI, Hansen LA, Wood JG (1986) Filamentous aggregates in Pick’s disease, progressive supranuclear palsy, and Alzheimer’s disease share antigenic determinants with microtubule-associated protein, tau. Lancet ii: 1211.

    Article  Google Scholar 

  • Posey WC (1904) Paralysis of the upward movement of the eyes. Ann Ophthal 13: 523–531.

    Google Scholar 

  • Schmidt MK, Lee VM-Y, Hurtig H, Trojanowski JQ (1988) Properties of antigenic determinants that distinguish neurofibrillary tangles in progressive supranuclear palsy and Alzheimer’s disease. Lab Invest 59: 460–466.

    PubMed  CAS  Google Scholar 

  • Shin R-W, Kitamoto T, Tateishi J (1991) Modified tau is present in younger nondemented persons: a study of subcortical nuclei in Alzheimer’s disease and progressive supranuclear palsy. Acta Neuropathol 81: 517–523.

    Article  PubMed  CAS  Google Scholar 

  • Spiller WG (1905) The importance in clinical diagnosis of paralysis of associated movements of the eyeballs (Blick-Lahmung), especially of upward and downward associated movements. J Nerv Ment Dis 32: 417–448 and 497-530.

    Article  Google Scholar 

  • Steele JC, Richardson JC, Olszewski T (1964) Progressive supranuclear palsy. Arch Neurol 10: 333–359.

    Article  PubMed  CAS  Google Scholar 

  • Steele JC (1975) Progressive supranuclear palsy. In: Vinken PJ, Bruyn GW, deJong JMB (eds) Handbook of clinical neurology, vol 22. System disorders and atrophies, part II North Holland, Amsterdam, pp 217–229.

    Google Scholar 

  • Takahashi H, Oyanagi K, Takedo S, Hinokuma K, Ikuta F (1989) Occurrence of 15-nm—wide straight tubules in neocortical neurons in progressive supranuclear palsy. Acta Neuropathol 79: 233–239.

    Article  PubMed  CAS  Google Scholar 

  • Takahashi H, Takeda S, Ikuta F, Homma Y (1987) Progressive supranuclear palsy with limbic system involvement: report of a case with ultrastructural investigation of neurofibrillary tangles in various locations. Clin Neuropathol 6: 271–276.

    PubMed  CAS  Google Scholar 

  • Tagliavini F, Pilleri G, Bouras C, Constantinidis J (1984) The basal nucleus of Meynert in patients with progressive supranuclear palsy. Neurosci Lett 44: 37–42.

    Article  PubMed  CAS  Google Scholar 

  • Tan N, Mastaglia FL, Masters CL, Beyreuther K, Kakulas BA (1988) Amyloid (A4) protein deposition in brain in progressive supranuclear palsy (PSP). Alzheimer Dis Assoc Dis 2: 264.

    Article  Google Scholar 

  • Tellez-Nagel I, Wisniewski HM (1973) Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch Neurol 29: 324–327.

    Article  PubMed  CAS  Google Scholar 

  • Tomonaga M (1977) Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropathol 37: 177–181.

    Article  PubMed  CAS  Google Scholar 

  • Tomonaga M (1983) Neuropathology of the locus coeruleus: a semi-quantitative study. J Neurol 230: 231–240.

    Article  PubMed  CAS  Google Scholar 

  • Will RG, Lees AJ, Gibb W, Barnard RO (1988) A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome. J Neurol Neurosurg Psychiatry 51: 1224–1227.

    Article  PubMed  CAS  Google Scholar 

  • Yagishita S, Itoh Y, Amano N, Nakano T, Saitoh A (1979) Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropathol 48: 27–30.

    Article  PubMed  CAS  Google Scholar 

  • Yagashita S, Itoh Y, Nakano T (1986) Hypertrophy of the olivary nucleus. An ultra-sctructural study. Acta Neuropathol 69: 132–138.

    Article  Google Scholar 

  • Yamamoto Y, Kawamura J, Hashimoto S, Nakamura H, Kobashi Y, Ichijima K (1991) Pallido-nigro-luysian atrophy, progressive supranuclear palsy and adult onset Hallervorden-Spatz disease: a case of akinesia as a predominant feature of par-kinsonism. J Neurol Sci 101: 98–106.

    Article  PubMed  CAS  Google Scholar 

  • Zweig RM, Whitehouse PJ, Casanova MF, Walker LC, Jankel WR, Price DL (1987) Loss of pedunculopontine neurons in progressive supranuclear palsy. Ann Neurol 22: 18–25.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neuropathology, Institute of Psychiatry, London, SE5 8AF, UK

    Dr. P. L. Lantos

Authors
  1. Dr. P. L. Lantos
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Neurology Service, Hospital Clinic, Faculty of Medicine, University of Barcelona, Spain

    E. Tolosa

  2. Neurology Service, Robert Wood Johnson University Hospital, New Brunswick, NJ, USA

    R. Duvoisin

  3. Neurological Tissue Bank, Faculty of Medicine, University of Barcelona, Spain

    F. F. Cruz-Sánchez

Rights and permissions

Reprints and permissions

Copyright information

© 1994 Springer-Verlag Wien

About this paper

Cite this paper

Lantos, P.L. (1994). The neuropathology of progressive supranuclear palsy. In: Tolosa, E., Duvoisin, R., Cruz-Sánchez, F.F. (eds) Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy. Journal of Neural Transmission. Supplementa, vol 42. Springer, Vienna. https://doi.org/10.1007/978-3-7091-6641-3_11

Download citation

  • DOI: https://doi.org/10.1007/978-3-7091-6641-3_11

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-82541-9

  • Online ISBN: 978-3-7091-6641-3

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation