SpringerLink
Log in

Pathologic changes of progressive supranuclear palsy, corticobasal degeneration, and multiple system atrophy

Prototype and clinicopathological diversity

  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA) are manifested as atypical parkinsonism. Both PSP and CBD are considered tauopathies because of the accumulation of tau protein in the brains, and MSA is considered a synucleinopathy owing to accumulation of α-synuclein. Although some pathological changes are found in both PSP and CBD, other changes allow the two conditions to be differentiated. Typical clinical manifestations of PSP and CBD have been described, but unusual clinical manifestations, which complicate clinical diagnosis, have been reported. MSA has been divided into the pathological subtypes of striatonigral degeneration and olivopontocerebellar atrophy, although some cases of MSA have features of both subtypes. Both the substantia nigra and the putamen are initially affected in the striatonigral degeneration subtype. Rare patients have both MSA and PSP.

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

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Arima K, Uesugi H, Fujita I, Sakurai Y, Oyanagi S, Andoh S, Izumiyama Y, Inose T (1994) Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ultrastructural and immunocytochemical studies. J Neurol Sci 127:186–197

    Article  PubMed  CAS  Google Scholar 

  2. Bergeron C, Pollanen MS, Weyer L, Black SE, Lang AE (1996) Unusual clinical presentations of cortical-basal ganglionic degeneration. Ann Neurol 40:893–900

    Article  PubMed  CAS  Google Scholar 

  3. Bergeron C, Pollanen MS, Weyer L, Lang AE (1997) Cortical degeneration in progressive supranuclear palsy. A comparison with cortical-basal ganglionic degeneration. J Neuropathol Exp Neurol 56:726–734

    PubMed  CAS  Google Scholar 

  4. Bigio EH, Brown DF, White CLr (1999) Progressive supranuclear palsy with dementia: cortical pathology. J Neuropathol Exp Neurol 58:359–364

    PubMed  CAS  Google Scholar 

  5. Daniel SE, de BV, Lees AJ (1995) The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. Brain 118:759–770

    PubMed  Google Scholar 

  6. Davis PH, Bergeron C, McLachlan DR (1985) Atypical presentation of progressive supranuclear palsy. Ann Neurol 17:337–343

    Article  PubMed  CAS  Google Scholar 

  7. Dickson DW, Bergeron C, Chin SS, Duyckaerts C,Horoupian D, Ikeda K, Jellinger K, Lantos PL, Lippa CF, Mirra SS, Tabaton M, Vonsattel JP, Wakabayashi K, Litvan I (2002) Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61:935–946

    PubMed  CAS  Google Scholar 

  8. Ferrer I, Hernandez I, Boada M, Llorente A, Rey MJ, Cardozo A, Ezquerra M, Puig B (2003) Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies. Acta Neuropathol (Berl) 106:419–435

    Article  PubMed  CAS  Google Scholar 

  9. Gibb WR, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192

    PubMed  Google Scholar 

  10. Hattori N, Goto K,Mori H, Tominaga I, Kondo T, Mizuno Y (1996) A 46-year-old man with right-side dominant parkinsonism, who suffered a sudden death. No To Shinkei 48:1059–1067

    PubMed  CAS  Google Scholar 

  11. Hauw JJ, Verny M, Delaere 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 

  12. Hishikawa N, Hashizume Y, Yoshida M, Niwa J, Tanaka F, Sobue G (2005) Tuft-shaped astrocytes in Lewy body disease. Acta Neuropathol (Berl) 109:373–380

    Article  PubMed  Google Scholar 

  13. Hof PR, Delacourte A, Bouras C (1992) Distribution of cortical neurofibrillary tangles in progressive supranuclear palsy: a quantitative analysis of six cases. Acta Neuropathol (Berl) 84:45–51

    Article  PubMed  CAS  Google Scholar 

  14. Ikebe S, Mori H, Sumino S, Takanashi M, Hamano Y, Shirai T, Ohkuma Y, Mizuno Y (2000) A 77-year-old man with gait and gaze disturbance. No To Shinkei 52:269–279

    PubMed  CAS  Google Scholar 

  15. Ikeda K, Akiyama H, Iritani S, Kase K, Arai T, Niizato K, Kuroki N, Kosaka K (1996) Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: case report and review. Acta Neuropathol (Berl) 92:534–539

    Article  PubMed  CAS  Google Scholar 

  16. Jellinger KA, Seppi K, Wenning GK (2005) Grading of neuropathology in multiple system atrophy: proposal for a novel scale. Mov Disord 20 (Suppl 12):S29–36

    Article  PubMed  Google Scholar 

  17. Katsuse O, Iseki E, Arai T, Akiyama H, Togo T, Uchikado H, Kato M, de Silva R, Lees A, Kosaka K (2003) 4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 106:251–260

    Article  PubMed  Google Scholar 

  18. Kertesz A, Martinez-Lage P, Davidson W, Munoz DG (2000) The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55:1368–1375

    PubMed  CAS  Google Scholar 

  19. Kertesz A, Munoz DG (2003) Primary progressive aphasia and Pick complex. J Neurol Sci 206:97–107

    Article  PubMed  Google Scholar 

  20. Lang AE (1992) Cortical basal ganglionic degeneration presenting with “progressive loss of speech output and orofacial dyspraxia”. J Neurol Neurosurg Psychiatry 55:1101

    PubMed  CAS  Google Scholar 

  21. Mathuranath PS, Xuereb JH, Bak T, Hodges JR (2000) Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature. J Neurol Neurosurg Psychiatry 68:304–312

    Article  PubMed  CAS  Google Scholar 

  22. Matsuo H, Takashima H, Kishikawa M, Kinoshita I, Mori M, Tsujihata M, Nagataki S (1991) Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 54:397–400

    Article  PubMed  CAS  Google Scholar 

  23. Mimura M, Oda T, Tsuchiya K, Kato M, Ikeda K,Hori K, Kashima H (2001) Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study. J Neurol Sci 183:19–26

    Article  PubMed  CAS  Google Scholar 

  24. Miyazaki H, Saito Y, Kijima Y, Akabane H, Tsuchiya K (1997) An autopsy case of corticobasal degeneration mimicking frontal Pick’s disease. No To Shinkei 49:277–282

    PubMed  CAS  Google Scholar 

  25. Mochizuki A, Ueda Y, Komatsuzaki Y, Tsuchiya K, Arai T, Shoji S (2003) Progressive supranuclear palsy presenting with primary progressive aphasia—clinicopathological report of an autopsy case. Acta Neuropathol (Berl) 105:610–614

    PubMed  CAS  Google Scholar 

  26. Mori H, Oda M, Komori T, Arai N, Takanashi M, Mizutani T, Hirai S, Mizuno Y (2002) Lewy bodies in progressive supranuclear palsy. Acta Neuropathol (Berl) 104:273–278

    PubMed  CAS  Google Scholar 

  27. Mori H, Oda M, Komori T, Arai N, Takanashi M, Ohta S, Mizutani T, Mizuno Y (2000) Progressive supranuclear palsy with Lewy bodies (abstract). Brain Pathol 10:523–525

    Google Scholar 

  28. Oda T, Ikeda K, Akamatsu W, Iwabuchi K, Akiyama H, Kondo H, Seta K, Kato Y, Kogure T, Hori K et al. (1995) An autopsy case of corticobasal degeneration clinically misdiagnosed as Pick’s disease. Seishin Shinkeigaku Zasshi 97:757–769

    PubMed  CAS  Google Scholar 

  29. Okuma Y, Hattori N, Nobukawa B, Mori H, Suda K, Takubo H, Mizuno Y (1998) A 74-year-old woman with parkinsonism and dementia who died four years after the onset. No To Shinkei 50:671–682

    PubMed  CAS  Google Scholar 

  30. Ozawa T, Paviour D, Quinn NP, Josephs KA, Sangha H, Kilford L, Healy DG, Wood NW, Lees AJ, Holton JL, Revesz T (2004) The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. Brain 127:2657–2671

    Article  PubMed  Google Scholar 

  31. Rebeiz JJ, Kolodny EH, Richardson EP (1968) Corticodentatonigral degeneration with neuronal degeneration. Arch Neurol 18:20–33

    PubMed  CAS  Google Scholar 

  32. Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Hornykiewicz O, Black S (1990) Cortical-basal ganglionic degeneration. Neurology 40:1203–1212

    PubMed  CAS  Google Scholar 

  33. Sergeant N, Wattez A, Delacourte A (1999) Neurofibrillary degeneration in progressive supranuclear palsy and corticobasal degeneration: tau pathologies with exclusively “exon 10” isoforms. J Neurochem 72:1243–1249

    Article  PubMed  CAS  Google Scholar 

  34. Shimo Y, Takanashi M, Ohta S, Terashima K, Mori H, Shirai T, Miwa H, Mizuno Y (2001) [A-56-year-old woman with parkinsonism, whose mother had Parkinson’s disease]. No To Shinkei 53:495–505

    PubMed  CAS  Google Scholar 

  35. Steele JC, Richardson JC, Olszewski J (1964) Progressive Supranuclear Palsy. A Heterogeneous Degeneration Involving The Brain Stem, Basal Ganglia And Cerebellum With Vertical Gaze And Pseudobulbar Palsy,Nuchal Dystonia And Dementia. Arch Neurol 10:333–359

    PubMed  CAS  Google Scholar 

  36. Takanashi M, Ohta S, Matsuoka S, Mori H, Mizuno Y (2002) Mixed multiple system atrophy and progressive supranuclear palsy: a clinical and pathological report of one case. Acta Neuropathol (Berl) 103:82–87

    Article  PubMed  Google Scholar 

  37. Tsuboi Y, Ahlskog JE, Apaydin H, Parisi JE, Dickson DW (2001) Lewy bodies are not increased in progressive supranuclear palsy compared with normal controls. Neurology 57:1675–1678

    PubMed  CAS  Google Scholar 

  38. Tsuboi Y, Josephs KA, Boeve BF, Litvan I, Caselli RJ, Caviness JN, Uitti RJ, Bott AD, Dickson DW (2005) Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 20:982–988

    Article  PubMed  Google Scholar 

  39. Uchikado H, Delledonne A, Uitti R, Dickson DW (2006) Coexistence of PSP and MSA: a case report and review of the literature. Acta Neuropathol 111:186–192

    Article  PubMed  Google Scholar 

  40. Utsumi H, Abe K, Yoshii O, Mori H, Suda K, Mizuno Y (1995) A 71-year-old woman with progressive gait disturbance and dementia. No To Shinkei 47:295–307

    PubMed  CAS  Google Scholar 

  41. Verny M, Jellinger KA, Hauw JJ, Bancher C, Litvan I, Agid Y (1996) Progressive supranuclear palsy: a clinicopathological study of 21 cases. Acta Neuropathol Berl 91:427–431

    Article  PubMed  CAS  Google Scholar 

  42. Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 128:1247–1258

    Article  PubMed  Google Scholar 

  43. Yokochi F (2002) Multiple system atrophy which had been treated as juvenile Parkinson’s disease. No To Shinkei 54:267–275

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Juntendo University School of Medicine, 2-1-1,Hongo, Bunkyo-ku, Tokyo 113-8421, Japan

    Hideo Mori MD

Authors
  1. Hideo Mori MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hideo Mori MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mori, H. Pathologic changes of progressive supranuclear palsy, corticobasal degeneration, and multiple system atrophy. J Neurol 253 (Suppl 3), iii41–iii46 (2006). https://doi.org/10.1007/s00415-006-3008-4

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-006-3008-4

Key words

Search

Navigation