Clinical heterogeneity of progressive supranuclear palsy

Cover Page


Cite item

Full Text

Abstract

Progressive supranuclear palsy (PSP) is a sporadic neurodegenerative disease characterized by oculomotor disorders, early onset of postural instability and cognitive dysfunction. Classic form of the disease (Richardson’s syndrome) develops only in 24% of all cases, while other phenotypes are registered in most patients. Clinical manifestations of the various types the PSP, as well as their similarities and differences with other nosological forms of extrapyramidal disorders are described in the current review. New diagnostic criteria of PSP developed by the International Society for the Parkinson's Disease and Movement Disorders in 2017 are discussed. Two clinical cases of PSP in patients of Yakhut ethnic origin are presented: in the first case there was classical variant of the disease (Richardson’s syndrome), and in the second case – a PSP-parkinsonism variant.

About the authors

Alexey A. Tappakhov

M.K. Ammosov North-Eastern Federal University

Author for correspondence.
Email: govorovatatyana@mail.ru
Россия, Yakutsk

Tatiana E. Popova

M.K. Ammosov North-Eastern Federal University

Email: govorovatatyana@mail.ru
ORCID iD: 0000-0003-1062-1540

D. Sci. (Med.), Deputy director for science

Россия, Yakutsk

Tatiana G. Govorova

M.K. Ammosov North-Eastern Federal University

Email: govorovatatyana@mail.ru
ORCID iD: 0000-0003-0610-3660

postgraduate student, Department of neurology and psychiatry, Head, Laboratory of neuropsychophysiological research, Medical Institute

Россия, Yakutsk

Alyona Yu. Petrova

Republican Hospital No. 2 — The center of the Emergency Medical Care

Email: govorovatatyana@mail.ru

neurologist, Department of neurology

Россия, Yakutsk

References

  1. Illarioshkin S.N., Vlasenko A.G., Fedotova E.Yu. Current means for identifying the latent stage of a neurodegenerative process. Annals of Clinical and Experimental Neurology. 2013; 2: 39–50. (in Russ.).
  2. Gitler A.D., Dhillon P., Shorter J. Neurodegenerative disease: models, mechanisms, and a new hope. Dis Model Mech 2017; 10(5): 499–502. doi: 10.1242/dmm.030205. PMID: 28468935.
  3. Dalvi A. Parkinsonism. Dis Mon 2012; 58(12): 690–707. doi: 10.1016/j.disamonth.2012.08.010. PMID: 23149522.
  4. Carreiro A., Mendonça A., de Carvalho M., Madeira S.C. Integrative biomarker discovery in neurodegenerative diseases. Wiley Interdiscip Rev Syst Biol Med 2015; 7(6): 357–379. doi: 10.1002/wsbm.1310. PMID: 26136395.
  5. Doronina O.B., Aftanas L.I., Doronina K.S. Heterogeneity of clinical manifestations and biomarkers of atypical parkinsonism. Nervnye bolezni. 2017; (2): 35–39. (in Russ).
  6. Morris H.R., Gibb G., Katzenschlager R. et al. Pathological, clinical and genetic heterogeneity in progressive supranuclear palsy. Brain 2002; 125(Pt 5): 969–975. PMID: 11960887.
  7. Ponomarev V.V. Neurodegenerative diseases. St.Petersburg: Foliant Publ.; 2013: 200 р. (in Russ).
  8. Fedotova E.Yu., Chechetkin A.O., Ivanova-Smolenskaya I.A., Illarioshkin S.N. The case of progressive supranuclear palsy with corticobasal syndrome. Nervnye bolezni. 2009; (2): 38–43. (in Russ.).
  9. Williams D.R., De Silva R., Paviour D.C. et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 2005; 128(Pt 6): 1247–1258. doi: 10.1093/brain/awh488. PMID: 15788542.
  10. Respondek G., Stamelou M., Kurz C. et al. The phenotypic spectrum of progressive supranuclear palsy: A retrospective multicenter study of 100 definite cases. Mov Disord 2014; 29(14): 1758–1766. doi: 10.1002/mds.26054. PMID: 25370486.
  11. Irwin D.J. Tauopathies as clinicopathological entities. Parkinsonism Relat Disord 2016; 22(Suppl 1): S29–S33. doi: 10.1016/j.parkreldis.2015.09.020. PMID: 26382841.
  12. Dickson D.W., Ahmed Z., Algom A.A. et al. Neuropathology of variants of progressive supranuclear palsy. Curr Opin Neurol 2010; 23(4): 394–400. doi: 10.1097/WCO.0b013e32833be924. PMID: 20610990.
  13. Kawashima M., Miyake M., Kusumi M. et al. Prevalence of progressive supranuclear palsy in Yonago, Japan. Mov Disord 2004; 19: 1239–1240. doi: 10.1002/mds.20149. PMID: 15390010.
  14. Schrag A., Ben-Shlomo Y., Quinn N.P. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999; 354(9192): 1771–1775. PMID: 10577638.
  15. Im S.Y., Kim Y.E., Kim Y.J. Genetics of progressive supranuclear palsy. J Mov Disord 2015; 8(3): 122–129. doi: 10.14802/jmd.15033. PMID: 26413239.
  16. Litvan I., Agid Y., Calne D. et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of the NINDS-SPSP International Workshop. Neurology 1996; 47(1): 1–9. doi: 10.1212/WNL.47.1.1. PMID: 8710059.
  17. Höglinger G.U., Respondek G., Stamelou M. et al. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord 2017; 32(6): 853–864. doi: 10.1002/mds.26987. PMID: 28467028.
  18. Williams D.R., Lees A.J. What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)? Mov Disord 2010; 25(3): 357–362. doi: 10.1002/mds.22977. PMID: 20108379.
  19. Williams D.R., Holton J.L., Strand C. et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain 2007; 130(Pt 6): 1566–1576. doi: 10.1093/brain/awm104. PMID: 17525140.
  20. Armstrong M.J. Progressive supranuclear palsy: an update. Curr Neurol Neurosci Rep 2018; 18(3): 12. doi: 10.1007/s11910-018-0819-5. PMID: 29455271.
  21. Grijalvo-Perez A.M., Litvan I. Corticobasal degeneration. Semin Neurol 2014; 34(2): 160–173. doi: 10.1055/s-0034-1381734. PMID: 24963675.
  22. Josephs K.A., Petersen R.C., Knopman D.S. et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 2006; 66(1): 41–48. doi: 10.1212/01.wnl.0000191307.69661.c3. PMID: 16401843.
  23. Vasenina E.E., Veryugina N.I., Levin O.S. Modern concepts of diagnosis and treatment of frontotemporal dementia. Sovremennaja terapija v psihiatrii i nevrologii. 2015; (3): 26–34. (in Russ.).
  24. Pressman P.S., Miller B.L. Diagnosis and management of behavioral variant frontotemporal dementia. Biol Psychiatry 2014; 75(7): 574–581. doi: 10.1016/j.biopsych.2013.11.006. PMID: 24315411.
  25. Donker Kaat L., Boon A.J., Kamphorst W. et al. Frontal presentation in progressive supranuclear palsy. Neurology 2007; 69(8): 723–729. doi: 10.1212/01.wnl.0000267643.24870.26. PMID: 17709703.
  26. Kurz C., Ebersbach G., Respondek G. et al. An autopsy-confirmed case of progressive supranuclear palsy with predominant postural instability. Acta Neuropathol Commun 2016; 4(1): 120. doi: 10.1186/s40478-016-0391-7. PMID: 27842578.

Supplementary files

Supplementary Files
Action
1. JATS XML

Copyright (c) 2018 Tappakhov A.A., Popova T.E., Govorova T.G., Petrova A.Y.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77-83204 от 12.05.2022.


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies