Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Progressive Supranuclear Palsy

  • Alexander I. TrösterEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_522

Synonyms

Steele-Richardson-Olszewski syndrome

Definition

Progressive supranuclear palsy (PSP) is a late onset (after age 40 years), progressive neurodegenerative condition traditionally considered a movement disorder. In its classic form, the movement disorder is characterized by early postural instability and falls. Patients with PSP may show the “rocket sign” arising rapidly from a chair just to fall back into it. Other clinical features include symmetrical bradykinesia and greater axial than limb rigidity. Oculomotor problems usually begin with slowing of vertical saccades, with eventual vertical (greater downward than upward) gaze restriction. Problems with downward gaze can manifest in messy eating (hence the “messy tie” sign). Diminished blinking can lead to subjective complaints of blurred vision and sore or tired eyes, and patients may show eyelid apraxia (inability to voluntarily move the eyelids). Speech can be affected early in PSP and may involve hypophonia (reduced volume)...

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References and Readings

  1. Brown, R. G., Lacomblez, L., Lanwehmeyer, B. G., Bak, T., Uttner, I., Dubois, B., Agid, Y., Ludolph, A., Bensimon, G., Payan, C., Leigh, N. P., & NIPPS Study Group. (2010). Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain, 133, 2382–2393.CrossRefPubMedPubMedCentralGoogle Scholar
  2. Colosimo, C., Bak, T. H., Bollogna, M., & Berardelli, A. (2014). Fifty years of progressive supranuclear palsy. Journal of Neurology, Neurosurgery and Psychiatry, 85, 936–942.CrossRefGoogle Scholar
  3. Eschlböck, S., Krismer, F., & Wenning, G. K. (2016). Interventional trials in atypical parkinsonism. Parkinsonism and Related Disorders, 22(Suppl 1), S82–S92.CrossRefPubMedPubMedCentralGoogle Scholar
  4. Gerstenecker, A. (2017). The Neuropsychology (Broadly Conceived) of Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration. Archives of Clinical Neuropsychology 32(7), 861–875.CrossRefPubMedPubMedCentralGoogle Scholar
  5. Gerstenecker, A., Duff, K., Mast, B., Litvan, I., & ENGENE-PSP Study Group. (2013a). Behavioral abnormalities in progressive supranuclear palsy. Psychiatry Research, 210, 1205–1210.CrossRefGoogle Scholar
  6. Gerstenecker, A., Mast, B., Duff, K., Ferman, T. J., Litvan, I., & ENGENE-PSP Study Group. (2013b). Executive dysfunction is the primary cognitive impairment in progressive supranuclear palsy. Archives of Clinical Neuropsychology, 28, 104–113.CrossRefGoogle Scholar
  7. Lee, Y. E., Williams, D. R., & Anderson, J. F. (2016). Frontal deficits differentiate progressive supranuclear palsy from Parkinson’s disease. Journal of Neuropsychology, 10, 1–14.CrossRefGoogle Scholar
  8. Litvan, I., Paulsen, J. S., Mega, M. S., & Cummings, J. L. (1998). Neuropsychiatric assessment of patients with hyperkinetic and hypokinetic movement disorders. Archives of Neurology, 55, 1313–1319.CrossRefGoogle Scholar
  9. Respondek, G., Stamelou, M., Kurz, C., Ferguson, L. W., Rajput, A., Chiu, W. Z., et al. (2014). The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. Movement Disorders, 29, 1758–1766.CrossRefGoogle Scholar
  10. Schrag, A., Selai, C., Davis, J., Lees, A. J., Jahanshahi, M., & Quinn, N. (2003). Health-related quality of life in patients with progressive supranuclear palsy. Movement Disorders, 18, 1464–1469.CrossRefGoogle Scholar
  11. Tröster, A. I. (Ed.). (2015). Clinical neuropsychology and cognitive neurology of Parkinson’s disease and other movement disorders. New York: Oxford University Press.Google Scholar
  12. Tröster, A. I., & Garrett, R. (2018). Parkinson’s disease and other movement disorders. In J. E. Morgan & J. H. Ricker (Eds.), Textbook of clinical neuropsychology (2nd ed.) (pp.507-559). New York: Routledge.Google Scholar
  13. Williams, D. R., de Silva, R., Paviour, D. C., Pittman, A., Watt, H. C., Kilford, L., et al. (2005). Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain, 128, 1247–1258.CrossRefGoogle Scholar
  14. Zampieri, C., & Di Fabio, R. P. (2006). Progressive supranuclear palsy: Disease profile and rehabilitation strategies. Physical Therapy, 86, 870–880.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Clinical Neuropsychology and Center for NeuromodulationBarrow Neurological InstitutePhoenixUSA