Skip to main content

Advertisement

Log in

Progressive supranuclear palsy

  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

  • •Unfortunately, no drug treatment has been shown to benefit patients with progressive supranuclear palsy (PSP) more than modestly or briefly. The multiplicity of neuronal systems and synaptic physiologies that degenerate in this disorder makes a neurotransmitter-specific approach, such as neurotransmitter replacement or reuptake inhibition, unlikely to succeed. Nevertheless, some of these are worth trying in nearly every patient with PSP. Useful drug treatment may ultimately take the form of enhancers of mitochondrial energy metabolism, inducers of endogenous neurotrophic factors, and, even further in the future, inhibitors of tau aggregation or regulators of alternative splicing.

  • •The neurologist familiar with PSP can assist patients and their family by providing prognostic information, by avoiding unnecessary diagnostic tests, and by prescribing measures to minimize aspiration and falling, the two most dangerous complications of PSP.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. Steele JC, Richardson JC, Olszewski J: PSP: a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum, with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964, 10:333–359.

    PubMed  CAS  Google Scholar 

  2. Jankovic J: PSP: clinical and pharmacologic update. Neurol Clin 1984, 2:473–486.

    PubMed  CAS  Google Scholar 

  3. Schrag A, Ben-Shlomo Y, Quinn N: Prevalence of PSP and MSA: a cross-sectional study. Lancet 1999, 354:1771–1772. These authors scrutinized several British general practitioners’ records, extracting and examinging all patients whose previous medical encounters offered even the slightest suggestion of PSP. Of the six such cases that met PSP diagnositc criteria on the authors’ examination, none had previously been diagnosed with PSP. This demonstrates that most PSP remains undiagnosed.

    Article  PubMed  CAS  Google Scholar 

  4. Davis PH, Golbe LI, Duvoisin RC, Schoenberg BS: Risk factors for PSP. Neurology 1988, 38:1546–1552.

    PubMed  CAS  Google Scholar 

  5. Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC: Prevalence and natural history of PSP. Neurology 1988, 38:1031–1034.

    PubMed  CAS  Google Scholar 

  6. Golbe LI: Progressive supranuclear palsy. In Movement Disorders: Neurologic Principles and Practice. Edited by Watts R, Koller W. New York: McGraw-Hill; 1997:279–295.

    Google Scholar 

  7. Golbe LI: Progressive supranuclear palsy. In Differential Diagnosis and Treatment of Movement Disorders. Edited by Tolosa E, Koller WC, Gershanik OS. Boston: Butterworth Heinemann; 1998:27–38.

    Google Scholar 

  8. Baker M, Litvan I, Houlden H, et al.: Association of an extended haplotype in the tau gene with PSP. Hum Mol Genet 1999, 8:711–715. This and the next reference (reference [9]) show that a genetic variant in or near the tau gene at chromosome 17q21 is present in nearly all patients with PSP, but in only about half of control individuals. This has since prompted searches for the precise genetic defect and for the other factors that are necessary, but not individually sufficient to cause the disease.

    Article  PubMed  CAS  Google Scholar 

  9. Higgins JJ, Adler RL, Loveless JM: Mutational analysis of the tau gene in PSP. Neurology 1999, 53:1421–1424. This and the previous reference (reference [8]) show that a genetic variant in or near the tau gene at chromosome 17q21 is present in nearly all patients with PSP, but in only about half of control individuals. This has since prompted searches for the precise genetic defect and for the other factors that are necessary, but not individually sufficient to cause the disease.

    PubMed  CAS  Google Scholar 

  10. Foster NL, Aldrich MS, Bluemlein L, et al.: Failure of cholinergic agonist RS-86 to improve cognition and movement in PSP despite effects on sleep. Neurology 1989, 39:257–261.

    PubMed  CAS  Google Scholar 

  11. Litvan I, Blesa R, Clark K, et al.: Pharmacological evaluation of the cholinergic system in PSP. Ann Neurol 1994, 36:55–61.

    Article  PubMed  CAS  Google Scholar 

  12. Jackson JA, Jankovic J, Ford J: PSP: clinical features and response to treatment in 16 patients. Ann Neurol 1983, 13:273–278.

    Article  PubMed  CAS  Google Scholar 

  13. Kompoliti K, Goetz CG, Litvan I, et al.: Pharmacological therapy in PSP. Arch Neurol 1998, 55:1099–1102.

    Article  PubMed  CAS  Google Scholar 

  14. Newman GC: Treatment of PSP with tricyclic antidepressants. Neurology 1985, 35:1189–1193.

    PubMed  CAS  Google Scholar 

  15. Nieforth KA, Golbe LI: Retrospective study of drug response in 87 patients with PSP. Clin Neuropharmacol 1993, 16:338–346.

    Article  PubMed  CAS  Google Scholar 

  16. Neophytides A, Lieberman AN, Goldstein M, et al.: The use of lisuride, a potent dopamine and serotonin agonist, in the treatment of PSP. J Neurol Neurosurg Psychiatry 1982, 45:261–263.

    Article  PubMed  CAS  Google Scholar 

  17. Weiner WJ, Minagar A, Shulman LM: Pramipexole in PSP. Neurology 1999, 52:873–874.

    PubMed  CAS  Google Scholar 

  18. Lepore FE: PSP. In Neuro-Ophthalmological Disorders: Diagnostic Workup and Management. Edited by Tusa RJ, Newman SA. New York: Marcel Dekker; 2001.

    Google Scholar 

  19. Piccione F, Mancini E, Tonin P, Bizzarini M: Botulinum toxin treatment of apraxia of eyelid opening in PSP: report of two cases. Arch Phys Med Rehab 1997, 78:525–529.

    Article  CAS  Google Scholar 

  20. Polo KB, Jabbari B: Botulinum toxin-A improves the rigidity of progressive supranculear palsy. Ann Neurol 1994, 35:237–239.

    Article  PubMed  CAS  Google Scholar 

  21. Sosner J, Wall GC, Sznajder J: PSP: clinical presentation and rehabilitation of two patients. Arch Phys Med Rehab 1993, 74:537–539.

    Article  CAS  Google Scholar 

  22. Gaudet RJ, Kessler II: Transparently blinded trials of methysergide. N Engl J Med 1987, 316:279–280.

    Article  PubMed  CAS  Google Scholar 

  23. Daniele A, Moro E, Bentivoglio AR: Zolpidem in PSP. N Engl J Med 1999, 341:543–544.

    Article  PubMed  CAS  Google Scholar 

  24. Koller WC, Morantz R, Vetere-Overfield B, Waxman M: Autologous adrenal medullary transplant in PSP. Neurology 1989, 39:1066–1068.

    PubMed  CAS  Google Scholar 

  25. Hauser RA, Trehan R: Initial experience with electroconvulsive therapy for PSP. Mov Disord 1994, 9:466–468.

    Article  Google Scholar 

  26. Rasmussen K, Abrams R: Treatment of Parkinson’s disease with electroconvulsive therapy. Psychiatr Clin North Am 1991, 14:925–933.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Golbe, L.I. Progressive supranuclear palsy. Curr Treat Options Neurol 3, 473–477 (2001). https://doi.org/10.1007/s11940-001-0010-0

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11940-001-0010-0

Keywords

Navigation