eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases
Progressive Supranuclear Palsy: Follow-up
Updated: Jan 5, 2010
Follow-up
Further Outpatient Care
- To help the patient and family adjust to the changing needs incurred by PSP, provide close follow-up care. Be vigilant for adverse effects related to any medication trials. Often, family-directed education is the most valuable component of clinical visits.
- Physical therapy and rehabilitation medicine involvement may help maximize ambulation safety and facilitate instruction in the use of a walker, wheelchair, or other aids.
- Preliminary evidence suggests that the combination of balance training complemented with eye movement and visual awareness exercises can be beneficial for enhancing suppression of fixation and gaze shift in patients with PSP.48
- Patients and families often benefit from contact with a PSP support group (see Patient Education).
Complications
The primary complications of PSP relate to impaired balance, decreased cognition, and immobility in late disease.
- Complications related to falls include orthopedic injury and other posttraumatic problems.
- Immobility in late disease leads to infectious complications such as pneumonia, urinary tract infection, and sepsis.
Prognosis
The disease runs a progressive and fatal course over 6-10 years in most patients (see Mortality/Morbidity).
Patient Education
- The primary educational aspects of PSP are directed at the patient and family to foster an understanding of the disease, prognosis, potential complications, and coping mechanisms.
- Many patients and families benefit from contact with a support group.
- For excellent patient education resources, visit eMedicine's Dementia Center. Also, see eMedicine's patient education article Progressive Supranuclear Palsy.
Miscellaneous
Medicolegal Pitfalls
Misdiagnosis is always a potential medicolegal pitfall, especially in diseases without objective diagnostic tests. Consider treatable disorders such as idiopathic Parkinson disease. Offering the patient and family a second opinion in the setting of an untreatable fatal illness is always worthy of consideration.
More on Progressive Supranuclear Palsy |
| Overview: Progressive Supranuclear Palsy |
| Differential Diagnoses & Workup: Progressive Supranuclear Palsy |
| Treatment & Medication: Progressive Supranuclear Palsy |
Follow-up: Progressive Supranuclear Palsy |
| References |
| « Previous Page |
References
Golbe LI. Progressive Supranuclear Palsy. Curr Treat Options Neurol. Nov 2001;3(6):473-477. [Medline].
Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. Mar 2009;8(3):270-9. [Medline].
Liao K, Wagner J, Joshi A, Estrovich I, Walker MF, Strupp M, et al. Why do patients with PSP fall? Evidence for abnormal otolith responses. Neurology. Mar 4 2008;70(10):802-9. [Medline].
Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy. Neurology. Jul 1988;38(7):1031-4. [Medline].
Jackson JA, Jankovic J, Ford J. Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Ann Neurol. Mar 1983;13(3):273-8. [Medline].
Mastaglia FL, Grainger K, Kee F, et al. Progressive supranuclear palsy (the Steele-Richardson-Olszewski syndrome) clinical and electrophysiological observations in eleven cases. Proc Aust Assoc Neurol. 1973;10(0):35-44. [Medline].
Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson- Olszewski syndrome (progressive supranuclear palsy). Neurology. Jul 1986;36(7):1005-8. [Medline].
Kristensen MO. Progressive supranuclear palsy--20 years later. Acta Neurol Scand. Mar 1985;71(3):177-89. [Medline].
Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogenous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol. Apr 1964;10:333-59. [Medline].
Litvan I, Mangone CA, McKee A, et al. Natural history of progressive supranuclear palsy (Steele-Richardson- Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. J Neurol Neurosurg Psychiatry. Jun 1996;60(6):615-20. [Medline].
Barclay CL, Lang AE. Dystonia in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry. Apr 1997;62(4):352-6. [Medline].
Gibb WR, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain. Oct 1989;112 ( Pt 5):1171-92. [Medline].
Sakakibara R, Hattori T, Tojo M, et al. Micturitional disturbance in progressive supranuclear palsy. J Auton Nerv Syst. Nov 1993;45(2):101-6. [Medline].
Tolosa E, Espuna M, Valls J. Bladder dysfunction in PSP and other parkinsonian disorders. Mov Disord. 1997;12:272.
Josephs KA, Duffy JR. Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy. Curr Opin Neurol. Dec 2008;21(6):688-92. [Medline].
Cooper AD, Josephs KA. Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: a prospective study. Parkinsonism Relat Disord. Jan 2009;15(1):59-61. [Medline].
Schmidt C, Herting B, Prieur S, Junghanns S, Schweitzer K, Globas C. Pupil diameter in darkness differentiates progressive supranuclear palsy (PSP) from other extrapyramidal syndromes. Mov Disord. Oct 31 2007;22(14):2123-6. [Medline].
Litvan I, Mega MS, Cummings JL, Fairbanks L. Neuropsychiatric aspects of progressive supranuclear palsy. Neurology. Nov 1996;47(5):1184-9. [Medline].
Litvan I, Agid Y, Jankovic J, et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology. Apr 1996;46(4):922-30. [Medline].
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. Jul 1996;47(1):1-9. [Medline].
Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. Nov 1994;44(11):2015-9. [Medline].
Golbe LI, Rubin RS, Cody RP, et al. Follow-up study of risk factors in progressive supranuclear palsy. Neurology. Jul 1996;47(1):148-54. [Medline].
Tetrud JW, Golbe LI, Forno LS, Farmer PM. Autopsy-proven progressive supranuclear palsy in two siblings. Neurology. Apr 1996;46(4):931-4. [Medline].
Kaat LD, Boon AJ, Azmani A, Kamphorst W, Breteler MM, Anar B, et al. Familial aggregation of parkinsonism in progressive supranuclear palsy. Neurology. Jul 14 2009;73(2):98-105. [Medline].
de Yebenes JG, Sarasa JL, Daniel SE, Lees AJ. Familial progressive supranuclear palsy. Description of a pedigree and review of the literature. Brain. Oct 1995;118 ( Pt 5):1095-103. [Medline].
Conrad C, Andreadis A, Trojanowski JQ, et al. Genetic evidence for the involvement of tau in progressive supranuclear palsy. Ann Neurol. Feb 1997;41(2):277-81. [Medline].
Borroni B, Malinverno M, Gardoni F, Alberici A, Parnetti L, Premi E, et al. Tau forms in CSF as a reliable biomarker for progressive supranuclear palsy. Neurology. Nov 25 2008;71(22):1796-803. [Medline].
Drayer BP, Olanow W, Burger P, et al. Parkinson plus syndrome: diagnosis using high field MR imaging of brain iron. Radiology. May 1986;159(2):493-8. [Medline].
Schonfeld SM, Golbe LI, Sage JI, et al. Computed tomographic findings in progressive supranuclear palsy: correlation with clinical grade. Mov Disord. 1987;2(4):263-78. [Medline].
Stern MB, Braffman BH, Skolnick BE, et al. Magnetic resonance imaging in Parkinson''s disease and parkinsonian syndromes. Neurology. Nov 1989;39(11):1524-6. [Medline].
Savoiardo M, Girotti F, Strada L, Ciceri E. Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders. J Neural Transm Suppl. 1994;42:93-110. [Medline].
Paviour DC, Price SL, Stevens JM, et al. Quantitative MRI measurement of superior cerebellar peduncle in progressive supranuclear palsy. Neurology. Feb 22 2005;64(4):675-9. [Medline].
Righini A, Antonini A, De Notaris R, et al. MR imaging of the superior profile of the midbrain: differential diagnosis between progressive supranuclear palsy and Parkinson disease. AJNR Am J Neuroradiol. Jun-Jul 2004;25(6):927-32. [Medline].
Foster NL, Gilman S, Berent S, et al. Cerebral hypometabolism in progressive supranuclear palsy studied with positron emission tomography. Ann Neurol. Sep 1988;24(3):399-406. [Medline].
Foster NL, Gilman S, Berent S, et al. Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities. J Neurol Neurosurg Psychiatry. Aug 1992;55(8):707-13. [Medline].
Blin J, Baron JC, Dubois B, et al. Positron emission tomography study in progressive supranuclear palsy. Brain hypometabolic pattern and clinicometabolic correlations. Arch Neurol. Jul 1990;47(7):747-52. [Medline].
Mishina M, Ishii K, Mitani K, et al. Midbrain hypometabolism as early diagnostic sign for progressive supranuclear palsy. Acta Neurol Scand. Aug 2004;110(2):128-35. [Medline].
Brooks DJ, Ibanez V, Sawle GV, et al. Differing patterns of striatal 18F-dopa uptake in Parkinson''s disease, multiple system atrophy, and progressive supranuclear palsy. Ann Neurol. Oct 1990;28(4):547-55. [Medline].
Arnold G, Tatsch K, Oertel WH, et al. Clinical progressive supranuclear palsy: differential diagnosis by IBZM- SPECT and MRI. J Neural Transm Suppl. 1994;42:111-8. [Medline].
Aldrich MS, Foster NL, White RF, et al. Sleep abnormalities in progressive supranuclear palsy. Ann Neurol. Jun 1989;25(6):577-81. [Medline].
Santamaria J, Iranzo A. Alteraciones del sueno en los trastornos del movimiento. Neurologia. 1997;12 (Suppl 3):35-47.
Gross RA, Spehlmann R, Daniels JC. Sleep disturbances in progressive supranuclear palsy. Electroencephalogr Clin Neurophysiol. Jul 1978;45(1):16-25. [Medline].
Laffont F, Autret A, Minz M, Beillevaire T, Gilbert A, Cathala HP, et al. [Polygraphic sleep recordings in 9 cases of Steele-Richardson's disease (author's transl)]. Rev Neurol (Paris). Feb 1979;135(2):127-41. [Medline].
Sixel-Döring F, Schweitzer M, Mollenhauer B, Trenkwalder C. Polysomnographic findings, video-based sleep analysis and sleep perception in progressive supranuclear palsy. Sleep Med. Apr 2009;10(4):407-15. [Medline].
Polo KB, Jabbari B. Botulinum toxin-A improves the rigidity of progressive supranuclear palsy. Ann Neurol. Feb 1994;35(2):237-9. [Medline].
Stamelou M, Reuss A, Pilatus U, Magerkurth J, Niklowitz P, Eggert KM. Short-term effects of coenzyme Q10 in progressive supranuclear palsy: a randomized, placebo-controlled trial. Mov Disord. May 15 2008;23(7):942-9. [Medline].
Stamelou M, Reuss A, Pilatus U, Magerkurth J, Niklowitz P, Eggert KM, et al. Short-term effects of coenzyme Q10 in progressive supranuclear palsy: a randomized, placebo-controlled trial. Mov Disord. May 15 2008;23(7):942-9. [Medline].
Zampieri C, Di Fabio RP. Improvement of gaze control after balance and eye movement training in patients with progressive supranuclear palsy: a quasi-randomized controlled trial. Arch Phys Med Rehabil. Feb 2009;90(2):263-70. [Medline].
Albers DS, Augood SJ. New insights into progressive supranuclear palsy. Trends Neurosci. Jun 2001;24(6):347-53. [Medline].
Hamilton SR. Neuro-ophthalmology of movement disorders. Curr Opin Ophthalmol. Dec 2000;11(6):403-7. [Medline].
Jankovic J, Friedman DI, Pirozzolo FJ, McCrary JA. Progressive supranuclear palsy: motor, neurobehavioral, and neuro- ophthalmic findings. Adv Neurol. 1990;53:293-304. [Medline].
Kuniyoshi S, Riley DE, Zee DS, Reich SG, Whitney C, Leigh RJ. Distinguishing progressive supranuclear palsy from other forms of Parkinson's disease: evaluation of new signs. Ann N Y Acad Sci. Apr 2002;956:484-6. [Medline].
Laffont F, Autret A, Minz M, et al. [Polygraphic study of nocturnal sleep in three degenerative diseases: ALS, oligo-ponto-cerebellar atrophy, and progressive supranuclear palsy]. Waking Sleeping. Jan 1979;3(1):17-30. [Medline].
Leigh JR, Zee DS. The neurology of eye movements. 3rd ed. New York, NY:. Oxford University Press;1999:521-525.
Litvan I. Diagnosis and management of progressive supranuclear palsy. Semin Neurol. 2001;21(1):41-8. [Medline].
Litvan I, Hauw JJ, Bartko JJ, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol. Jan 1996;55(1):97-105. [Medline].
Mark MH. Lumping and splitting the Parkinson Plus syndromes: dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, and cortical-basal ganglionic degeneration. Neurol Clin. Aug 2001;19(3):607-27, vi. [Medline].
Nath U, Ben-Shlomo Y, Thomson RG, et al. The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain. Jul 2001;124(Pt 7):1438-49. [Medline].
Osaki Y, Ben-Shlomo Y, Lees AJ, et al. Accuracy of clinical diagnosis of progressive supranuclear palsy. Mov Disord. Feb 2004;19(2):181-9. [Medline].
Rafal RD, Friedman JH. Limb dystonia in progressive supranuclear palsy. Neurology. Sep 1987;37(9):1546-9. [Medline].
Schrag A, Selai C, Davis J, et al. Health-related quality of life in patients with progressive supranuclear palsy. Mov Disord. Dec 2003;18(12):1464-9. [Medline].
Tolosa E, Valldeoriola F. Progressive supranuclear palsy. In: Jankovic J, Tolosa E, eds. Parkinson's Disease and Movement Disorders. 3rd ed. Baltimore, Md: William & Wilkins; 1998:221-243.
Warmuth-Metz M, Naumann M, Csoti I, Solymosi L. Measurement of the midbrain diameter on routine magnetic resonance imaging: a simple and accurate method of differentiating between Parkinson disease and progressive supranuclear palsy. Arch Neurol. Jul 2001;58(7):1076-9. [Medline].
Further Reading
Keywords
Steele-Richardson-Olszewski syndrome, PSP, parkinsonism, neurodegenerative disease, imbalance, immobility, dementia, visual symptoms, dysphagia, dysarthria, vertical gaze palsy
Follow-up: Progressive Supranuclear Palsy