Progressive Supranuclear Palsy Treatment & Management

  • Author: Eric R Eggenberger, DO, MS, FAAN; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Apr 11, 2012
 

Approach Considerations

Treatment of progressive supranuclear palsy (PSP) is challenging at best. Only a few patients respond to dopaminergic or anticholinergic drugs, and responses often are short-lived and incomplete. No medication is effective in halting the progression of the disease; however, several medications, including dopamine agonists, tricyclic antidepressants, and methysergide, may provide modest symptomatic improvement with respect to some of the clinical features.

Electroconvulsive therapy (ECT) may ameliorate motor symptoms in some patients with PSP. However, long hospitalizations and significant adverse effects (eg, confusion) limit the usefulness of ECT.

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Pharmacologic Therapy

The combination of carbidopa and levodopa generally produces no dramatic symptomatic improvement in patients with PSP, in sharp contrast with its effect in patients with idiopathic Parkinson disease. Accordingly, administration of carbidopa-levodopa may serve as a diagnostic test to help eliminate the possibility of Parkinson disease.

Some clinicians think that bromocriptine may have somewhat greater effect in individuals with PSP, though the effect is modest and short-lived in most patients. Tricyclic antidepressants have also been used. Other medications that have been tried with limited success include amantadine and trazodone.

OnabotulinumtoxinA has been found to be useful in the treatment of rigidity (nuchal rigidity in particular) and dystonia (eg, blepharospasm, bruxism, and focal limb dystonia).[47] It may also be useful for sialorrhea.

A small phase II clinical trial using coenzyme Q10 in patients with PSP showed modest clinical improvement in the short term.[48]

Chronic conjunctivitis is common in individuals with PSP because of the reduced blink rate in these patients. It can be treated with applications of methylcellulose or methyl alcohol drops in the eyes.

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Consultations

Consultation with a neuro-ophthalmologist or an ophthalmologist may help define the cause and outline the treatment of symptoms such as episodic diplopia.

Consultation with a rehabilitation medicine specialist may assist in maximizing gait stability and safety.

When swallowing starts to become affected, consultation with a speech therapist may help in modifying the diet.

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Diet and Activity

Patients should follow a well-balanced diet. When feeding becomes impossible because of dysphagia and the high risk of bronchoaspiration, patients should be encouraged to accept gastrostomy as a good option.

A double-blind, randomized, placebo-controlled, phase II trial found that supplemental coenzyme Q10, 5 mg/kg/day for 6 weeks, produced favorable changes in cerebral energy metabolites and slight but significant improvement in some measures of motor and neuropsychological dysfunction.[48]

Gait disturbances and falls are 2 of the major causes of disability in individuals with PSP. Physiotherapy after gait analysis, occupational therapy to find the best solutions to improve mobility, safe exercise programs, and appropriate mobility aids may decrease falls and related morbidity.

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Long-Term Monitoring

To help the patient and family adjust to the changing needs incurred by PSP, close follow-up care should be provided, with particular attention to potential adverse effects related to any medication trials. Often, family-directed education is the most valuable component of clinical visits. Patients and families often benefit from contact with a PSP support group (see Patient Education).

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.[49]

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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Eric R Eggenberger, DO, MS, FAAN  Professor, Vice-Chairman, Department of Neurology and Ophthalmology, Colleges of Osteopathic Medicine and Human Medicine, Michigan State University; Director of Michigan State University Ocular Motility Laboratory; Director of National Multiple Sclerosis Society Clinic, Michigan State University

Eric R Eggenberger, DO, MS, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Osteopathic Association, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Coauthor(s)

David Clark, DO  Chief Neurology Resident, Department of Neurology and Ophthalmology, Michigan State University College of Human Medicine

David Clark, DO is a member of the following medical societies: American Academy of Neurology and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Additional Contributors

Nestor Galvez-Jimenez, MD, MSc, MHA Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Zeba F Vanek, MD, MBBS, DCN Associate Professor of Neurology, David Geffen School of Medicine at UCLA; Director, UCLA Spasticity Clinic

Zeba F Vanek, MD, MBBS, DCN is a member of the following medical societies: Movement Disorders Society

Disclosure: Nothing to disclose.

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Sagittal T1-weighted image shows atrophy of midbrain, preservation of pontine volume, and atrophy of the tectum, suggestive of progressive supranuclear palsy (Steele-Olszewski-Richardson disease).
Characteristic facial appearance of patient with progressive supranuclear palsy.
 
 
 
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