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Progressive supranuclear palsy

Dementia - nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclear

Progressive supranuclear palsy (PSP) is a movement disorder that occurs from damage to certain nerve cells in the brain.

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Central nervous system and peripheral nervous system

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Causes

PSP is a condition that causes symptoms similar to those of Parkinson disease.

It involves damage to many cells of the brain. Many areas are affected, including the part of the brainstem where cells that control eye movement are located. The area of the brain that controls steadiness when you walk is also affected. The frontal lobes of the brain are also affected, leading to personality changes.

The cause of the damage to the brain cells is unknown. PSP gets worse over time.

People with PSP have deposits in brain tissues that look like those found in people with Alzheimer disease. There is a loss of tissue in most areas of the brain and in some parts of the spinal cord.

The disorder is most often seen in people over 60 years old, and is somewhat more common in men.

Symptoms

Symptoms may include any of the following:

Exams and Tests

An exam of the nervous system (neurologic examination) may show:

The health care provider may do the following tests to rule out other diseases:

Treatment

The goal of treatment is to control symptoms. There is no known cure for PSP.

Medicines such as levodopa may be tried. These drugs raise the level of a brain chemical called dopamine. Dopamine is involved in the control of movement. The medicines may reduce some symptoms, such as rigid limbs or slow movements for a time. But they are usually not as effective as they are for Parkinson disease.

Many people with PSP will eventually need around-the-clock care and monitoring as they lose brain functions.

Outlook (Prognosis)

Treatment sometimes can reduce symptoms for a while, but the condition will get worse. Brain function will decline over time. Death commonly occurs in 5 to 7 years.

Newer drugs are being studied to treat this condition.

Possible Complications

Complications of PSP include:

When to Contact a Medical Professional

Call your provider if you often fall, and if you have a stiff neck/body, and vision problems.

Also, call if a loved one has been diagnosed with PSP and the condition has declined so much that you can no longer care for the person at home.

Related Information

Parkinson disease
Dementia
Alzheimer disease
Myelin
Deep vein thrombosis
Community-acquired pneumonia in adults

References

Jankovic J. Parkinson disease and other movement disorders. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 96.

Ling H. Clinical approach to progressive supranuclear palsy. J Mov Disord. 2016;9(1):3-13. PMID: 26828211 www.ncbi.nlm.nih.gov/pubmed/26828211.

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Review Date: 4/30/2018  

Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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