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Progressive supranuclear palsy


Other Names for this Disease

  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is progressive supranuclear palsy?

What are the signs and symptoms of progressive supranuclear palsy?


What causes progressive supranuclear palsy?


How is progressive supranuclear palsy diagnosed?


How might progressive supranuclear palsy be treated?


What is the long-term outlook for people with progressive supranuclear palsy?

What is progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) is a movement disorder caused by damage to certain nerve cells in the brain. It affects brain cells that control the movement of the eyes. This leads to serious and permanent problems with balance and the way affected people walk. It usually occurs in middle-aged or elderly people. Symptoms are very different in each person, but may include personality changes; and speech, vision or swallowing problems. Doctors sometimes confuse PSP with Parkinson's disease or Alzheimer's disease.[1] In most cases, the genetic cause of PSP is unknown. Rarely, the disease is known to result from mutations in the MAPT gene.[2] PSP has no effective treatments, but some symptoms may be managed with medication.[1]
Last updated: 8/28/2014

What are the signs and symptoms of progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) displays a wide range of symptoms. The pattern of signs and symptoms can be quite different among affected people. The most frequent first symptom of is a loss of balance while walking. Affected people may have unexplained falls, or a stiffness and awkwardness in gait. Sometimes the falls are described as attacks of dizziness. This often prompts suspicion of an inner ear problem.[3]

Other common early symptoms are changes in personality (such as a loss of interest regular activities); increased irritability; cantankerousness; and forgetfulness. Affected people may suddenly laugh or cry for no apparent reason; they may also be apathetic, or have occasional angry outbursts.[3] 

As the disease progresses, most people will begin to develop a blurring of vision and problems controlling eye movement. Eye problems usually offer the first definitive clue that PSP is the diagnosis. Those with PSP have trouble shifting their gaze downward, and/or controlling their eyelids. This can lead to involuntary closing of the eyes, prolonged or infrequent blinking, or difficulty in opening the eyes.[3] Another common visual problem is an inability to maintain eye contact during a conversation. This can give the mistaken impression that the affected person is hostile or uninterested.[3]

Weakened movements of the mouth, tongue and throat can lead to slurred speech and difficulty swallowing. The inability of throat muscles to create a watertight seal outside the person's lungs often results in aspiration pneumonia - the most common cause of death in people with PSP.[3]

Last updated: 8/28/2014

What causes progressive supranuclear palsy?

The signs and symptoms of progressive supranuclear palsy (PSP) appear to be related to abnormalities in the tau protein. The defective tau protein in affected people forms clumps within neurons and other brain cells. Cells in brain structures responsible for coordinating movement gradually die, causing the movement abnormalities and other features of PSP.[2]

In most cases, the underlying cause of PSP remains unknown. In a few cases, PSP has been associated with mutations in the MAPT gene. Researchers suspect that other genetic and environmental factors also contribute to PSP, but these factors have not yet been identified.[2]
Last updated: 8/28/2014

How is progressive supranuclear palsy diagnosed?

Progressive supranuclear palsy (PSP) is often hard to diagnose because its symptoms can mimick those of other, more common movement disorders. It may also be hard to diagnose because some of the most characteristic symptoms may develop late, or not at all. The key to diagnosing PSP is identifying early gait instability and difficulty moving the eyes (the hallmark of the disease), as well as ruling out other conditions, some of which are treatable.[3]

A neurological exam in a person with PSP may show the following:[1][3]

  • Symptoms of disequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness
  • Visual complaints including blurred vision, difficulty looking up or down, double vision, light sensitivity, burning eyes, or other eye problems
  • Slurred speech
  • Various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood
Last updated: 8/28/2014

How might progressive supranuclear palsy be treated?

There is currently no effective treatment for progressive supranuclear palsy, although scientists are searching for better ways to manage the disease. In some patients the slowness, stiffness, and balance problems may respond to antiparkinsonian agents such as levodopa, or levodopa combined with anticholinergic agents or amantadine, but the effect is usually temporary. The speech, vision, and swallowing difficulties usually do not respond to any drug treatment.[3]

Another group of drugs that has been of some modest success in progressive supranuclear palsy are antidepressant medications. The most commonly used of these drugs are fluoxetine (Prozac), amitriptyline (Elavil), and imipramine (Tofranil). The benefit of these drugs seems not to be related to their ability to relieve depression.[3]

Non-drug treatment for progressive supranuclear palsy can take many forms. Patients frequently use weighted walking aids because of their tendency to fall backward. Bifocals or special glasses called prisms are sometimes prescribed for patients to remedy the difficulty of looking down. Although formal physical therapy has not proven helpful in progressive supranuclear palsy, certain exercises can be done to keep the joints limber.[3]

A surgical procedure that may be necessary when there are swallowing disturbances is a gastrostomy. A gastrostomy (or a jejunostomy) is a minimally invasive procedure which is performed when the patient has difficulty swallowing or when severe choking is a definite risk. This surgery involves the placement of a tube through the skin of the abdomen into the stomach (intestine) for feeding purposes.[3]

Additional information about treatment of progressive supranuclear palsy can be obtained through the following web pages developed by WE MOVE and Cure PSP: 
http://www.wemove.org/psp/psp_tre.html
http://www.psp.org/materials/disease_management.pdf
Last updated: 3/12/2012

What is the long-term outlook for people with progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) gets progressively worse. The condition predisposes people to serious complications such as pneumonia secondary to difficulty in swallowing (dysphagia).  Pneumonia is the most common cause of death. Other complications that people experience include choking, head injury, and fractures caused by falls. With good attention to medical and nutritional needs, it is possible for most affected people to live a decade or more after the first symptoms of the disease appear.[3]
Last updated: 8/28/2014

References
  1. Progressive supranuclear palsy. MedlinePlus Medical Encyclopedia. May 2010; http://www.nlm.nih.gov/medlineplus/ency/article/000767.htm. Accessed 8/18/2011.
  2. Progressive supranuclear palsy. Genetics Home Reference. March 2011; http://ghr.nlm.nih.gov/condition/progressive-supranuclear-palsy. Accessed 9/7/2012.
  3. Progressive Supranuclear Palsy Fact Sheet. National Institute for Neurological Disorders and Stroke (NINDS). August 16, 2011; http://www.ninds.nih.gov/disorders/psp/detail_psp.htm. Accessed 8/18/2011.


Other Names for this Disease
  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.