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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
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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 progressive supranuclear palsy?

Progressive supranuclear palsy 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 individuals walk. It usually occurs in middle-aged or elderly people. Symptoms are very different in each person, but may include personality changes, speech, vision and swallowing problems. Doctors sometimes confuse progressive supranuclear palsy with Parkinson's disease or Alzheimer's disease. Progressive supranuclear palsy has no cure and no effective treatments.[1] In most cases, the genetic cause of this condition is unknown. Rarely, the disease results from mutations in the MAPT gene.[2] 
Last updated: 8/18/2011

What are the signs and symptoms of progressive supranuclear palsy?

Progressive supranuclear palsy displays a wide range of symptoms. The pattern of signs and symptoms can be quite different from person to person. The most frequent first symptom of is a loss of balance while walking. Affected individuals may experience unexplained falls or a stiffness and awkwardness in gait. Sometimes the falls are described by the person experiencing them 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 in ordinary pleasurable activities or increased irritability, cantankerousness, and forgetfulness. Affected individuals may suddenly laugh or cry for no apparent reason; they may also be apathetic, or have occasional angry outbursts, also for no apparent reason.[3] 

As the disease progresses, most people will begin to develop a blurring of vision and problems controlling eye movement. In fact, eye problems usually offer the first definitive clue that progressive supranuclear palsy is the proper diagnosis. Those with progressive supranuclear palsy have trouble voluntarily shifting their gaze downward, and also can have trouble 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 patient 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 those with progressive supranuclear palsy.[3]

In rare cases, some affected individuals will notice shaking of the hands.[3]
Last updated: 8/18/2011

What causes progressive supranuclear palsy?

The symptoms of progressive supranuclear palsy are caused by a gradual deterioration of brain cells in a few tiny but important places at the base of the brain, in the region called the brainstem. However, scientists do not know what causes these brain cells to degenerate.[3] In most cases, the genetic cause of this condition is unknown. Rarely, the disease results from mutations in the MAPT gene.[2] 

There are, however, several theories about progressive supranuclear palsy's cause. One possibility is that a virus-like agent infects the body and takes years or decades to start producing visible effects. Another possibility is that random genetic mutations occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain.[3]

Another possible cause is cellular damage caused by free radicals, reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in mechanisms for clearing free radicals from the system, scientists suspect that, under certain circumstances, free radicals can react with and damage other molecules. More research is needed and underway to help the medical field better understand this condition and its cause.[3]
Last updated: 8/18/2011

How is progressive supranuclear palsy diagnosed?

Progressive supranuclear palsy is often difficult to diagnose because its symptoms can be very much like those of other, more common movement disorders, and because some of the most characteristic symptoms may develop late or not at all. The key to diagnosing progressive supranuclear palsy is identifying early gait instability and difficulty moving the eyes, the hallmark of the disease, as well as ruling out other similar disorders, some of which are treatable.[3]

An exam of the nervous system (neurological examination) may show features from the following categories from which primary complaints of this condition fall:[1][3]

  • Symptoms of disequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness
  • Visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble
  • Slurred speech
  • Various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood
Last updated: 3/12/2012

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

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.