Progressive supranuclear palsy
Other Names for this Disease
- Familial progressive supranuclear palsy (type)
- Steele-Richardson-Olszewski Syndrome
- Supranuclear palsy, progressive
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?
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.
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. 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.
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.In rare cases, some affected individuals will notice shaking of the hands.
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.
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.
- 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
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.
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.
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.Additional information about treatment of progressive supranuclear palsy can be obtained through the following web pages developed by WE MOVE and Cure PSP:
- Progressive supranuclear palsy. MedlinePlus Medical Encyclopedia. May 2010; http://www.nlm.nih.gov/medlineplus/ency/article/000767.htm. Accessed 8/18/2011.
- Progressive supranuclear palsy. Genetics Home Reference. March 2011; http://ghr.nlm.nih.gov/condition/progressive-supranuclear-palsy. Accessed 9/7/2012.
- 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.