Steele-Richardson-Olszewski Syndrome
Table of Contents (click to jump to sections)
What is Progressive Supranuclear Palsy?Is there any treatment?What is the prognosis?What research is being done?OrganizationsPublicaciones en Español
Additional resources from MEDLINEplus
What is Progressive Supranuclear Palsy?
Progressive supranuclear palsy (PSP) is a rare brain disorder that causes serious and permanent problems with control of gait
and balance. The most obvious sign of the disease is an inability to aim the eyes properly, which occurs because of lesions
in the area of the brain that coordinates eye movements. Some patients describe this effect as a blurring. PSP patients often
show alterations of mood and behavior, including depression and apathy as well as progressive mild dementia. It must be emphasized
that the pattern of signs and symptoms can be quite different from person to person. The symptoms of PSP 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.
PSP is often misdiagnosed because some of its symptoms are very much like those of Parkinson's disease, Alzheimer's disease,
and more rare neurodegenerative disorders, such as Creutzfeldt-Jakob disease. The key to establishing the diagnosis of PSP
is the identification of 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. Although PSP gets progressively worse, no one dies from PSP itself.
Is there any treatment?
There is currently no effective treatment for PSP, although scientists are searching for better ways to manage the disease.
In some patients the slowness, stiffness, and balance problems of PSP may respond to antiparkinsonian agents such as levodopa,
or levodopa combined with anticholinergic agents, but the effect is usually temporary. The speech, vision, and swallowing
difficulties usually do not respond to any drug treatment.. Another group of drugs that has been of some modest success in
PSP are antidepressant medications. The most commonly used of these drugs are Prozac, Elavil, and Tofranil. The anti-PSP benefit
of these drugs seems not to be related to their ability to relieve depression. Non-drug treatment for PSP 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 PSP patients to remedy the difficulty of looking down. Formal physical therapy is of no
proven benefit in PSP, but certain exercises can be done to keep the joints limber. A surgical procedure, a gastrostomy, may
be necessary when there are swallowing disturbances. This surgery involves the placement of a tube through the skin of the
abdomen into the stomach (intestine) for feeding purposes.
What is the prognosis?
PSP gets progressively worse but is not itself directly life-threatening. It does, however, predispose patients to serious
complications such as pneumonia secondary to difficulty in swallowing (dysphagia). The most common complications are choking
and pneumonia, head injury, and fractures caused by falls. The most common cause of death is pneumonia. With good attention
to medical and nutritional needs, however, most PSP patients live well into their 70s and beyond.
What research is being done?
Therapeutic trials with free radical scavengers (agents that can get rid of potentially harmful free radicals) are being planned
for the future. Research is ongoing on Parkinson's and Alzheimer's diseases. Better understanding of those common, related
disorders will go a long way toward solving the problem of PSP, just as studying PSP may help shed light on Parkinson's and
Alzheimer's diseases.
Select this link
to view a list of studies currently seeking patients.
Organizations
CUREPSP (Society for Progressive Supranuclear Palsy)
Executive Plaza III
11350 McCormick Road, Ste. 906
Hunt Valley,
MD
21031
info@curepsp.org
http://www.curepsp.org
Tel: 410-785-7004
800-457-4777
Fax: 410-785-7009
|
WE MOVE (Worldwide Education & Awareness for Movement Disorders)
204 West 84th Street
New York,
NY
10024
wemove@wemove.org
http://www.wemove.org
Tel: 212-875-8312 866-546-3136
Fax: 212-875-8389
|
Publicaciones en Español
-
Parálisis Supranuclear ProgresivaInformation on Paralisis Supranuclear Progresiva/Spanish-language fact sheet on Progressive Supranuclear Palsy. Compiled by
the National Institute of Neurological Disorders and Stroke (NINDS).
Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by
or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice
on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined
that patient or is familiar with that patient's medical history.
All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.
Return to top
Last updated April 24, 2007
