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$Unique_ID{BRK04139}
$Pretitle{}
$Title{Progressive Supranuclear Palsy}
$Subject{Progressive Supranuclear Palsy PSP Steele-Richardson-Olszewski
syndrome Nuchal Dystonia Dementia syndrome}
$Volume{}
$Log{}
Copyright (C) 1986, 1987, 1988, 1990, 1992 National Organization for Rare
Disorders, Inc.
287:
Progressive Supranuclear Palsy
** IMPORTANT **
It is possible the main title of the article (Progressive Supranuclear
Palsy) is not the name you expected. Please check the SYNONYMS listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
PSP
Steele-Richardson-Olszewski syndrome
Nuchal Dystonia Dementia syndrome
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Progressive Supranuclear Palsy (PSP) is a neurologic disorder associated
with spastic weakness of muscles affected by the cranial nerves; i.e.,
muscles of the face, throat and tongue. Onset of this disorder occurs
usually during middle age.
Symptoms
The first noticeable symptom of Progressive Supranuclear Palsy (PSP) is
usually loss of balance while walking. Patients may have unexplained falls
or a stiff awkwardness in the walk. Other symptoms may resemble Parkinson's
disease. (For more information on Parkinson's Disease, please choose
"Parkinson" as your search term in the Rare Disease Database.) Other common
early symptoms include forgetfulness and personality changes such as loss of
interest in formerly pleasurable activities or increased irritability. Less
common symptoms are vision disturbances, slurred speech and mild shaking in
the hands. New symptoms can develop during the course of PSP, and
previously mild problems tend to become more severe with time.
Blurred vision usually develops three to five years after the walking
problems, and is characterized as an inability to aim the eyes properly or to
move them up or down. This is caused by a weakness or paralysis (palsy) of
the muscles that move the eyeballs. Eventually, reading, driving, or even
maintaining eye contact becomes difficult because the eyes may not aim or
focus properly. Additionally, abnormal eyelid control can develop. Eyes may
close involuntarily (blepharospasm) for seconds or minutes. Some patients
may not be able to open their eyes at times, and others may have trouble
closing their eyes or may blink much less than normal. This can cause the
eyes to become dry and red.
Movements of the mouth, tongue and throat are also affected in PSP.
Speech usually becomes slurred three or four years after symptoms first
begin. Similarly, swallowing some foods can become difficult because of
throat muscle weakness. This tends to occur after the walking, vision, and
speech problems appear. Slight forgetfulness, personality changes and poor
eye contact during conversations can give an incorrect impression of
senility. Although mental confusion in patients with PSP is mostly only an
impression, a mild or moderate degree of mental impairment eventually occurs
which may be misdiagnosed as Alzheimer's Disease (AD). (For more information
on Alzheimer's Disease, please choose "Alzheimer" as your search term in the
Rare Disease Database.)
Causes
The cause of Progressive Supranuclear Palsy is unknown. It is possibly a
long-term degenerative process caused by an unknown slow virus. Slow viruses
tend to stay dormant in humans for extended periods of time, then for reasons
yet unknown, become activated. Some scientists believe that viruses are not
involved , and that exposure to some unknown chemical may cause PSP by slowly
damaging certain areas of the brain. The poor control of vertical gaze and
oral-pharyngeal movement is indicative of damage to corticobulbar regulatory
pathways of the brain.
Affected Population
Progressive Supranuclear Palsy affects approximately 20,000 people in the
United States. It usually begins during middle age and predominantly affects
males.
Related Disorders
Lacunar State is a similar neurologic disorder which is differentiated from
Progressive Supranuclear Palsy by the history of a step-wise progression of
symptoms that seems characteristic of strokes.
Pseudobulbar Palsy is a disorder characterized by an inability to control
movements such as chewing, swallowing and talking. The term "bulbar palsy"
means a paralysis of the part of the brain (the bulb) which controls these
movements. Pseudobulbar Palsy is usually the result of multiple strokes.
The patient often smiles, laughs or cries uncontrollably, but his ability to
move his eyes is normal.
Parkinson's Disease (PD) is a progressive neurologic disorder
characterized by tremor, muscular rigidity, slowness in initiating movements
and difficulty in maintaining balance. (For more information on this
disorder, choose "Parkinson" as your search term in the Rare Disease
Database.)
Therapies: Standard
Treatment of Progressive Supranuclear Palsy is symptomatic and supportive.
Tricyclic antidepressant drugs such as amitriptyline (Elavil) and imipramine
(Tofranil) help relieve some of the symptoms of PSP. Some other drugs that
can improve the muscle rigidity and slow voluntary movements (extrapyramidal
symptoms) temporarily when used in the early stages of the disorder, are the
drugs bromocriptine (Parlodel), carbidopalevodopa (Sinemet), amantadine
hydrochloride (Symmetrel), trihexyphenidyl (Artane) and benztropine
(Cogentin). The dopamine agonist and ergot alkaloid levodopa may improve
symptoms in some patients; others may show temporary improvement with
pergolide (Permax). The use of these drugs should be monitored carefully by
a neurologist experienced in their use.
Walking aids such as a walker weighted in front, and wearing shoes with
built-up heels may help in preventing patients from falling backwards.
Therapies: Investigational
The National Institute of Neurological Disorders and Stroke (NINDS) is
seeking certain individuals affected by Progressive Supranuclear Palsy for
participation in a clinical research study. For complete information, those
interested should have their physicians contact:
Dr. Irene Litvan
NINCDS Experimental Therapeutics Branch
Bldg. 10, Room 5C106
Bethesda, MD 20892
(301) 496-7993
Idazoxan, a British drug developed as an antidepressant, is now being
studied as a treatment for PSP. Dr. John H. Growdon of Harvard Medical
School is investigating its benefits in treating PSP patients. The drug was
not effective as an antidepressant and is no longer being manufactured in
Britain. It has shown promising results in Dr. Growdon's study.
This disease entry is based upon medical information available through
April 1992. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Progressive Supranuclear Palsy, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Society for Progressive Supranuclear Palsy (SPSP)
2904-B Marnat Rd.
Baltimore, MD 21209
(301) 484-8771
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
International Tremor Foundation
360 West Superior Street
Chicago, IL 60610
(312) 664-2344
PSP Research Fund
Dept. of Neurology
UMDNJ-Robert Wood Johnson Medical School
CN19
New Brunswick, NJ 08903
References
PROGRESSIVE SUPRANUCLEAR PALSY: HL Klawans; United Parkinson Foundation,
1981.