$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.