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Progressive Supranuclear Palsy

Description

Progressive supranuclear palsy (PSP) is a rare brain disorder that causes serious and progressive problems with control of gait and balance, along with complex eye movement and thinking problems. One of the classic signs 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 individuals describe this effect as a blurring. Affected individuals often show alterations of mood and behavior, including depression and apathy as well as progressive mild dementia.

The disorder's long name indicates that the disease begins slowly and continues to get worse (progressive), and causes weakness (palsy) by damaging certain parts of the brain above pea-sized structures called nuclei that control eye movements (supranuclear).

PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson's disease. It is sometimes referred to as Steele-Richardson-Olszewski syndrome, reflecting the combined names of the scientists who defined the disorder. Although PSP gets progressively worse, no one dies from PSP itself.

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.

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.

Research

PSP is one of the diseases being studied as part of the NINDS Parkinsons Disease Biomarkers Program, which aims to discover ways to identify individuals at risk for developing Parkinson's and related disorders, and to track disease progression.  Other researchers are studying genes that might increase a person's risk of developing PSP and to identify specific disease-causing mutations, which could point to additional targets for therapy development.  Investigators ae also developing animal models of PSP and other protein tau-related disorders, for research on disease mechanisms and preclinical testing of potential drugs. Information from the National Library of Medicine’s MedlinePlusProgressive Supranuclear Palsy

CurePSP

Address:
1216 Broadway, 2nd Floor
New York City, NY 10001

Website: https://www.psp.org/
Phone: 347-294-2873; 800-457-4777
Fax: 410-785-7009

CurePSP is the leading source of information and support for patients and their families, other caregivers, researchers and healthcare professionals on prime of life neurodegeneration including PSP, CBD and related diseases. A nonprofit voluntary health organization whose mission is to increase awareness of progressive supranuclear palsy, corticobasal degeneration and related brain diseases (including atypical parkinsonisms); fund research toward cure and prevention; educate healthcare professionals; and provide support, information and hope for affected persons and their families.



Information sourced through CNF’s partnership with The National Institute of Neurological Disorders and Stroke (NINDS), US National Institutes of Health.