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SHARE: Progressive Supranuclear Palsy (PSP)
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            What is progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) is a rare neurological disorder that affects body movements, walking and balance, and eye movements. PSP is caused by damage to nerve cells in areas of the brain that control thinking and body movements. It is one of a family of neurological conditions called atypical parkinsonism and belongs to the category offrontotemporal disorders.

PSP is different fromParkinson's disease, although some of their symptoms are similar. PSP typically begins in a person’s mid- to late-60s, later than when Parkinson’s disease symptoms typically develop. The disease usually worsens rapidly and most people with PSP develop severe disability within three to five years of symptom onset. PSP can lead to serious complications such as pneumonia, choking, or head injuries from falls.

Symptoms of Progressive Supranuclear Palsy

PSP affects a person’s movements, and can lead to loss of balance, difficulty walking or swallowing, slurred speech, problems with eye movements. PSP can also affect a person’s mood, behavior, and thinking. The most frequent first symptom of PSP is a loss of balance while walking which can lead to abrupt and unexplained falls. People with PSP may also have stiffness and slow movement.

As the disease progresses, most people develop eye problems. Eye and vision symptoms may include:

Slow eye movements
	Trouble looking up or down
	Trouble controlling eyelids, involuntary closing of the eyes, decreased blinking, or difficulty opening the eyes
	Tendency to move the head rather than just the eyes to look in different directionsPeople with PSP and their loved ones may notice changes in mood or behavior. These may include:

Depression
	Lack of motivation
	Changes in judgment, insight, and problem solving
	Difficulty finding words
	Forgetfulness
	Loss of interest in activities the person used to enjoy
	Increased irritability
	Sudden laughing, crying, or angry outbursts for no apparent reason
	Personality changes
	Slowed, slurred, or monotone speech
	Difficulty swallowing
	Mask-like facial expressions
	Sleep problemsHow is PSP different from Parkinson's disease?

PSP is often misdiagnosed as Parkinson's disease, especially early in the disorder. The two disorders share many symptoms; however, PSP progresses more rapidly than Parkinson's.

There are several key differences between PSP and Parkinson's:

People with PSP tend to lean backwards and extend their neck. The unexplained falls that accompany PSP usually arise from falling backward. This is termed “axial rigidity.” People with Parkinson's tend to bend forward rather than backward.
	Problems with speech and swallowing are much more common and severe in PSP than in Parkinson's and usually show up earlier in the disease.
	People with PSP develop unique eye movement problems with looking up and down.
	Tremor is rare in PSP but very common in individuals with Parkinson's.
	Individuals with Parkinson’s disease often show great benefit from levodopa therapy, while people with PSP have minimal or no response.
	A signature of PSP is the accumulation in affected brain cells of a protein known as tau, whereas in Parkinson's, a different protein called alpha-synuclein accumulates in diseased brain cells.Who is more likely to get PSP?

The exact cause of PSP is unknown, but research suggests that it involves progressive damage to cells in a few specific areas in the brain, mainly in the brain stem. The death of brain cells in one of these areas, the substantia nigra, accounts in part for the motor symptoms that PSP and Parkinson's have in common. The tau protein deposits that accumulate in nerve cells in PSP appear to spread to different regions of the brain and cause cells to stop working and die. This in turn destroys the neuronal connections that underlie healthy brain function.

PSP is usually sporadic, meaning that it occurs randomly without a known cause. In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau in brain cells. For most people with PSP, no known genetic factor is found.

Several theories suggest that PSP might be caused by:

Random genetic changes that occur in particular cells or genes in a specific combination that injures these cells.
	Exposure to some unknown environmental factor (such as chemicals in food, air, or water), which slowly damages certain vulnerable areas of the brain.
	Cellular damage caused by free radicals, which are reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in ways to clear free radicals from the body, scientists suspect that—under certain circumstances—free radicals can react with and damage other molecules.How is PSP diagnosed and treated?

Diagnosing PSP

Currently there are no tests or brain imaging techniques to definitively diagnose PSP. A physician will review your medical history and perform a physical and neurological exam. It is important to identify problems with gait, eye movements, speech, and swallowing early. Exams can help rule out other similar disorders. Diagnostic imaging may show shrinkage at the top of the brain stem and help doctors examine brain activity in certain areas.

Treating PSP

There is currently no treatment that effectively stops or slows the progression of PSP, and symptoms usually do not respond well to medications.

Parkinson's disease medications rarely help people with PSP. In some individuals, levodopa can treat the slowness, stiffness, and balance problems associated with PSP, but the effect is usually minimal and short-lasting.
	Injections of botulinum toxin into muscles around the eyes can help with PSP-associated eye closing.
	Some antidepressant drugs may offer some benefits beyond treating depression, such as pain relief and decreasing drooling.There are also non-drug treatment options for PSP symptoms.

Weighted walking aids can reduce the risk of falling backward.
	Bifocals or special glasses called prisms can help reduce difficulty looking down.
	Exercise and physical activity (supervised by a healthcare professional) can keep joints limber.
	People who have significant trouble swallowing and are at risk of serious choking may have a surgical procedure to place a tube into the stomach to allow the person to get nutrients without having to chew and swallow.Deep brain stimulation via devices that deliver electrical stimulation to parts of the brain and other surgical procedures commonly used in Parkinson's have not  proven effective in PSP.

What are the latest updates on PSP?

NINDS, a component of the National Institutes of Health (NIH), is the primary funder of research on the brain and nervous system.NIHis the leading funder of biomedical research in the world.

PSP is one of the disorders being studied as part of theNINDS Parkinson's Disease Biomarkers Program. This initiative is aimed at discovering ways to identify individuals at risk for developing Parkinson's and related disorders, and to track the progression of these diseases. NINDS also supports clinical research studies to develop brain imaging that may allow for earlier and more accurate diagnosis of PSP.

Understanding genetics and the tau protein

Researchers hope to identify specific gene changes that cause PSP and are also studying how genetics and the environment interact to make people more susceptible to the disease. Previous studies have linked multiple genes, including the gene for making the tau protein (MAPT), with PSP.

Scientists hope to understand the mechanisms involving tau that lead to PSP and its symptoms.NINDSsupports research to characterize and distinguish the different formations of the tau protein and to better understand the role of accumulated tau protein in PSP. Other studies are exploring ways to improve how to image or visualize tau in the brain.

Understanding the underlying disease mechanisms

Animal or cell models may identify how PSP occurs and progresses, and this knowledge may lead to the discovery of potential therapies. Other studies in animal models and patients focus on learning about the parts of the brain affected by PSP, such as those involved in motor control and thinking, which can also help us better understand the disease mechanisms.

Developing treatments

There have been significant efforts to develop new therapies for PSP to slow down or stop the disease progression. Many of these studies focus on developing anti-tau therapy to stop the accumulation and spread of the tau protein. Other studies have focused on different PSP-related changes in the nervous system, including inflammation and problems with mitochondria (the energy factories in cells). Other studies hope to find ways to improve brain activity using a special type of brain stimulation that does not involve surgery. Some researchers are exploring stem cell-based therapies to help symptoms of PSP. For a current list of clinical trials for PSP, please visitclinicaltrials.gov.

The****Rare Diseases Clinical Research Network, led byNIH'sNational Center for Advancing Translational Sciences(NCATS), is designed to facilitate research collaboration, study enrollment, and data sharing among rare diseases researchers. TheRare Diseases Clinical Research Networkfunded under this project studies neurological disorders, including PSP.

Research on PSP and related disorders can be found usingNIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies. NIH RePORTER also includes links to publications and patents citing support from these projects.

How can I or my loved one improve care for someone with PSP?

file-medical

Learn About Clinical Trials

Clinical trials are studies that allow us to learn more about disorders and improve care. They can help connect patients with new and upcoming treatment options.

If you or someone you know has recently been diagnosed, contact the organizations referenced below to find out more about care, support, and research. It is important to educate family, friends, and caregivers about your or a loved one's diagnosis. In-person and online support groups offered by nonprofit organizations can give you, families, and caregivers additional resources and opportunities to share experiences and learn about strategies for care and support.

Participate in a clinical trial or study

Consider participating in a clinical trial so clinicians and scientists can learn more about PSP. Clinical research uses human volunteers to help researchers learn more about a disorder and find better ways to safely detect, treat, or prevent disease.All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

The FTD Disorders Registry is a contact and research registry for people diagnosed with frontotemporal disorders, including PSP. It is open to family members, caregivers, or friends of people diagnosed with an FTD. Registries help speed up trials by gathering a list of “willing and able” potential participants. People who sign up may be referred to specific studies but are not obligated to participate.

For information about participating in clinical research visitNIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with PSP atClinicaltrials.gov, a searchable database of current and past clinical studies.

Where can I find more information about PSP?

Information may be available from the following resources:

CUREPSPPhone: 347-294-2873 or 800-457-4777National Organization for Rare Disorders (NORD)
Phone: 203-744-0100 or 800-999-6673; 844-259-7178 Spanish

FTD Registry
Phone: 888-840-9980**Information about PSP is also available:**

PubMed

Learn about related topics

Parkinson's Disease
	Frontotemporal Dementia
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