What is psp

Although there is no known cure, medicines and devices can help you live with the symptoms. Work with your healthcare provider to find ways to make walking safer and improve your vision.

PSP is not fatal but it is important that you do not breathe in food particles aspirate while you are eating because it could be life threatening. Although it's easy to try to brush off initial symptoms as being a little clumsy or maybe having an ear infection, it's a good idea to see a doctor at the earliest sign of symptoms, especially if you have problems with your eyes or vision.

Always seek advice from your healthcare provider if you or your caregiver notice sudden or significant changes in your symptoms. Health Home Conditions and Diseases. Progressive supranuclear palsy PSP is a complex condition that affects the brain.

Supranuclear refers to the region of the brain affected by the disorder — the section above 2 small areas called nuclei. Palsy is a disorder that results in weakness of certain muscles. What causes progressive supranuclear palsy?

What are the symptoms of progressive supranuclear palsy? These are also early signs of PSP: Becoming more forgetful and cranky Having unusual emotional outbursts, like crying or laughing at unexpected times Becoming angry for no real reason Tremors in the hands Trouble controlling eye movements Blurred vision Slurred speech Trouble swallowing Dementia Depression Trouble directing your eyes where you want them to go Inability to control the eyelids, such as unwanted blinking or being unable to open your eyes Trouble holding someone's gaze How is progressive supranuclear palsy diagnosed?

How is progressive supranuclear palsy treated? If you have PSP, you may be able to use certain aids to make life easier. For example: Special glasses with prisms may improve your vision. A weighted tool that helps you walk more easily can prevent you from falling backward. Physical therapy and exercise may slightly improve flexibility in some people. What are the complications of progressive supranuclear palsy? Living with supranuclear palsy Although there is no known cure, medicines and devices can help you live with the symptoms.

When should I call my healthcare provider? Key points about supranuclear palsy Experts are still working to understand more about progressive supranuclear palsy and find more effective ways to treat it. Although the disease itself isn't life threatening, its complications can be. The exact cause of PSP is unknown, but research suggests that it involves a gradual deterioration of brain cells in a few specific areas in the brain, mainly in 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 hallmark of PSP is the accumulation of abnormal deposits of the protein tau in nerve cells in the brain.

These deposits cause the cells to malfunction and die, which stops the flow of information to other nerve cells. PSP is usually sporadic, meaning that it occurs infrequently and 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 to the nerve cell. Genetic factors have not been implicated in most individuals. Currently there are no tests or brain imaging techniques to definitively diagnose PSP.

Identifying early gait problems, problems moving the eyes, speech and swallowing abnormalities, as well as ruling out other similar disorders is important. Diagnostic imaging may show shrinkage at the top of the brain stem and look at brain activity in known areas of degeneration.

There is currently no effective treatment for PSP and symptoms usually do not respond to medications. A gastrostomy a surgical procedure that involves the placement of a tube through the skin of the abdomen into the stomach for feeding purposes may be necessary when there are swallowing disturbances or the definite risk of severe choking.

Deep brain stimulation—which uses surgically implanted electrodes and a pacemaker-like medical device to deliver electrical stimulation to specific areas in the brain to block signals that cause the motor symptoms of several neurological disorders—and other surgical procedures commonly used in individuals with Parkinson's disease have not been proven effective in PSP.

The disease gets progressively worse, with people becoming severely disabled within three to five years of onset. Affected individuals are predisposed to serious complications such as pneumonia, choking, head injury, and fractures. The most common cause of death is pneumonia.

With good attention to medical and nutritional needs, it is possible for individuals with PSP to live a decade or more after the first symptoms of the disease appear. Therefore, people with PSP should carefully consider the meager evidence to date for the benefit of CoQ10 before taking that long-term financial plunge. Their neurologists should do a careful exam upon starting the drug and repeat it two months later to determine whether the treatment is working, and if not, discontinue it.

Botox or Myobloc , two types of botulinum toxin, are a different sort of drug that can be useful for people whose PSP is complicated by blepharospasm. A very dilute solution of the toxin, which is produced by certain bacteria that can contaminate food, can be carefully injected by a neurologist into the eyelid muscles as a temporary remedy for abnormal involuntary eyelid closure. Botox can also be used for involuntary turning or bending of the head that occurs in PSP, but injecting it into the neck muscles can sometimes cause slight weakness of the nearby swallowing muscles.

In PSP, where swallowing is already impaired in many patients, caution should be used when considering use of Botox in neck muscles.

In the past 20 years, many drugs have been tested in patients with PSP. Some of these are intended to actually slow the long-term brain cell loss; i. Unfortunately, none has helped. The last drug did show a glimmer of promise; MRI scans showed less loss of brain mass in the patients on tideglusib than in those on placebo. This may justify further study of the drug, but it is doubtful that any drug company will want to pursue it after it failed to slow progression of the actual symptoms of the disease.

Two other approaches to slowing the progression of PSP have just entered clinical trials. One gives antibodies designed to attack the abnormal tau protein as if it were an invading virus or bacterium. Two current, small, preliminary trials use existing drugs that are marketed for other diseases. One uses Salsalate, a nonsteroidal anti-inflammatory drug, which may prevent the tau protein from aggregating via a mechanism unrelated to its anti-inflammatory effect.

Two drugs that reduce the tendency of tau protein to aggregate by altering the ability of phosphate molecules to attach to it will enter trials in the next year or two. The two drug companies involved are Asceneuron and Merck. For extreme cases of poor swallowing where choking is a definite risk, the placement of a tube through the abdomen into the stomach called gastrostomy or percutaneous endoscopic gastrostomy or PEG may be advised.

PEG feeding may allow patients to regain lost weight, avoid hunger and receive the nourishment they need to fight off other potential complications of PSP. A patient receiving the necessary nutrients and fluids is much happier and stronger overall and will probably find general movement, speech and thinking easier. PEG placement may be considered when any of the following occur: a first episode of aspiration pneumonia; small amounts of aspiration with each swallow; significant weight loss from insufficient feeding; or when the prolonged time required for a meal disrupts the operation of the household.

The PEG tube can be inserted with the patient awake but sedated, often as an outpatient procedure. The tube is clamped shut and hidden under the clothes when not in use. The skin site where the tube enters requires only a little care that can easily be provided by a family member or even by the patient in some cases.

If the need for tube feeding abates as through a new medication, for example , normal oral feeding can be resumed and the tube can be kept as a backup or removed.

Some patients in the advanced stages of PSP may feel that their quality of life is so poor that prolonging that life by having a PEG installed is not what they want. Not so far, unfortunately. One is based on the theory that the basal ganglia the group of nuclei that control movement to the rest of the brain is overactive. The most common operation to dampen down this overactivity at present is subthalamic nucleus stimulation.

The previous approach, pallidotomy, is rarely performed now. In PSP, however, the output area of the basal ganglia is damaged, so its activity is already dampened. The operation would only make things worse. There are, however, trials now in progress in people with PSP to test stimulation of the area of the brain that serves balance, the pedunculo-pontine nucleus PPN. The PPN is in the brainstem, which is an area tightly packed with critical circuitry.

Many of those other nuclei receive their input from the substantia nigra, so replacing only the first link in the chain would not help much and, at this point, it would cause too much trauma to the brain to replace cells in all of the nuclei involved in PSP.

An exciting experiment in progress in Milan, Italy, but one that has only a slim chance of success, is the injection of stem cells directly into the arteries feeding the brain. This project is still in the very early phases designed to test its safety.

If it succeeds, then a large project will test for benefit. The intent is not to replace lost cells, as the injections land in random spots, but to stimulate the brain to produce its own growth factors to repair the damage of PSP.

Formal physical therapy is worth a trial in PSP, especially with the goal of teaching the patient to use gait-assistive devices such as a walker. Certain exercises done in the home by oneself on a regular schedule can keep the joints limber. Exercise also has a clear psychological benefit that improves a sense of well-being for anyone with a chronic illness.

The special balance problems in PSP dictate caution in performing any exercises while standing. Many useful exercises can be performed seated in a chair or lying on a mat. Using a stationary bicycle is usually feasible as long as there is help in mounting and dismounting safely.

The best strategy is to have an evaluation and treatment plan from a physical therapist or physiatrist a physician specializing in rehabilitation of chronic conditions. For example, walking, writing and eating may be poor one hour and better the next. Walking aids are often important for patients with PSP.

Because of the tendency to fall backwards, if a walker is required it should be weighted in front with sandbags over the lower rung. A better but more expensive solution is a large, heavy walker resembling a small shopping cart with three or four fat, soft rubber wheels and a hand brake.

The tendency to fall backwards can also be countered by the use of built-up heels. Leg braces are not helpful because the problem in PSP is coordination and balance rather than actual muscle weakness.

Shoes with smooth soles are often better than rubber-soled athletic shoes. People with such symptoms can fall if they move their body forward before the foot moves. In these cases, smooth soles could make it easier to slide the first foot forward. Handrails installed in the home, especially in the bathroom, may also be helpful.

To remedy the difficulty of looking down, bifocals or special prism glasses are sometimes prescribed for people with PSP. CurePSP offers a variety of resources to patients and their families, other caregivers, physicians, and allied health professionals. If you do not find the information you need here, please call our office at or e-mail info curepsp. CurePSP encourages and organizes activities that foster face-to-face communication, exchange of ideas and information, and interaction for comfort and mutual benefit to group members.

CurePSP welcomes donations to its research grants program. CurePSP favors projects with the potential to produce preliminary findings that would support an application to a government agency for a much larger grant in the future.

It does not restrict its grants to any country or continent. To apply for a grant, or learn more about our research please visit this page. Another way to help research and yourself is to participate in clinical trials.

Please visit our clinical trials page for more information. Donations to the Brain Bank can make a significant impact in research. Brains donated there are stored and used only for research in PSP by legitimate researchers who request it. Donating to a brain bank does not interfere with funeral arrangements. Expenses for brain removal will occur, which may be reimbursed in part by CurePSP. The family will receive, at no charge, a full diagnostic autopsy report from the Mayo Clinic pathologist, Dennis W.

There are several other brain banks throughout the country, generally located at major university hospitals. These resources are for patients, families, and friends seeking to educate themselves, and others, about these diseases — the causes, symptoms, trajectory, treatments, and management.

This information is sourced from credible peer-reviewed scientific sources only, and is updated as breakthroughs in our understanding of these diseases occur. This blog is for clinicians, scientists, patients and families fighting progressive supranuclear palsy.

I did a couple of years of internal medicine residency at Hahnemann Hospital in Philadelphia before transferring into a neurology residency at NYU-Bellevue Hospital, which I completed in The information package includes various informational brochures, instructional DVDs, and medical alert cards. As the production of these materials has been paid for by donors, we kindly ask you to help the next person who needs them by making a contribution here. To submit your request, click the link below and provide the information requested in the form.

What is PSP? What is progressive supranuclear palsy? Why has no one heard of PSP? What are the common types of PSP and their early symptoms?

What are the most common early symptoms of PSP? Are there other, rare types of PSP? What happens next?