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Progressive supranuclear palsy (PSP) is a rare and progressive condition that can cause problems with balance, movement, vision, speech and swallowing.
It's caused by increasing numbers of brain cells becoming damaged over time.
The PSP Association estimates there are around 4,000 people with PSP living in the UK. However, it's thought that the true figure could be much higher, because many cases may be misdiagnosed.
Most cases of PSP develop in people who are over 60 years of age.
PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau.
Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with PSP, it isn't broken down properly and forms harmful clumps in brain cells. The amount of abnormal tau in the brain can vary among people with PSP, as can the location of these clumps. This means the condition can have a wide range of symptoms.
The condition has been linked to changes in certain genes, but these genetic faults are not inherited and the risk to other family members, including children or siblings of someone with PSP, is very low.
The symptoms of PSP usually get gradually worse over time. At first, the symptoms can be similar to some other conditions, which makes it difficult to diagnose early on.
Some of the main symptoms of PSP include:
The rate at which the symptoms progress can vary widely from person to person.
Read more about the symptoms of PSP.
There's no single test for PSP. Instead, the diagnosis is based on the pattern of your symptoms. Your doctor will try to rule out other conditions that can cause similar symptoms, such as Parkinson's disease.
The large number of possible symptoms of PSP also makes it difficult to diagnose correctly and can mean it takes a while to get a definitive diagnosis.
You may need to have a brain scan to look for other possible causes of your symptoms, as well as tests of your memory, concentration and ability to understand language.
The diagnosis must be made or confirmed by a consultant with expertise in PSP. This will usually be a neurologist (a specialist in conditions affecting the brain and nerves).
Read more about how PSP is diagnosed.
There's currently no cure for PSP, but research into new treatments that aim to relieve symptoms and prevent the condition getting worse is continuing.
Treatment currently focuses on relieving the symptoms, while trying to ensure that someone with PSP has the best possible quality of life.
As someone with PSP can be affected in many different ways, treatment and care is provided by a team of health and social care professionals working together. Treatment will be tailored to meet the needs of each individual:
Read more about how PSP is treated.
Unfortunately, there's currently nothing that can be done to stop PSP gradually worsening, although research into new treatments gives hope that this may be possible in the future.
Good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications. It’s a good idea to talk to your doctor about what you’d like to happen when the condition reaches this stage.
Difficulty swallowing can cause choking, or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life-threatening; however, help from a speech and language therapist at an early stage can lower this risk for as long as possible.
As a result of these complications, the average life expectancy for someone with PSP is around six or seven years from when their symptoms start. However, it can be much longer, as the timespan varies from person to person.
If you have PSP, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).
This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.
People with progressive supranuclear palsy (PSP) develop a range of difficulties with balance, movement, vision, speech and swallowing.
The condition tends to develop gradually, which means it can be mistaken for another, more common, condition at first.
The symptoms typically become more severe over several years, although the speed at which they worsen varies.
Some of the main symptoms of PSP are outlined below. Most people with the condition won't experience all of these.
The initial symptoms of PSP can include:
Some people have early symptoms that are very similar to those of Parkinson's disease, such as tremors (involuntary shaking of particular parts of the body) and slow movement.
Over time, the initial symptoms of PSP will become more severe.
Worsening balance and mobility problems may mean that walking becomes impossible and a wheelchair is needed. Controlling the eye muscles will become more difficult, increasing the risk of falls and making everyday tasks, such as reading and eating, more problematic.
New symptoms can also develop at this stage, such as:
As PSP progresses to an advanced stage, people with the condition normally begin to experience increasing difficulties controlling the muscles of their mouth, throat and tongue.
Speech may become increasingly slow and slurred, making it harder to understand. There may also be some problems with thinking, concentration and memory (dementia), although these are generally mild and the person will normally retain an awareness of themselves.
The loss of control of the throat muscles can lead to severe swallowing problems, which may mean a feeding tube is required at some point to prevent choking or chest infections caused by fluid or small food particles passing into the lungs.
Many people with PSP also develop problems with their bowels and bladder functions. Constipation and difficulty passing urine are common, as is the need to pass urine several times during the night. Some people may lose control over their bladder or bowel movements (incontinence).
It can be difficult to diagnose progressive supranuclear palsy (PSP), as there’s no single test for it, and the condition can have similar symptoms to a number of others.
There are also many possible symptoms of PSP and several different sub-types that vary slightly, making it hard to make a definitive diagnosis in the early stages of the condition.
Your doctor will need to carry out assessments of your symptoms, plus other tests and scans.
The diagnosis must be made or confirmed by a consultant with expertise in PSP. This will usually be a neurologist (specialist in conditions affecting the brain and nerves).
If you have symptoms of PSP that suggest there's something wrong with your brain, it's likely you'll be referred for a brain scan.
Types of scan that you may have include:
These scans can be useful in ruling out other possible conditions, such as brain tumours or strokes.
MRI scans can also detect abnormal changes to the brain that are consistent with a diagnosis of PSP, such as shrinkage of certain areas.
Scans that show the build-up of the tau protein in the brain that's associated with PSP are currently under development.
You may be prescribed a short course of a medication called levodopa to determine whether your symptoms are caused by PSP or Parkinson's disease.
People with Parkinson's disease usually experience a significant improvement in their symptoms after taking levodopa, whereas the medication only has a limited beneficial effect for some people with PSP.
It's also likely you'll be referred to a neurologist and possibly also a psychologist for neuropsychological testing.
This involves having a series of tests that are designed to evaluate the full extent of your symptoms and their impact on your mental abilities.
The tests will look at abilities such as:
Most people with PSP have a distinct pattern in terms of their mental abilities, including poor concentration, a low attention span and problems with spoken language and processing visual information. Their memory of previously learned facts isn't usually significantly affected.
Being told that you have PSP can be devastating and difficult to take in.
You may feel numb, overwhelmed, angry, distressed, scared or in denial. Some people are relieved that a cause for their symptoms has finally been found. There's no right or wrong way to feel – everybody is different and copes in their own way.
Support from your family and care team can help you come to terms with the diagnosis.
The PSP Association can give you information and practical advice about living with PSP, as well as providing support to help you cope with the emotional impact of the condition.
You can get in touch with the PSP Association by calling their helpline on 0300 0110 122, or by emailing: email@example.com.
There's currently no cure for progressive supranuclear palsy (PSP) and no treatment to slow it down, but there are lots of things that can be done to help manage the symptoms.
As PSP can affect many different areas of your health, you'll be cared for by a team of health and social care professionals working together. This is known as a multidisciplinary team (MDT).
Members of your MDT may include:
Some of the main treatments that may be recommended are outlined below.
There are currently no medications that treat PSP specifically, but some people in the early stages of the condition may benefit from taking levodopa, amantadine or other medications used to treat Parkinson's disease.
These medications can improve balance and stiffness for some people with PSP, although the effect is often limited and only lasts for up to a few years.
It's important to tell your doctor about the symptoms you’re experiencing, so they can consider which of these treatments is best for you.
A physiotherapist can give you advice about making the most of your remaining mobility using exercise, while making sure you don't overexert yourself. Regular exercise may help strengthen your muscles, improve your posture and prevent stiffening of your joints.
Your physiotherapist can advise about equipment that could benefit you, such as a walking frame or specially designed shoes to reduce the risk of slipping and falling.
They can teach you breathing exercises to use when you eat, to reduce your risk of developing aspiration pneumonia (a chest infection caused by food particles falling into your lungs).
Read more about physiotherapy.
A speech and language therapist (SLT) can help you improve your speech and swallowing problems (dysphagia).
They can teach you a number of techniques to help make your voice as clear as possible and can advise you about suitable communication aids or devices that you may need as PSP progresses.
Your therapist can also advise you about different swallowing techniques and, working together with a dietitian (see below), they may suggest altering the consistency of your food to make swallowing easier.
As your swallowing problems become more severe, you'll need additional treatment to compensate for your swallowing difficulties.
You may be referred to a dietitian, who will advise you about making changes to your diet, such as including food and liquids that are easier to swallow, while ensuring that you have a healthy, balanced diet.
For example, mashed potatoes are a good source of carbohydrates, while scrambled eggs and cheese are high in protein and calcium.
Feeding tubes may be recommended for severe swallowing problems, where the risk of malnutrition and dehydration is increased. You should discuss the pros and cons of feeding tubes with your family and care team, preferably when your symptoms of dysphagia are at an early stage.
The main type of feeding tube used is called a percutaneous endoscopic gastrostomy (PEG) tube. This tube is placed into your stomach through your abdomen (tummy) during an operation.
Read more about treating dysphagia.
An occupational therapist (OT) can advise you about how you can increase your safety, and prevent trips and falls during your day-to-day activities.
For example, many people with PSP benefit from having bars placed along the sides of their bath to make it easier for them to get in and out.
The OT will also be able to spot potential hazards in your home that could lead to a fall, such as poor lighting, badly secured rugs and crowded walkways and corridors.
Read more about occupational therapy.
If you're having problems controlling your eyelids, injections of botulinum toxin can be used to help relax the muscles of your eyelids. It works by blocking the signals from the brain to the affected muscles. The effects of the injections usually last for up to three months.
If you're experiencing dry eyes because of reduced blinking, eyedrops and artificial tears can be used to lubricate them and reduce irritation.
Glasses with specially designed lenses can help some people with PSP who have difficulty looking down. Wearing wraparound, dark glasses can help those who are sensitive to bright light (photophobia).
Palliative care can be offered at any stage of PSP, alongside other treatments. It aims to relieve pain and other distressing symptoms while providing psychological, social and spiritual support.
Palliative care can be received:
Read more about accessing palliative care.
Many people with PSP consider making plans for the future that outline their wishes (both medical and other decisions) and make them known to both their family and the health professionals involved in their care.
This can be useful in case you’re unable to communicate your decisions later on because you’re too ill, although it’s voluntary and you don’t have to do it if you don’t want to.
Issues that you may want to cover might include:
If you decide to discuss these issues, they can be written down in a number of ways:
Your care team can provide you with more information and advice about these decisions and how best to record them.
Read more about end of life care.
If someone you know develops PSP, you may need information and advice about caring for them.
The Care and support section has a wide range of useful information about all aspects of caring for others, and advice for carers themselves.
The Parkinson’s nurse within your local hospital may be able to provide you with useful information and support.