Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a rare neurological 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.

Most cases of PSP develop in people who are over the age of 60.

What causes PSP?

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 aren't inherited and the risk to other family members, including the children or siblings of someone with PSP, is very low.

PSP symptoms

The symptoms of PSP usually get worse over time.

At first, they can be similar to some other conditions, which makes it difficult to diagnose early on.

Some of the main symptoms of PSP include:

• problems with balance and mobility, including frequent falls
• changes in behaviour, such as irritability or apathy (lack of interest)
• muscle stiffness
• an inability to control eye and eyelid movement, including focusing on specific objects or looking up or down at something
• slow, quiet or slurred speech
• difficulty swallowing (dysphagia)
• slowness of thought and some memory problems

The rate at which the symptoms progress can vary widely from person to person.

Read more about the symptoms of PSP.

Diagnosing 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.

Treatments for PSP

There's currently no cure for PSP, but research is continuing into new treatments that aim to relieve symptoms and slow down the progression of the condition.

Treatment currently focuses on relieving the symptoms while trying to make sure 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 and may include:

• medication to improve stiffness and other symptoms
• physiotherapy to help with movement and balance difficulties
• speech and language therapy to help with speech or swallowing problems
• occupational therapy to help improve the skills needed for daily activities
• Botox (botulinum toxin injections) or special glasses to help with eye problems
• feeding tubes to help manage dysphagia and avoid malnutrition or dehydration

Read more about how PSP is treated.

Outlook

There is currently no treatment that can stop the progression of PSP. But good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life.

There will come a point when a person with PSP is at risk of developing serious complications. So, it's a good idea to talk to your care team about what you'd like to happen when the condition reaches this stage.

Sharing your information

If you have PSP, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Disease 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.

 Symptoms Progressive supranuclear palsy 

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.

Most people with PSP will not have all of the symptoms.

Early symptoms

The initial symptoms of PSP can include:

• sudden loss of balance when walking that usually results in repeated falls, often backwards
• extreme tiredness
• slow, quiet or slurred speech
• changes in personality and behaviour
• difficulties with tasks involving planning and organisation
• a dislike of bright lights (photophobia)
• difficulty controlling the eye muscles (particularly problems with looking up and down)
• blurred or double vision

Some people have early symptoms that are very similar to those of Parkinson's disease, such as muscle stiffness and slow movement.

Mid-stage symptoms

The symptoms of PSP usually get worse over time, but how the condition progresses can vary from person to person.

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:

• problems swallowing (dysphagia)
• eyelid problems, such as twitching or drooping eyelids
• disturbed sleep
• slowness of thought and some memory problems
• muscle stiffness, particularly in the neck
• neck, back and joint pain
• headaches

Advanced stages

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 increasing problems with thinking, concentration and memory.

The loss of control of the throat muscles can lead to severe swallowing problems.

This may mean a feeding tube is required at some point. This is to prevent choking and chest infections caused by fluid or small food particles passing into the lungs.

Many people with PSP also develop problems with their bowel and bladder function. 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).

 Diagnosis Progressive supranuclear palsy 

It can be difficult to diagnose progressive supranuclear palsy (PSP), as there's no single test for it and it can have similar symptoms to a number of other conditions.

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.

A diagnosis of PSP will be based on the pattern of your symptoms and by ruling out conditions that can cause similar symptoms, such as Parkinson's disease or a stroke.

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 (a specialist in conditions affecting the brain and nerves).

Brain scans

If you have symptoms of PSP, you may be referred for a brain scan.

Types of scan that you may have include:

• an MRI scan (where a strong magnetic field and radio waves are used to produce detailed images of the inside of the brain)
• a PET scan (this detects the radiation given off by a substance injected beforehand)

These scans can be useful in ruling out other possible conditions, such as brain tumours or strokes.

MRI scans can also detect changes to the brain that are consistent with a diagnosis of PSP, such as shrinkage of certain areas.

Ruling out Parkinson's disease

You may be prescribed a short course of a medicine 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 medicine only has a limited beneficial effect for some people with PSP.

Neuropsychological testing

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:

• memory
• mental tasks such as planning and problem solving
• understanding language
• the processing of information, such as words and pictures

Coping with a diagnosis

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.

You can get in touch with the PSP Association by:

• calling their helpline on 0300 011 0122

 Treatment Progressive supranuclear palsy 

There's currently no cure for progressive supranuclear palsy (PSP), 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:

• a neurologist (a specialist in conditions that affect the brain and nerves)
• a physiotherapist (who can help with movement and balance difficulties)
• a speech and language therapist (who can help with speech or swallowing problems)
• an occupational therapist (who can help you improve the skills you need for daily activities, such as washing or dressing)
• a social worker (who can advise you about the support available from social services)
• an ophthalmologist or orthoptist (specialists in treating eye conditions)
• a specialist neurology nurse (who may act as your point of contact with the rest of the team)

Medication

There are currently no medications that treat PSP specifically, but some people in the early stages of the condition may benefit from taking medications used to treat Parkinson's disease.

These medications may improve muscle stiffness and slowness of movement for some people with PSP, although the effect is often limited and only lasts for up to a few years.

Botox injections may also provide some relief from muscle spasms.

Antidepressants can help with the depression that's often associated with PSP.

Some may also help pain, bladder and bowel problems and disturbed sleep.

It's important to tell your doctor about the symptoms you're experiencing so they can consider which treatments are best for you.

Physiotherapy

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.

Speech and language therapy

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 you may need as the condition progresses.

Your therapist can also advise you about different swallowing techniques and may suggest altering the consistency of your food to make swallowing easier

Diet and severe swallowing problems

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'll advise you on how to have a healthy, balanced diet when making any suggested changes to the consistency or texture of what you eat.

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 problems with swallowing 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 tummy during an operation.

Read more about treating swallowing problems (dysphagia).

Occupational therapy

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.

Treating eye problems

If you're having problems controlling your eyelids, injections of botulinum toxin (such as Botox) 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 3 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 dark wraparound glasses can help those who are sensitive to bright light (photophobia).

Palliative care

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:

• in a hospice or care home
• at home
• on a day patient basis in a hospice
• in a hospital

Advanced care planning

Many people with PSP consider making plans for the future that outline their wishes (both medical and other decisions), and make these 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:

• whether you want to be treated at home or in a hospice or hospital when you reach the final stages of PSP
• the type of painkillers you'd be willing to take
• whether you'd be willing to use a feeding tube if you were no longer able to swallow food and liquid
• whether you'd be willing to be resuscitated
• whether you're willing to donate a sample of your brain tissue after you die for research purposes

If you want to decide about these issues, they can be written down in a number of ways:

• advance decision to refuse treatment
• advance statement about your wishes
• emergency healthcare plan
• lasting power of attorney

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.

Research into treating PSP

There have been several trials of different medications that aim to help symptoms of PSP or slow its progression.

Further research into treatments, scans and causes of the condition is ongoing.

If you wish to get involved in research, you can ask your care team about any ongoing or planned trials you may be suitable for.