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Encyclopaedia


Progressive supranuclear palsy

Introduction

Progressive supranuclear palsy (PSP) is a rare and progressive condition in which increasing numbers of brain cells become damaged over time. This is known as neurodegeneration.

The causes of PSP are unknown, although current research suggests that it is caused by a combination of environmental and genetic factors. Most cases of PSP first develop in people who are 60-65 years of age, although the condition has been diagnosed in people as young as 40.

What are the symptoms of PSP?

As PSP is a progressive condition, the initial symptoms will become more severe over time, and new symptoms can develop. The rate at which the symptoms of PSP progress can vary widely from person to person but early symptoms can include:

  • progressive problems with balance and mobility
  • changes in behaviour, such as irritability or apathy (lack of interest)
  • an inability to control eye and eyelid movement, including focusing on specific objects or looking down at something (dystonia)

Later symptoms can include:

  • increasing difficulties with speaking and swallowing (dysphagia)
  • changes in thought, such as becoming increasingly forgetful or having difficulty concentrating

Diagnosing PSP

There's no single test for diagnosing PSP. Instead, the diagnosis is based on the type and pattern of your symptoms. Other conditions that can cause similar symptoms, such as Parkinson's disease, will also be ruled out.

The diagnosis must be made or confirmed by a consultant with expertise in PSP (this will usually be a neurologist).

If you have symptoms that suggest there's something wrong with your brain, it's likely you'll be referred for a brain imaging scan.

Read more about how PSP is diagnosed.

Treating PSP

Although research into PSP continues, there is currently no cure for the condition. Treatment focuses on relieving symptoms while trying to ensure that someone with PSP has the best possible quality of life.

Treatment for PSP is provided by a team of health and social care professionals working together, as a person may be affected in many different ways. Specific symptoms of PSP might be treated with:

  • medication - to improve balance, stiffness and other symptoms
  • botulinum 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.

Complications of PSP

Although PSP itself is not life-threatening, it can lead to serious complications. One of the common complications of PSP is difficulty swallowing (dysphagia), leading to choking or inhaling food or liquid into the airways (aspiration). Aspiration can develop into pneumonia.

Problems with mobility can also make someone with PSP vulnerable to falls, which can lead to head injuries and fractures in severe cases.

Good care and assistance can help people with PSP to have better quality of life and more independence.

Read more about the complications of PSP.

How common is PSP?

Progressive supranuclear palsy (PSP) is a rare condition. The PSP Association estimates that there are around 4,000 people with PSP living in the UK at any one time.

However, it's thought that this figure could be much higher (up to 10,000) because many cases may be misdiagnosed as Parkinson’s disease.

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Symptoms

As the name suggests, progressive supranuclear palsy (PSP) is a progressive condition.

This means that the symptoms often develop gradually, which makes it easy for them to be mistaken for the symptoms of other, more common conditions.

The symptoms of PSP become more severe over time. Individual symptoms can also vary in severity from person to person. Only a few people will experience all of the symptoms listed below.

Initial symptoms

Initial symptoms of PSP may include:

  • sudden loss of balance when walking that usually results in repeated falls, often backwards
  • muscle stiffness, particularly in the neck
  • changes in personality such as irritability, apathy (lack of interest) and sudden mood swings
  • changes in behaviour such as recklessness and poor judgement
  • a dislike of bright lights (photophobia)
  • difficulty controlling the muscles of the eyes, particularly problems with looking up and down (this is the supranuclear palsy that gives the condition its name)
  • blurred or double vision

Intermediate symptoms

Over time, the initial symptoms of PSP will become more severe and new symptoms will develop. The loss of balance can become so severe that walking becomes impossible and a wheelchair will eventually be required. A person with PSP may develop slow, quiet or slurred speech with some swallowing problems.

Controlling the eye muscles will become more difficult, increasing the risk of falls and making everyday tasks, such as reading or eating, more problematic.

Some people with PSP will also experience problems controlling their eyelids, including:

  • reduced blinking reflex, which can cause the eyes to dry out and become irritated
  • involuntary closing of the eyes, which can last from several seconds to hours
  • difficulties opening the eyelids

Advanced stages

As PSP progresses to an advanced stage, people with the condition may 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 word finding, although severe dementia is rare.

The loss of control of the throat muscles leads to difficulties swallowing (dysphagia). At some point, many people with PSP will need to consider using a feeding tube.

Many people with PSP also develop problems with their bowel and bladder functions. Constipation and difficulties in passing urine are common, as is the need to pass urine several times during the night.

Due to dysphagia, many people with PSP will experience repeated chest infections that are caused by fluids or small particles of food inadvertently passing into their lungs. This is known as aspiration pneumonia and it is a leading cause of death in cases of PSP. Read more about the complications of PSP.

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Causes

Progressive supranuclear palsy (PSP) is caused by damage to brain cells in the parts of the brain that help control movement, balance, vision and speech.

Tau protein

Some of the brain cells are damaged by clumps of a protein called tau. Tau occurs naturally in the brain, but people with PSP have much higher levels.

Tau is normally broken down in the brain. In PSP the tau changes slightly, allowing clumps to build up and damage cells.

PSP has similar symptoms to another neurodegenerative condition called corticobasal degeneration (CBD), although the pattern of brain damage is slightly different. It may be the case that both PSP and CBD are in fact two related syndromes that are the result of the same underlying cause (or causes).

It is not known what leads to the overproduction of the tau protein and the resulting death of brain cells. Recent research has indicated that genetic factors can make someone more likely to develop PSP, but cases of PSP are nearly always a one-off in a family. The risk to other members of the family, including children or siblings of someone with PSP, is very low.

It is important to note that there is no evidence to suggest that PSP can be passed down from a parent to their child, and most experts think that it is unlikely that PSP is an inherited condition.

Environmental factors

Further research is ongoing to identify what other factors may trigger PSP. Suggested environmental triggers include:

  • an, as yet, unidentified virus or other type of infection, which may slowly infect the brain over the course of many years
  • an unidentified neurotoxin (a poison that damages brain and nerve cells) that may be present in the environment
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Diagnosis

Diagnosing progressive supranuclear palsy (PSP) can be difficult because its symptoms are similar to other more common movement disorders.

A diagnosis will be based on the type and pattern of your symptoms and ruling out other conditions that can cause similar symptoms.

The diagnosis must be made by a consultant with expertise in PSP (usually a neurologist).

Imaging scans

If you have symptoms of PSP that suggest there is something wrong with your brain it is likely that you will be referred for a brain imaging scan.

Brain imaging scans may include:

These types of imaging studies can be useful in ruling out other possible conditions such as brain tumour or stroke.

They can also detect abnormal changes to the brain that are consistent with a diagnosis of PSP, such as shrinkage in the cortex and basal ganglia.

Ruling out Parkinson’s disease

You may be prescribed a medication called levodopa which can help to determine whether your symptoms are caused by PSP or Parkinson’s disease. People with Parkinson’s disease usually experience a marked improvement in their symptoms after taking levodopa.

However, this is not the case for people with PSP as levodopa usually only has a limited beneficial effect in one in three people with the condition.

Neuropsychological testing

It is also likely that you will be referred to a neurologist (a nerve and brain specialist) and possibly also a psychologist for what is known as 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
  • concentration
  • ability to understand language
  • the ability to process visual information, such as words and pictures

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 is usually unaffected.

Emotional support

Once other possible causes of your symptoms have been ruled out, a confident diagnosis of PSP can usually be made.

Being told that you have PSP can be an emotionally devastating experience, and the news can often be difficult to take in. Therefore, at this time, it is important that you have the support of your family and care team who will be able to help you to come to terms with the diagnosis.

A charity called The PSP Association can give you practical advice and information about living with PSP, as well as providing you with support to help you cope with the emotional impact of the condition.

As your symptoms progress you will require full-time care and equipment, such as a wheelchair, to help with mobility.

Driving with PSP

If you are diagnosed with PSP, the condition could affect your ability to drive. Therefore, you have a legal obligation to inform the Drivers and Vehicle Licensing Agency (DVLA) about your diagnosis. They will decide whether it is safe for you to continue driving.

Failure to inform the DVLA about a condition that could affect your ability to drive is a criminal offence. See the UK Gov website for more information.

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Treatment

There's no cure for progressive supranuclear palsy (PSP) so the aim of treatment is to help control the symptoms.

Multidisciplinary team

As PSP can affect many different areas of your health, treatment is provided by a team of health and social care professionals working together.

This type of team is known as a multidisciplinary team (MDT) because it's made up of experts from many different disciplines. The team will include:

  • a neurologist - a specialist in conditions that affect the brain and nervous system
  • a physiotherapist - a therapist who helps ill, injured or disabled people recover movement and function to their full potential
  • a speech and language therapist
  • an occupational therapist - a therapist who helps people improve the skills they need for daily activities, such as washing or dressing
  • a psychologist
  • a social worker - who will be able to advise you about the support available from social services
  • an ophthalmologist or orthoptist - specialists in treating eye conditions
  • a palliative care specialist - a specialist in treating people with terminal illnesses
  • a specialist neurology nurse - who may act as your point of contact with the rest of the team

Medication

Medication that might be prescribed for PSP includes:

  • levodopa, amantadine and other medications used to treat Parkinson's disease; these improve balance and stiffness in around 1 in 3 people
  • antidepressants to help with depression that's often associated with PSP; antidepressants may only be effective on a short-term basis
  • amitriptylline, which in low doses is sometimes used to treat pain and sleep problems in PSP

People with PSP can be more sensitive to the side effects of other medications, so your specialist may suggest that you stop taking them.

Physiotherapy

A physiotherapist can give you advice about making the most of your remaining mobility using exercise. Regular exercise can help strengthen your muscles, improve your posture and prevent stiffening of your joints.

Your physiotherapist will also be able to advise you about equipment that could be of benefit to you, such as a walking frame or specially designed shoes to reduce the risk of slipping and falling.

A physiotherapist will also be able to teach you breathing exercises that you can use when you eat to reduce your risk of developing aspiration pneumonia (a chest infection caused by small particles of food falling into your lungs).

Read more about physiotherapy.

Speech and language therapy (SLT)

A speech and language therapist 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.

As PSP progresses, you may need some sort of assistive technology to help you communicate. A range of communication aids are available, and your therapist will be able to advise you about the devices that will be most suitable for you.

Your therapist may be able to teach you exercises that can stimulate the nerves that are used to trigger your swallowing reflex, as well as strengthening the muscles that are used during swallowing.

There are also techniques that can be used to make swallowing easier. For example, some people find that moving their chin forward while swallowing helps prevent food entering their airways.

As your dysphagia becomes more severe, you'll need additional treatment to compensate for your swallowing difficulties (see below).

Diet and severe dysphagia

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 receive 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 in severe cases of dysphagia, 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.

There are two types of feeding tubes:

  • nasogastric tube - a temporary tube that's passed down your nose and into your stomach
  • percutaneous endoscopic gastrostomy (PEG) tube - a tube that's surgically implanted directly into your stomach, which passes through a small incision on the surface of your stomach or abdomen

Nasogastric tubes are designed for short-term use and last for 10-28 days before they need to be replaced. PEG tubes are designed for long-term use.

Read more information about how dysphagia is treated.

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, 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 injection usually last for up to three months.

If you're experiencing dryness of your eyes due to reduced blinking, eyedrops 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

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

Advance decisions

Many people with PSP draw up an advance decision. An advance decision is where you make your treatment preferences known in advance in case you're unable to communicate your decisions later on because you're too ill.

Issues covered by an advance decision include:

  • whether you want to be treated at home, in a hospice or in a hospital when you reach the final stages of PSP
  • the type of painkillers you would be willing to take
  • whether you would be willing to use a feeding tube if you were no longer able to swallow food and liquid
  • whether you're willing to donate any of your organs after you die (the brains of people with PSP are very useful for research purposes)
  • if you experience respiratory failure (loss of lung function) due to aspiration pneumonia, whether you would be willing to be resuscitated by artificial means - for example, by having a breathing tube inserted into your neck

You can't request anything illegal in your advance decision, such as assisted suicide.

Your care team will be able to provide you with more information and advice about advance decisions.

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Complications

Progressive supranuclear palsy (PSP) can have serious complications including choking and pneumonia caused by problems with swallowing (dysphagia), or injuries caused by falls.

Falls

Falls are a common complication of PSP due to the associated problems with balance and vision. They can be very serious as people often fall backwards and without warning.

In the most serious cases falls can cause fractures or head injuries, which can be life-threatening.

The risk of a fall can often be reduced through good care and assistance. Treatment includes the use of walking aids, methods to improve balance and eyesight problems, and identifying potential hazards in the home.

Aspiration pneumonia

Aspiration pneumonia is one of the most potentially serious complications of progressive supranuclear palsy (PSP). Aspiration pneumonia is a lung infection that is triggered when fluids or small pieces of food enter your lungs.

People with PSP are particularly vulnerable to aspiration pneumonia because their impaired swallowing reflexes mean that their larynx (voice box) does not close when they swallow, increasing the risk of food and fluid entering their lungs.

The symptoms of aspiration pneumonia include:

  • high temperature (fever) above 38°C (100.4°F)
  • fatigue
  • chest pain
  • shortness of breath
  • blue skin (cyanosis) - due to a lack of oxygen
  • wheezing

You may also have a cough which sometimes produces foul-smelling phlegm and may contain traces of blood and pus.

Contact your care team immediately if you are being treated for PSP and you develop the above symptoms. If this is not possible, you should contact your local out-of-hours service, or call NHS Direct Wales on 0845 46 47.

The symptoms of aspiration pneumonia can range from mild to severe. Severe cases will require hospital admission and treatment with intravenous antibiotics (antibiotics that are given through a vein).

In particularly vulnerable or frail people, there is a risk that the infection could lead to their lungs becoming filled with fluid, preventing them from working properly. This is known as acute respiratory distress syndrome (ARDS).

Unfortunately, as most people with advanced PSP are vulnerable and frail, repeated episodes of aspiration pneumonia can be fatal.

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The information on this page has been adapted by NHS Wales from original content supplied by NHS Choices.
Last Updated: 05/08/2014 14:30:16

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