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Progressive supranuclear palsy

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Progressive supranuclear palsy



Introduction 

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

The causes of PSP are unknown, but current research suggests that it's 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.

PSP symptoms

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 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 difficulty speaking and swallowing (dysphagia)



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.

Treating PSP

There's currently no cure for PSP but research into the condition is continuing.

Treatment 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. 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 malnutritionor dehydration



Complications of PSP

PSP isn't life-threatening itself, but it can lead to serious complications.

Difficulty swallowing (dysphagia) is one of the common complications of PSP and can cause choking or inhaling food or liquid into the airways (aspiration). Aspiration can develop into pneumonia.

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

 

 


 


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.

Symptoms of progressive supranuclear palsy 

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

The 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 mood swings



changes in behaviour, such as recklessness and poor judgement



a dislike of bright lights (photophobia)



difficulty controlling the eye muscles, 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 be 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 and 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 difficulty passing urine are common, as is the need to pass urine several times during the night.

Because of dysphagia, people with PSP often experience repeated chest infections caused by fluids or small particles of food passing into their lungs. This is known as aspiration pneumonia and it's a leading cause of death in cases of PSP.

Causes of progressive supranuclear palsy 

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 a protein called tau. Tau occurs naturally in the brain, but people with PSP have much higher levels. 

Tau is usually broken down in the brain. However, 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 that both PSP and CBD are related syndromes that are the result of the same underlying cause or causes.

It's not known what leads to the overproduction of 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's important to note that there's no evidence to suggest that PSP can be passed from a parent to their child, and most experts think that it's unlikely that PSP is an inherited condition.

Environmental factors

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



an 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

Diagnosis of progressive supranuclear palsy 

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's something wrong with your brain, it's likely you'll be referred for a brain imaging scan.

Types of scan that you may have include:



magnetic resonance imaging (MRI) scan  - where a strong magnetic field and radio waves are used to produce detailed images of the inside of the brain



positive emission tomography (PET) scan - similar to an MRI scan, a PET scan has the added advantage of being able to assess how different parts of the brain are functioning



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

They can also help 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 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 1 in 3 people with the condition.

Neuropsychological testing

It's also likely you'll be referred to a neurologist (a nerve and brain specialist) 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



concentration



understanding language



the processing of 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, it's important that you have the support of your family and care team, who will be able to help you come to terms with the diagnosis.

A charity called The PSP Association can give you information and practical advice 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'll require full-time care and equipment, such as a wheelchair, to help with mobility..

Driving

PSP could affect your ability to drive.

You have a legal obligation to tell the Drivers & Vehicle Licensing Agency (DVLA) about your condition. They'll then decide whether it's 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.

Treatment of progressive supranuclear palsy 

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).

 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.

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.

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.

Care and support

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 as well as advice for carers themselves.

You can also contact the PSP Association for help and advice. Their email address is: helpline@pspassociation.org.uk

Complications of progressive supranuclear palsy 

Progressive supranuclear palsy (PSP) can have serious complications including choking and pneumonia caused by swallowing problems 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 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 can also be a serious complication of PSP. It's a lung infection that's 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 voice box (larynx) doesn't 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're being treated for PSP and you develop the above symptoms. If this isn't possible, you should contact your local out-of-hours service or call  111.

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

In particularly vulnerable or frail people, there's 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 sometimes be fatal.
Progressive supranuclear palsy