Progressive supranuclear palsy (PSP)
Progressive supranuclear palsy (PSP) is a rare progressive condition that causes 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 over 60 years of age. There's currently no cure for PSP.
Cause of 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 are not inherited and the risk to other family members, including children or siblings of someone with PSP, is low.
Symptoms of PSP
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:
- 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.
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.
Diagnosing PSP
There's no single test for PSP. 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 many 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).
Treatments for PSP
There's no cure for PSP, but research into new treatments that aim to relieve symptoms and prevent the condition getting worse is continuing.
Treatment focuses on relieving the symptoms, while trying to make sure 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:
- medication – to improve balance, 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
- botulinum toxin injections or special glasses – to help with eye problems
- feeding tubes – to help manage dysphagia and avoid malnutrition or dehydration
Outlook
There's nothing that can be done to stop PSP gradually worsening.
Good care and support 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.
Help from a speech and language therapist at an early stage can lower this risk for as long as possible.
The average life expectancy for someone with PSP is around six or seven years from when their symptoms start. This time span varies from person to person.
More useful links
The information on this page has been adapted from original content from the NHS website.
For further information see terms and conditions.