Introduction

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, becausemany cases may be misdiagnosed.

Most cases of PSP develop in people who are over60 years of age.

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

PSP symptoms

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:

• 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 thesymptoms progress can vary widely from person to person.

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).

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 helpmanage dysphagia and avoid malnutrition or dehydration

Its a good idea to talk to your doctor about what youd 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.

Information about you

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.

Find out more about the register .