Progressive supranuclear palsy (PSP)

Progressive supranuclear palsy (PSP) is a neurological condition. It happens when nerve cells are lost in parts of the brain, and this can cause problems with balance, movement, vision, speech and swallowing.

Progressive supranuclear palsy (PSP) usually develops slowly, and doctors can sometimes mistake it for other conditions. The symptoms tend to get worse over many years, but there are many ways to try to manage them.

The first symptoms of progressive supranuclear palsy (PSP) can include:

• repeated falls (often backwards) caused by a sudden loss of balance when walking
• muscle stiffness, especially in the neck
• fatigue
• changes in personality, like mood swings and irritability
• changes in behaviour, like recklessness and poor judgement
• dislike of bright lights (photophobia)
• eye problems, like blurred or double vision, problems looking up and down, or involuntary eye closure
• tremors (involuntary shaking of parts of the body)
• slow movement

In progressive supranuclear palsy (PSP), the symptoms you already have, such as balance and mobility problems, are likely to get steadily worse over time.

New symptoms can also appear at this stage. These include:

• slurred, slow or quiet speech
• problems with swallowing (dysphagia)
• involuntary blinking or twitching of the eyelids
• reduced blinking reflex, which causes the eyes to become dry and irritated
• problems sleeping
• memory problems
• slowness of thought
• back, neck and joint pain

As progressive supranuclear palsy (PSP) gets worse, your speech might become slower and more slurred. Mobility problems can increase too, and you might need a wheelchair to get around.

At the advanced stage, you might find it hard to control the muscles of your mouth, tongue and throat. Because the throat muscles are no longer under your control, swallowing problems can become worse.

PSP can also cause constipation and difficulty passing urine (peeing). Some people need to pee often during the night.

Doctors believe progressive supranuclear palsy (PSP) starts with a protein called tau. It builds up in certain areas of the brain and forms into clumps, and these clumps damage the nerve cells. Over time, this build-up can lead to the symptoms of PSP.

The location of the clumps and the amount of tau differ from one person with PSP to another. This also affects the type and severity of the symptoms.

PSP doesn't tend to run in families, and in most cases it is not believed to be hereditary.

There's no single test for PSP (progressive supranuclear palsy). The symptoms vary a great deal from person to person, and that can make the condition hard to diagnose.

A specialist doctor will ask you about your symptoms so they can rule out other similar conditions, like Parkinson's disease. You might also have other tests and scans.

For progressive supranuclear palsy (PSP), your doctor may arrange one or both of these brain scans:

• an MRI scan
• a dopamine SPECT scan, where you're given an injection of radioactive material so that a gamma camera can create a picture of the brain

The scans also help rule out other conditions that can cause similar symptoms.

You may be asked to have neuropsychological testing if your doctor suspects progressive supranuclear palsy (PSP). These tests look at your:

• concentration
• memory
• communication
• ability to process visual information like pictures and words

There's no specific medicine that can cure or reverse progressive supranuclear palsy (PSP). Medicines used to treat Parkinson's disease can help some people with PSP, improving stiffness, movement and balance. The effect is sometimes limited, though, and might only last a few years.

Some antidepressants can help with pain, stiffness and sleep problems. You might also be offered antidepressants to help with depression.

Discuss your symptoms with your doctor, so you can be sure you're prescribed the best treatment.

A physiotherapist can give you exercises to do on a regular basis. These help keep your muscles strong and stop your joints from stiffening, which matters if you have progressive supranuclear palsy (PSP).

The physiotherapist will also look at whether any equipment could help you. A walking frame, for example, may lower your risk of falls.

You can also try breathing exercises. They can reduce the chance of aspiration pneumonia when you eat. Aspiration pneumonia is a chest infection that happens when you breathe particles of food into your lungs.

A speech and language therapist can help with the speech problems and the swallowing difficulty (dysphagia) that come with progressive supranuclear palsy (PSP). They might suggest changing the consistency of your food so it's easier to swallow.

They may also teach you techniques to make your voice clearer. If they think communication aids or devices could help you, they'll suggest those too.

A dietitian can talk with you about eating a healthy, balanced diet, and help you add more foods and liquids that are easy to swallow in your meals.

If there's a risk of malnutrition and dehydration with progressive supranuclear palsy, you might be offered a feeding tube.

An occupational therapist will look around your home for anything that could cause a fall and an injury. They may suggest fitting a few things to make the house safer for you, such as handles around the bath.

If you have trouble controlling your eyelids because of progressive supranuclear palsy (PSP), botulinum toxin (like Botox) can help relax the eyelid muscles. The effect usually lasts up to 3 months.

PSP can also reduce how often you blink, and that leaves the eyes dry. Eyedrops can ease the irritation.

If bright light bothers your eyes (photophobia), you might try dark wraparound glasses. And if you find it hard to look down, there are special glasses with prisms that can help.

You can choose to have palliative care at any stage of progressive supranuclear palsy (PSP), alongside your other treatments.

Palliative care works to relieve pain and other symptoms. It can also give you psychological, spiritual and social support.

You might receive palliative care:

• at home
• in a hospice or care home
• as an outpatient in a hospital
• in a hospital

You may want to think about making plans now for your future care, so that your family and the health professionals looking after you know what you would want. This can be useful with progressive supranuclear palsy (PSP), because you may later become too ill to communicate your wishes.

You don't have to make these plans if you don't want to.

Don't include personal information such as your name, location or any personal health conditions.