Progressive supranuclear palsy (PSP)

What causes PSP?

Experts believe a protein called tau builds up in certain parts of the brain and forms clumps, damaging nerve cells. Over time, this buildup can lead to the symptoms of PSP.

Where these clumps form, and how much tau is involved, differs from person to person. This also affects the type and severity of symptoms.

PSP doesn't usually run in families and isn't thought to be hereditary in most cases.

There's no single test for PSP. Because the symptoms vary so much from person to person, doctors can find it hard to pin down.

A specialist will start by asking about your symptoms, mainly to rule out other conditions that look similar, such as Parkinson's disease. You may also be asked to have further tests and scans.

Brain scans

You might have:

• an MRI scan
• a dopamine SPECT scan, where a small amount of radioactive material is injected so that a gamma camera can take pictures of the brain

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

Neuropsychological testing

You might also have neuropsychological testing, which looks at how well you can:

• concentrate
• remember things
• communicate
• process visual information, such as pictures and words

There is no cure for PSP, but there is a great deal that can be done to manage the symptoms and keep you comfortable. You will usually have a multi disciplinary team, or MDT, a group of different specialists who work together to look after your care. It can also help to contact the PSP Association for information and support on living with PSP. They have a dedicated PSP Care Navigator who can guide and support you.

Medication

No medicine can cure or reverse PSP. Medicines used for Parkinson's disease help some people with PSP, easing stiffness, movement and balance, though the benefit is often limited and may only last a few years. Certain antidepressants can ease pain, stiffness and sleep problems, and you may also be offered antidepressants if you are dealing with depression. Talk to your doctor about your symptoms so you are given the treatment that suits you best.

Physiotherapy

A physiotherapist can guide you through regular exercises that strengthen your muscles and keep your joints from stiffening. They will also look at whether any equipment would help you, such as a walking frame, which can lower your risk of falls. Breathing exercises can also be useful, since they lower the chance of aspiration pneumonia, a chest infection that happens when small particles of food are breathed into the lungs while eating.

Speech and language therapy

A speech and language therapist can help with difficulties in speaking and swallowing (dysphagia). They may suggest changing the texture of your food so it is easier to swallow, and can teach you techniques to make your voice clearer. If it would help, they may also recommend communication aids or devices.

Nutritional support

A dietitian can advise you on eating a healthy, balanced diet, and can suggest foods and drinks that are easier to swallow. If there is a risk of malnutrition or dehydration, a feeding tube may be offered.

Occupational therapy

An occupational therapist will check your home for anything that could cause a fall or injury. They may recommend fittings such as handles around the bath to make your home safer. Read more about occupational therapy.

Eye problems

If you have trouble controlling your eyelids, botulinum toxin (such as Botox) can help relax the eyelid muscles, and the effect usually lasts up to 3 months. If reduced blinking is causing dry eyes, eyedrops can ease the irritation. If you are sensitive to light (photophobia), dark wraparound glasses may help, and if you have trouble looking downward, glasses fitted with prisms are also available.

Palliative care

Palliative care can be added alongside your other treatments at any stage of PSP. It focuses on relieving pain and other symptoms, and can also offer psychological, spiritual and social support. You might receive this care at home, in a hospice or care home, as an outpatient at a hospital, or as an inpatient in a hospital. Read more about palliative care.

Advance care planning

You may want to think about planning ahead, so that your family and health professionals know how to care for you later on, particularly if you become too unwell to communicate your wishes. This is entirely your choice, and you are not obliged to make such plans if you would rather not. If you do, avoid including personal details such as your name, location or other health conditions.