Progressive Supranuclear Palsy (Steele-Richardson-Olszewski Syndrome)

What is Progressive Supranuclear Palsy?

Progressive supranuclear palsy (known as PSP) is a rare disorder that occurs as a result of damage to the brain cells. It causes problems with balance, coordination and speech and also affects vision and the ability to swallow properly. PSP is a progressive condition, which means that it gets worse over the course of time.

In most cases, PSP affects people aged over 60 years of age. It is thought to affect around 4,000 people in the UK, but the number may actually be higher as some cases are misdiagnosed.

What causes PSP?

PSP results from damage to the brain cells, which occurs when a form of protein called tau collects in the brain. It is normal to find tau in the brain, but it is normally broken down before it can reach excessive levels. In people with PSP, the protein is not broken down and this leads to a build-up, which results in collections in parts of the brain.

Although there is a belief that genetics may be involved, there is no evidence to suggest that faulty genes are inherited or passed down through generations and if you have a close relative with PSP, this doesn’t increase your risk of developing the condition.

What are the symptoms and signs?

PSP can cause a range of symptoms and it affects people in different ways. The nature and severity of symptoms may be heavily dependent on the location of the tau collections and the amount of tau in the brain. Examples of symptoms include:

• lack of balance and an increased risk of falling
• stiffness in the muscles
• involuntary eye movements
• slurred speech
• a loss of interest and enthusiasm
• dysphagia (problems with swallowing)
• difficulties related to memories
• sensitivity to light
• blurred vision
• double vision
• disturbed sleep patterns

As PSP is progressive, symptoms get worse with time. However, the rate at which the condition accelerates varies from one individual to another. When symptoms reach an advanced stage, it is common for people to need round the clock care. Swallowing is a particular worry, as there is a risk of choking and a feeding tube may be recommended. As balance and coordination deteriorate, there is also a high risk of falls and injuries, such as fractures, head injuries, bruising and dislocations.

How is PSP diagnosed?

PSP can be difficult to diagnose because the early symptoms are similar to other health conditions, most notably Parkinson’s disease.

Several types of test may be carried out to rule out other potential causes of symptoms, including strokes and Parkinson’s disease. Tests include CT and MRI scans and a PET scan (positive emission tomography). This test is used to identify radiation produced by substances injected into the body before the test begins. A DaT test can also be used to produce detailed images of the brain.

Sometimes it’s difficult to differentiate between PSP and Parkinson’s disease. In this case, a short course of medication may be prescribed to see if symptoms respond to treatment. The medication is known as levodopa. In cases of Parkinson’s disease, there is usually a significant improvement. However, in cases of PSP, improvement is minimal.

In some cases, patients may also be referred to a specialist neurologist for neuropsychological tests. These tests assess the ability to remember, concentrate and process information.

Treatment for PSP

There is no cure for PSP and sadly, there is no way of slowing the progress of the condition. However, there are treatments that can help to make people feel more comfortable and tackle some of the symptoms. Every individual with PSP is cared for by a multi-disciplinary team that includes a range of health professionals such as neurologists, physiotherapists, occupational therapist, speech therapists, social workers, ophthalmologists and specialist nurses.

There are no medicines proven to improve symptoms of PSP, however in the early stages, medication used to treat Parkinson’s disease may be beneficial. Sometimes, PSP can increase the risk of depression and anti-depressants may be prescribed in this case.

Physiotherapy can help to reduce stiffness, increase mobility and strengthen the muscles. Exercise is important, but it’s essential to do the right kinds of exercise and avoid overexertion. Breathing exercises can also help to reduce the risk of aspiration pneumonia, an infection that occurs when food debris falls into the lungs.

Speech and language therapy can help to improve speech and communication and also improve swallowing. As the ability to swallow becomes impaired, it may also be beneficial to follow an eating plan drawn up by a nutritionist.

PSP can affect eye sight and vision and regular eye tests are recommended. Botox can also be used to prevent involuntary movement of the muscles around the eyes. Some people prefer to wear dark glasses, as they become more sensitive to light.

As PSP is a progressive condition, palliative care is often provided in the advanced stages. This may be provided at a nursing home, hospice or hospital. It can often be beneficial for individuals and their families to plan in advance for when times get tougher and more assistance is required. The individual may have preferences in terms of where they want to be cared for and what kinds of care they hope to receive. Planning can also help to alleviate stress associated with making difficult decisions further down the line.

Living with PSP

Being diagnosed with PSP can be very distressing for the individual and their family. As the disease is progressive, you know that symptoms are going to get worse and gradually, you will need more help, support and assistance from the people around you, as well as health professionals. It can take time to come to terms with a diagnosis, but there are people who can help. Your care team can arrange counselling and the PSP Association also provides support for people affected by the condition.