Progressive Supranuclear Palsy

What is Progressive Supranuclear Palsy?

Progressive Supranuclear Palsy (PSP) is a progressive neurological disorder that affects both the brainstem, and deep (basal ganglia) parts of the brain important for control of balance, vision and speech. It is a relatively rare disorder, affecting only around 4,000 people in the UK according to the PSP association.

Why haven’t I heard of PSP before?

PSP is rare and so many people will never come across it.  Because of patients’ physical difficulties and stiffness of the limbs, patients are often assumed to be suffering from Parkinson’s disease or ‘atypical Parkinsonism’.

What are the symptoms?

A common first symptom is difficulty in walking, with loss of balance. People may have unexplained falls, usually backwards, and may experience stiffness, particularly in the neck. People may have difficulty shifting their gaze up or down or controlling their eyelids, leading to slow or infrequent blinking and difficulty opening the eyes. This may also result in visual difficulties. Problems in speech may also occur in PSP.  Sufferers’ speech may become slurred (dysarthria), and they can find it difficult to generate speech.  Swallowing problems may also occur.

In addition to these physical symptoms, there may be problems in cognition.  However, these are usually mild, and may include slowness in thinking, increased reaction times, as well as difficulties in memory and planning ability.

Will the symptoms become worse?

The disease is progressive, so symptoms do get worse over time.  However, there is a wide variation in the speed of progression.  Some people show little decline over the course of a year whereas others change more quickly.  Usually, monitoring a person’s progress over a year will give a good guide as to the likely future rate of progression.

At what age are people affected?

The disorder most commonly occurs after the age of 60, although it can occur in younger people. 

What causes the disease?

The disorder is a degenerative condition of the brain. This means that cells in certain parts of the brain gradually stop working properly, which is how symptoms develop. This is related to the build-up in the brain of a particular protein called Tau. This causes damage and the areas of the brain affected may shrink (atrophy). We do not know why this protein build-up occurs and this is the subject of a lot of research. We know that this condition is not caused by external environmental or lifestyle factors, such as diet, occupational exposure or head injury. It can affect people from all walks of life and is not simply the result of ‘over-use’ or ‘under-use’ of the brain.

Is PSP inherited?

In most cases, PSP is sporadic, which means  it occurs ‘out of the blue’, with no history of similar problems in other family members. However, in a very small number of cases, the condition appears to run in families, suggesting a genetic component. Generally, the risk of PSP being inherited is very low if no other family members are affected.  If you or your family have particular concerns or questions please let us know. 

Is there any treatment?

Current treatments do not provide a cure for the disease. However, there are drugs available, which are designed to alleviate symptoms. Some of these drugs are the same as those used in Parkinson’s disease. The most effective way to manage the disorder is through occupational, physical, and speech therapy.  Although the disease will continue to progress, therapies such as these may help to improve aspects of day-to-day living.  Physical therapy may help to maintain a range of motion in stiff joints, in turn helping to prevent pain and sustain mobility.  Occupational therapy may be useful in providing and developing helpful adaptive equipment.  Speech therapy may improve clarity and volume of the voice. Your doctor will discuss treatment options with you in your clinic appointment.

Is there any research into PSP?

Yes, certainly. The interest in this area of research increases each year. The aim is to understand the mechanisms that underlie this disorder, so that better treatments can be developed. If you would like to find out more about the research carried out into PSP, please contact the PSP Association or ask when you attend the clinic.