PSP may look like Parkinson’s, but affects vision, thinking and balance early on.

Progressive Supranuclear Palsy (PSP) is an adult onset, neurological condition that affects balance, thinking and eye movements.

What is Progressive Supranuclear Palsy?

Progressive Supranuclear Palsy (PSP) is an adult-onset neurological disease, classified as a degenerative brain disorder. It is named due to characteristic eye movement problems that result from damage deep in the brain in an area called the midbrain. Aside from eye movement problems, PSP may affect movement and balance, thinking and speech.

PSP is sometimes referred to as atypical parkinsonism or “Parkinson’s-plus” disorder as it may share some symptoms with Parkinson’s disease. However, PSP is a distinct condition that progresses and is managed differently.

There are many different “types” of PSP that are based on the symptoms the person experiences, particularly early on in the condition, but they are all caused by the same brain changes. The most common type is referred to as PSP Richardson syndrome (or PSP-RS).

PSP is a rare disease and based on global estimates affects 6 to 7 out of 100,000 people. These figures mean that in Australia, it is estimated that around 1,800 people are currently living with PSP. These numbers are likely underestimates due to frequent misdiagnosis of Parkinson’s disease or another frontotemporal dementia. The average age of onset of Progressive Supranuclear Palsy (PSP) symptoms is between 50 and 70 years, but may be a little older or younger, and males are more commonly affected.

Causes

Progressive Supranuclear Palsy (PSP) is a neurodegenerative condition. This means that abnormal proteins accumulate in certain brain regions and result in damage over time. In PSP, a protein called tau builds up inside brain cells (in Parkinson’s, it’s alpha-synuclein and in Alzheimer’s disease it’s amyloid and tau proteins). These clumps of tau seem to spread through different parts of the brain, causing cells to stop working properly and eventually die. As more cells are lost, the connections that keep the brain functioning smoothly also break down and cause the symptoms of PSP.

We still don’t know the exact reasons why these tau proteins become misfolded and accumulate, but research shows that the damage usually happens in parts of the brain that help control movement and balance, especially in a part of the brain stem called the midbrain. Sometimes the proteins accumulate in different brain areas first, or may spread differently from person to person, and this explains why different people with PSP may have very different symptoms, especially early on.

In general, PSP is not a genetic condition that is passed down in families. Only a very small number of cases are linked to changes in the MAPT gene, which gives the body instructions for making tau. When this gene doesn’t work as it should, the tau protein can form abnormally.

Researchers are exploring several possible explanations for why PSP develops, including:

  • Random genetic changes in certain brain cells that, when combined in a particular way, may cause harm.
  • Environmental factors, such as long‑term exposure to something in food, air, or water that could slowly damage vulnerable parts of the brain.
  • Damage from free radicals, which are reactive molecules produced naturally by the body. Normally, the body clears them away, but under some conditions, they may build up and harm cells.

Symptoms

Remember, there is considerable variation in the symptoms and rate of progression in people with PSP. The most common early signs and symptoms of PSP include loss of balance and falls (often, but not always falling backwards), a wide-eyed staring expression and difficulty moving eyes up and down (which can cause issues with reading and vision, and discomfort when looking at bright light, called photophobia), stiffness of muscles particularly in the neck which can cause headaches, and cognitive decline. Sometimes PSP may look very similar to Parkinson’s disease with a tremor of one arm or leg, or present with trouble speaking.

As the disease progresses over years, people usually notice progression in their symptoms. Not all people experience all symptoms, but may notice some of these changes:

  • Difficulty swallowing food and fluids (called dysphagia) which can cause choking or aspiration and lung infections
  • Speech issues include reduced volume and harsh or growling character, mixing up of words or using the wrong word can also occur
  • Vision issues include difficulties with focusing, looking up or down, jerky eye movements or double vision
  • Involuntary closing (or inability to open) your eyelids “eyelid opening apraxia” or lack of blinking resulting in dry and irritated eyes
  • Behavioural and cognitive changes ranging from irritability, impulsivity, memory problems, slow processing speed, difficulties with planning or abstract reasoning, anxiety, depression or apathy. Changes in personality are commonly reported by family members and carers.
  • Sometimes people with PSP lack insight into the severity of their symptoms, especially cognitive impairment. For example, they may insist that they are safe to drive despite visual problems, poor spatial awareness, and slow reactions.
  • Involuntary laughing or crying can occur, and often happens in the absence of feeling happy or sad, this is sometimes referred to as emotional incontinence or “pseudobulbar affect”
  • People become increasingly immobile as PSP progresses, and will usually require assistance with a walking stick, four-wheel walker or wheelchair at some point
  • Constipation, lack of bowel control and urinary problems may develop.

Diagnosis

The diagnosis of PSP is “clinical” meaning that there is no blood test or brain scan that can confidently diagnose or exclude the condition. Due to the variety of the initial symptoms, it can be very difficult for doctors to diagnose PSP accurately, especially at the first visit. In the early stages, PSP can look a lot like Parkinson’s or types of dementia, which means it’s sometimes mistaken for these conditions before a clearer diagnosis is made.

Often a specialist, usually a neurologist, is required to make a diagnosis of PSP, and not all doctors or allied health staff will know much about the condition.

Brain scans (MRI or PET scan) can be helpful to look for shrinkage (atrophy) of certain brain regions and to exclude other causes of similar symptoms. Blood tests are usually not helpful to help make a diagnosis but may be important to exclude other conditions.

Consider participating in a PSP clinical trial: https://www.parkinsons.org.au/clinical-trial/assessing-cognitive-trajectories-in-psp/

Your Care

Your GP is an essential member of your care team, and you should have regular visits with them as your condition changes. Communication between your GP, geriatrician, neurological specialist and allied health providers is essential for ensuring you have the best possible care.

Access and support in the community are provided by two government programs, depending on your age and other factors. Your GP may also be able to assist with chronic disease management plans or GP mental health care plans.

Those who are diagnosed under the age of 65 may be eligible for the NDIS, and it is important for you to ask your doctor and care team if you should apply for this and how to access it. After the age of 65, you will likely be eligible to receive government support through the MyAgedCare “Support at Home program” (formerly called “Home care package”).

There is currently no cure for PSP. Treatment focuses on managing symptoms and improving quality of life.

There are so many things you CAN do to help manage symptoms:

  • Medications:
    • Some drugs used for Parkinson’s (e.g., levodopa) may provide limited benefit, and are often trialled by your specialist
    • Anti-depressants are generally safe and may help with mood symptoms or emotional incontinence or pseuobulbar affect
    • Sometimes botulinum toxin injections can be used by your specialist. In PSP, these injections can help manage stiffness and pain of the neck muscles, or to treat involuntary closing of the eyelid muscles.
  • Physiotherapy and Accredited Exercise Physiologist
    • Physical activity can help maintain mobility, balance and reduce fall risk.
    • Can assist with the provision of safe walking aides
    • Regular physical therapy can help with stiffness of muscles and joints
  • Speech pathology:
    • Early assessment by an experienced speech pathologist is usually recommended by your specialist
    • PSP can cause swallowing problems, and this can lead to choking or aspiration and lung infections
    • Speech pathologists can also assist with communication problems, which may arise due to a strained, growling and low-volume speech, or problems with language (mixing up words)
  • Occupational therapy:
    • Assists with daily activities and home safety. Functional independence is helped by occupational therapy. Changes to the home environment are often necessary. As PSP is progressive, some future needs can be anticipated, and equipment can be accessed and installed ahead of time. A safe home can help people maintain independence for as long as possible and reduce the risk of falls.
    • You may be asked to undergo an occupational therapy driving assessment by your doctor
  • Optometrist:
    • If eye movements are restricted, prism lenses and other strategies can be effective to help manage these symptoms
  • Clinical psychologist
    • Mental health support can be useful for adjusting to the life-changing diagnosis and management of depression or anxiety symptoms for both the person diagnosed and their care partner
  • Social worker
    • A social worker can be particularly helpful in navigating the complexities of the healthcare system and provide support to people with PSP and their carers

Parkinson’s nurse specialists are familiar with this condition and can be a valuable resource to support the person and their family.

Palliative Care

As PSP progresses, palliative care becomes essential to manage symptoms and maintain dignity. Early engagement with specialist palliative care services is advised, and they can provide support throughout different stages of the illness.

TOP TIPS

Prognosis:

The prognosis is quite different for different people with PSP. The most common type, PSP Richardson syndrome, tends to progress faster than others. On average, people live for 6 to 10 years after their symptoms begin, but this can vary from person to person.

The most common complications for PSP are injuries due to falls, lung infections from aspiration or choking on food and urinary tract or other infections. It is important to have regular evaluations with your care team to prevent these complications.

Advance Care Planning (ACP)

As PSP is a progressive illness without a cure advance care planning (ACP) is strongly recommended. ACP helps avoid crisis decisions and ensures your wishes are being met.

  • Document preferences for medical care early.
  • Discuss wishes with family and your care team.
  • Consider advance directives to ensure care aligns with personal values.

Carer support

  • Get signed up with the Carer Gateway as soon as possible to access key supports like respite and carer payments
  • Find your supports, carer help apps and more can help manage the complexity of caring for someone with PSP
  • Reach out to friends and family when you need to

Key Statistics

Based on the 2023 meta-analysis prevalence estimate, around 1,800–1,900 Australians are likely living with Progressive Supranuclear Palsy (PSP). These numbers are likely underestimates due to frequent misdiagnosis of Parkinson’s disease or another frontotemporal dementia.

⚠️ Important note: This is a statistical estimate. More Australian research is needed.

Infosheet kindly reviewed by PSP researcher, Timothy Siejka

Dr Timothy Siejka is a neurologist at Eastern Health and Alfred Health, Melbourne. He has subspecialty training and expertise in movement disorders and cognitive neurology, specialising in neurodegenerative disease including Alzheimer’s disease, Parkinson’s disease, and Progressive Supranuclear Palsy (PSP).

He is a PhD candidate at Monash University, School of Translational Medicine supervised by Professor Amy Brodtmann. His research focusses on improving the measurement of cognitive decline in PSP. Outside of his PhD, he led the establishment of the Alfred-Monash Biological DataBank for atypical parkinsonian syndromes, which has recruited over 60 participants to date.

References

Happy middle aged man with neat white beard and glasses looking at a laptop

Donations

Your donation helps us to achieve our core business, to improve the lives of people affected by Parkinson’s in Australia through national advocacy, raising awareness and connecting people living with Parkinson’s, their families and carers, researchers and health professionals in the Parkinson’s community.