Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a rare and progressive neurodegenerative disorder.

PSP can affect eye movements, walking, balance, speech, swallowing, mood, behaviour and thought processes (cognition).

Although there’s currently no cure for PSP, a range of drugs, treatments and therapies are available to manage the symptoms.

The management of PSP is focused on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL).

PSP symptoms most commonly occur in the person’s 60s, with slightly more men than women affected. The minimum age of onset for diagnostic purposes is currently set at 40.

PSP affects differing parts of the brain, including the brain stem, the basal ganglia and outer part of the brain – the cerebral cortex.

Some of the PSP symptoms are similar to those of Parkinson’s disease (PD), especially in the early stages of PSP.

PSP differs to PD in that it progresses faster and the symptoms are more severe.

Diagnosis of progressive supranuclear palsy

The diagnosis of PSP can be difficult to make, especially in the early stages.
There are currently no diagnostic tests available to confirm PSP.

A neurologist specialising in movement disorders is the recommended medical person to see when seeking a diagnosis.

The diagnosis of PSP can be difficulty to make because

the initial symptoms can vary greatly
the order in which the symptoms appear also varies
the early symptoms are often found with other conditions

PSP is often misdiagnosed as PD because

the early symptoms of PSP can be similar to those found in PD, such as stiffness and slowed movements
the PD like symptoms may improve with the use of PD medications, especially in the early stages

Causes of progressive supranuclear palsy

The deterioration in brain cells in several areas of the brain leads to the symptoms found in PSP.

In PSP there is an abnormal accumulation of the protein called tau in the brain.

The clumping of tau in nerve cells is thought to disrupt their function.

It is though that once the tau clumps in one cell, it goes on to infect connected cells with these protein clumps.

The cause of the tau clumping is not known.

In PSP there is damage above nerve cell clusters called nuclei, which disrupts the control of eye movements.

The substantia nigra in the basal ganglia is also affected in PSP. Changes to this part of the brain cause movements to be Parkinsonian or reduced in size and slow.

The basal ganglia is also affected in PD which explains why a person with PSP may have similar symptoms to someone with PD.

The cause of the brain cell degeneration is unknown.

To date genetics has not been found to be the cause of most cases of PSP. In rare cases, the mutation of the MAPT gene is said to have caused PSP.

No environmental or infective factors have been linked to PSP to date.

Symptoms of progressive supranuclear palsy

The onset of the symptoms, their progression and severity can vary greatly between people.

PSP results in a wide range of symptoms, which include:

stiffness or rigidity
slow movements
walking difficulties
falls
difficulty moving the eyes
mood and behavioural changes
thinking or cognitive problems
speech and swallowing difficulties
postural problems due to dystonia

PSP can be divided into the following clinical sub-types:

Steele-Richardson-Olszewski syndrome
PSP-parkinsonism
PSP-pure akinesia with ‘freezing’ when walking
PSP-corticobasal syndrome
PSP-progressive non-fluent aphasia

The most common symptoms in the early stages are unsteadiness and loss of balance when walking plus changes in thinking or behaviour and non-specific blurred vision. These symptoms occur in the Steele-Richardson-Olszewski syndrome.

In a third of people with PSP, their symptoms in the first 2 years are Parkinsonian and include slowness of movement, stiffness or rigidity and jerky tremor. Stiffness in the trunk and limbs is common. People with this PSP-parkinsonism do not experience unsteadiness, eye movement problems or changes to their thinking in these first few years.

As PSP progresses, most people with this disorder go on to develop all of the above symptoms. Symptoms tend to progress rapidly and unexplained falls increase in frequency and walking becomes more unsteady.

Dystonia, where muscle tightening is sustained and joints stiffen can cause the trunk to stiffen and the head to bend back. These postures may interfere with communication, eating, drinking and balance.

Moving the eyes becomes a more obvious problem as PSP progresses. Initially voluntarily moving the eyes up becomes difficult, progressing to difficulty moving the eyes down and eventually left and right. The eyes may close involuntarily, blinking becomes less frequent and opening the eyes may be delayed or difficult to do voluntarily.

Speech and swallowing difficulties are common in PSP and usually present early. These problems are more severe and progress more rapidly than in PD. Speech becomes slurred and slow. Swallowing fluids and solid foods can be difficult with the risk of choking and the food or fluids entering the lungs.

Changes in cognition (memory and thinking) and mood are more common in the early stages of PSP and are more severe in PSP compared to PD.

Early cognitive changes include memory and planning difficulties.

The most common mood changes are depression, apathy, and irritability.

Fatigue affects many people with PSP causing overwhelming tiredness, lack of energy and exhaustion.

Pain is very common with PSP.

Treatment of progressive supranuclear palsy

Currently there is no treatment available to stop or slow the progression of PSP.

There are, however, a variety of medical treatments and therapies available to manage the symptoms.

A multidisciplinary team that includes a neurologist and therapists who specialise in movement disorders is recommended for the management of the complex PSP symptoms. The appropriate multidisciplinary team members should be seen from the time the diagnosis of PSP is received.

Medical treatment

A neurologist specialised in movement disorders can advise on medical treatments to treat the presenting symptoms.

The symptoms of slow movement and rigidity may improve in the early stages with PD medications, especially levodopa preparations such as sinemet or madopar.

Botulinum injections may be used to treat troublesome dystonias.

Depression can be managed effectively with a range of antidepressants.

Education of symptoms and managing medications

A nurse with specialist training in PSP can provide education and support to the person with PSP and their caregiver. This may include:

monitoring the effects of changes to PSP medications asrecommended by the neurologist and liaising with the neurologist
providing education and support around the recommended medications and the assist with the minimisation of side effects
providing individualised education for the person with PSP and their caregivers to ensure optimal understanding and management of the various PSP symptoms as the condition progresses
arranging referrals to other relevant health professionals within the specialist team

Treatments for walking difficulties, balance, falls and posture problems

A physiotherapist specialised in movement disorders such as PSP can assess walking and balance problems and recommend ways to improve mobility and safety.

An occupational therapist specialised in movement disorders such as PSP can assess the person at home and suggest ways to improve safety, both inside and outside the home.

As PSP progresses the physiotherapist and occupational therapist can advise on appropriate seating to help achieve a good posture that is comfortable and makes eating and drinking easier.

A physiotherapist can advise on ways to help the person walk safely, reduce risk of falls and remain independent as long as possible. This may include the use of equipment, walking frames, protective garments such as hip protectors to reduce risk of hip fractures and the use of a wheelchair.

A physiotherapist can also develop an exercise program to improve or maintain flexibility, strength and fitness.

An occupational therapist may come to the home to advise on ways to improve mobility and safety in the home and the community. An occupational therapist can advise on safety aids to suit the needs of the person, such as rails next to steps, equipment for the shower and toilet. If home modifications are required, they can help with the planning of them.

If posture is affected and is interfering with communication, eating, drinking and comfort, the physiotherapist can prescribe exercises to stretch the tight muscles and joints and the occupational therapist can advise on equipment, such as seating.

The physiotherapist and occupational therapist can also assess the possible causes of any pain, such as poor posture, pain secondary to falls, pressure areas due to staying in one position too long.

An occupational therapist can assess and advise on pressure relieving equipment, such as specialised seating cushions and mattresses.

There are various funding bodies that can fully or partially cover the cost of some home modifications and adaptive equipment such the State Wide Equipment Program (SWEP). An occupational therapist can advise you on how to apply for funds to assist with the costs if required.

The person’s neurologist may suggest injections of botulinum toxin into the cramping or dystonic muscles that cause pain and poor posture.

Treatments for speech and swallowing difficulties

A speech pathologist specialised in movement disorders such as PSP can assess the person’s speech and teach strategies to improve them.

Changes to voice will vary between people and as the PSP progresses.

Swallowing difficulties are common in PSP and may lead to embarrassment when eating with others socially, loss of enjoyment of food, weight loss, poor nutrition and aspiration into the lungs. A speech pathologist can assess the person’s swallowing and suggest strategies to improve it.

It is important to see a speech pathologist if any of the following swallowing problems develop as they increase the risk of aspirating fluids or solids into the lung which may then lead to pneumonia:

if there is a need to clear the throat when eating solid foods or feeling food gets stuck
difficulty swallowing tablets which may feel ‘stuck’ in the back of the throat
coughing especially after drinking thin fluids such a tea and coffee
choking on solid foods such as meat, toast
wet or gurgly voice after having a drink

If there is a need to go on to a specialised thickened diet to avoid aspiration, the speech pathologist and dietitian will work together to advise on the food groups to select and the best way to prepare them.

PSP can lead to changes in the person’s saliva

the automatic swallowing of saliva can be disrupted in PSP leading to drooling
saliva or mucous may be very thick which can be very difficult to swallow
the mouth may be dry due to medications

These changes in saliva may cause social embarrassment as well as oral hygiene problems.

The speech pathologist can recommend ways to improve these problems with saliva and advise on ways to maintain good oral hygiene.

Management of nutrition

Adequate intake of nutritional food is essential with PSP.

Due to swallowing difficulties, mood changes and feeling of extreme fatigue, the person with PSP may not be eating well and therefore not getting enough nutrients. A dietitian can help plan nutritional meals to ensure the diet is right.

A dietitian specialised in movement disorders such as PSP can assess the person’s diet and provide them with suggested menus to improve any dietary problems they may have.

The dietitian can ensure the person with PSP is fully informed on what foods they need to eat in order to maintain a nutritional diet, how diet can improve constipation and how to avoid weight loss.

The dietitian will work closely with the speech pathologist to ensure the person with PSP and their caregiver and family know how to lessen the risk of aspiration when eating and drinking.

Managing the social and emotional impact

The effects of PSP are broad and impact on the social and economic life of the person and their family from the time of diagnosis in a variety of ways.

Coping and adjusting to the diagnosis, facing increasing challenges at work and planning for the future can be a daunting undertaking.

Navigating this newfound uncertainty requires careful consideration and appropriate specialised support.

As the disorder progresses, caregiver burden also increases and quality of life can decrease.

A social worker specialised in movement disorders such as PSP can assist the person with PSP, their caregiver and family negotiate the challenges faced across the life time of the person’s condition.

Social workers are highly skilled professionals who can assist you and your loved ones by:

providing a range of different therapy’s like counselling
assistance with linking to other support services
directing you to financial support services, information provision of entitlements for care packages
help to facilitate suitable respite arrangements for the caregiver
assistance and support with navigating the pathway to permanent residential care if the need arises.

After receiving a diagnosis of PSP it is normal to experience a change in mood. A social worker has the appropriate specialised skills to help you deal with the reaction to your diagnosis.

Depression and anxiety are common clinical symptoms of PSP. Depression and anxiety can benefit from a variety of interventions and approaches. It is important to talk openly with your doctor or neurologist if experiencing any of these to determine the best treatment plan for you.

Social workers are specifically trained to support you in understanding how best to manage such symptoms.

Cognition and behaviour

PSP causes changes to the person’s cognition. Cognition refers to our mental processes and includes the ability to learn, to reason and remember.

Worsening of cognition may make the person feel less confident and anxious.

Behaviour changes are common with PSP and include apathy, impulsive behaviour, mood swings.

It is important that the person with PSP, their caregiver and family speak to the neurologist about these changes.

A neuropsychologist specialised in movement disorders such as PSP can assess a person’s cognition and provide strategies to optimise independence.

The common cognitive changes include

Slowed thinking
having difficulty learning new things, storing information and recalling old information
having difficulty following conversations and finding the right words to express themselves
Poor planning and reasoning

The common behavioural changes include

apathy where there is a loss of enthusiasm with usual hobbies, loss if interest in socialising with family and friends
being impulsive which may increase the risk of harm, such getting up and walking unassisted even though they know not to walk alone

There are specialists, such as a neuropsychologist, who can assess the severity of these changes.

A neuropsychologist can assess cognition when the person is experiencing problems that are interfering with their ability to work or live independently and suggest strategies to use to address the underlying cognitive problems.

An occupational therapist specialised in PSP also has the skills to train the person in the use of a range of strategies to improve their independence, safety and reduce their impulsive behaviour.