Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder that damages certain areas of your brain. It affects how you walk, think, swallow and move your eyes. “Progressive” means that it gets worse over time.

PSP may closely resemble Parkinson’s disease (PD), especially early on. But PSP symptoms change faster than those of PD. Healthcare providers tend to misdiagnose it as Parkinson’s disease at first.

About 1 per 100,000 people get a PSP diagnosis each year. It used to be called Steele-Richardson-Olszewski syndrome.

Types of this condition

There are at least eight types of progressive supranuclear palsy. They all share similar symptoms. But certain symptoms are the main features for each type. The two most common types include:

• PSP-Richardson syndrome (PSP-RS): This is the classic form of the condition. Its initial features are instability, frequent falls and an inability to look down.
• Parkinson’s disease-like variant (PSP-P): A tremor (shakiness) is the main symptom of this type. PSP-P may respond better to antiparkinsonian medications than other types, at least for some time.

These two types make up 3 in 4 cases of PSP.

Symptoms of progressive supranuclear palsy

Progressive supranuclear palsy symptoms vary depending on the type. At first, you may have just a few and then develop more later. In general, each symptom becomes more severe over time.

The most common first signs of PSP include:

• A loss of balance, which can lead to falling backward
• A stiff walking pattern (gait)
• Difficulty looking downward with your eyes
• A wide-eyed staring expression

Other symptoms of PSP include:

• Personality changes, like apathy and impulsivity
• Stiff muscles in your neck and trunk that affect how you move
• Difficulty swallowing
• Difficulty speaking, like distorted speech and poor volume control
• Blurry or double vision
• Depression and/or anxiety
• Uncontrolled or inappropriate episodes of laughing or crying (pseudobulbar affect)
• Trouble with reasoning and problem-solving
• Sleep difficulties, like insomnia

Progressive supranuclear palsy causes

Scientists don’t know the exact cause of progressive supranuclear palsy. But they know that a protein called tau is involved. Tau is important for brain health. When you have PSP, the tau in your brain clumps together. The clumps then damage nerve cells.

This disease impacts various parts of your brain at different stages. The condition tends to affect your basal ganglia and brainstem early and more severely. Eventually, it’ll spread to most of your brain.

Researchers have several theories for why tau clumping might happen. Some include:

• Random genetic changes (not inherited)
• Unknown infectious agents
• An unknown chemical in the air, water or your food that slowly damages your brain

PSP rarely runs in families. If you have the disease, there’s very little additional risk that your biological siblings or children will get it.

Risk factors

The main risk factor for PSP is age. The condition most commonly affects people over the age of 60. It very rarely develops before the age of 40.

There’s no known way to prevent this disease.

End stages of PSP

During the later stages of PSP, care is necessary for all daily activities. This can happen as early as three to four years into living with the condition. Complications involve:

• Inability to walk
• Significant muscle stiffness
• Severe difficulty with swallowing and talking
• Problems with bowel and bladder functions
• Weight loss

PSP complications can be fatal. The most common cause of death is aspiration pneumonia. This often results from swallowing difficulties. You inhale something other than air (like food) into your lungs. Falls resulting in bone breaks or head injuries can be fatal complications as well.

How doctors diagnose progressive supranuclear palsy

It can be difficult for healthcare providers to diagnose PSP. They often mistake it for Parkinson’s disease at first. There’s no single test that can diagnose PSP. Providers usually diagnose it based on your symptoms and imaging tests.

A brain MRI is the go-to imaging test. This helps rule out other possible causes of your symptoms.

The diagnostic process can be frustrating. You may feel like you’re fighting for an answer to understand what’s happening to your body. If you feel like you aren’t being heard, don’t hesitate to push back. If possible, try to speak to healthcare providers who specialize in movement disorders.

How is PSP treated?

There’s currently no cure for progressive supranuclear palsy and no way to slow it down. But several treatments can help manage your symptoms and improve your quality of life. Your care team will include a range of medical specialists.

Examples of treatments include:

• Antiparkinsonian medications, like levodopa and amantadine, which may help movement issues temporarily
• Antidepressants for clinical depression
• Talk therapy to aid in coping with the condition
• Physical therapy to manage pain, stiffness and discomfort
• Occupational therapy to help you do daily tasks safely
• Speech therapy to improve speaking and swallowing issues
• Botox® (botulinum toxin) injections to temporarily relax the muscles of your eyelids
• Eye drops and artificial tears to alleviate dry eyes due to reduced blinking
• Special glasses with prism lenses to help you see what’s below your line of vision
• Wraparound glasses if you’re sensitive to bright light
• Percutaneous endoscopic gastrostomy (PEG) if you develop significant trouble swallowing

Researchers are also studying new therapies. Ask your healthcare team if a clinical trial is an option for you.

Palliative care

Palliative care is an important part of progressive supranuclear palsy care. And hospice is a valuable resource in the late stages.

Palliative care complements the care you receive from your healthcare team. It helps you live more comfortably — with the medical, social and emotional support needed to cope with a serious illness.

It also provides support to caregivers and loved ones.

What can I expect if I have this disease?

Most people with PSP need around-the-clock care within three or four years of symptoms beginning. On average, the condition leads to death six to nine years after the diagnosis. The sooner you receive a diagnosis and start a treatment plan, the better quality of life you’ll have.

Each case is different. You’ll be in frequent contact with your healthcare team. They’ll keep you updated on your outlook as time goes by. Don’t hesitate to ask them questions.

How can I care for someone with progressive supranuclear palsy?

Support from family and friends is just as important as medical care for someone with PSP. Here are ways you can help:

• Learn about the condition. Learning how PSP progresses and what each symptom looks like can help you spot changes early. This will help your loved one get timely care.
• Ask which symptoms bother them the most. Knowing your loved one’s unique experience with PSP can help you support them better.
• Don’t assume they’re in distress. Symptoms like uncontrollable crying or repetitive vocalizations may be alarming to witness. But they don’t necessarily mean your loved one is distressed. Before assuming, ask them how they’re feeling.
• Help make their living space safer. Get rid of rugs and other tripping hazards to help prevent falls.
• Seek help from social, legal and financial services. These services are just as important as medical care, especially as the end-of-life stage approaches.
• Take care of yourself: It can be challenging to adjust to the changing moods and physical needs of PSP. Caregiver burnout is a real thing. Take breaks and seek support from your community and/or a mental health professional.