Progressive Supranuclear Palsy
Progressive supranuclear palsy is a rare brain disease that primarily affects how you walk, move your eyes, think, and swallow. Although it shares some features with Parkinson’s disease, it progresses faster and has unique characteristics that set it apart.
Table of contents
- What is Progressive Supranuclear Palsy?
- Alternative Names
- How Common is PSP?
- Common Symptoms
- How PSP Differs from Parkinson’s Disease
- Types of Progressive Supranuclear Palsy
- What Causes PSP?
- How is PSP Diagnosed?
- Treatment Options
- Outlook and Life Expectancy
What is Progressive Supranuclear Palsy?
Progressive supranuclear palsy, or PSP, is a rare brain disease that affects certain areas of your brain over time. The name provides clues about the condition: “progressive” means symptoms get worse over time, “supranuclear” refers to the part of the brain above the centers that control eye movement, and “palsy” means weakness or difficulty using your muscles[1][2].
The disease results from damage to brain cells in areas that control body movement, coordination, thinking, and other important functions. As these brain cells become damaged and die, they affect how you walk, balance yourself, move your eyes, speak, swallow, and think[1][3].
PSP belongs to a group of conditions called atypical parkinsonism (also known as Parkinson-plus disorders) because it shares some features with Parkinson’s disease but has distinct differences[2][4].
Alternative Names
Steele-Richardson-Olszewski syndrome
PSP is also known as Steele-Richardson-Olszewski syndrome, named after the doctors who first officially described it in 1963[1][2][6].
How Common is PSP?
PSP is considered a rare condition. Around 5 to 10 people out of every 100,000 have it, with about 30,000 cases in the United States[2][5]. The actual numbers may be higher because PSP is frequently misdiagnosed as other conditions[5].
The condition most commonly affects people over the age of 60, with symptoms typically beginning in a person’s mid- to late-60s. It very rarely develops before the age of 40[2][4][5]. PSP is slightly more common in men than women, but it has no known geographical, occupational, or racial preference[5].
Common Symptoms
The symptoms of progressive supranuclear palsy vary from person to person and tend to begin gradually, typically becoming more severe over years[2].
The most common first signs of PSP include a loss of balance when walking or climbing stairs, which often leads to frequent falls, especially falling backward. Many people also experience difficulty looking downward with their eyes and develop a wide-eyed staring expression[1][2][4].
As the disease progresses, additional symptoms develop. Problems with eye movements become more noticeable. People may have slow eye movements, trouble looking up or down, difficulty controlling their eyelids, involuntary closing of the eyes, decreased blinking, or difficulty opening the eyes. Some people develop a tendency to move their head rather than just their eyes to look in different directions[4].
Other physical symptoms include stiff muscles that affect your ability to move, difficulty speaking (speech might be quieter, slurred, or monotone), and difficulty swallowing. The swallowing problems can lead to choking or cause food to enter the lungs, potentially resulting in pneumonia (a serious lung infection)[1][2][4].
PSP also affects thinking, mood, and behavior. People may experience mood changes such as depression, apathy (loss of interest in activities), and irritability. Personality changes and behavioral changes including impulsivity and poor judgment can occur. Some people develop memory problems, difficulty finding words, slowed thinking, and forgetfulness. As the disease advances, some individuals may develop dementia (a decline in thinking abilities severe enough to interfere with daily life)[2][4].
Sleep problems are also common, including insomnia and a condition called REM sleep behavior disorder. Many people with PSP experience sensitivity to bright light[2].
How PSP Differs from Parkinson’s Disease
PSP is often mistaken for Parkinson’s disease, especially in the early stages, because they share some symptoms including stiffness, slowness of movement, and problems with balance[2][4]. In fact, more than 50% of people with PSP are initially misdiagnosed with Parkinson’s disease[5].
However, there are several key differences between the two conditions. People with PSP tend to lean backward and extend their neck, while those with Parkinson’s tend to bend forward. Falls in PSP usually result from falling backward, while people with Parkinson’s tend to fall forward[4].
A major difference is that tremor (shaking) is a characteristic symptom of Parkinson’s disease, but people with PSP typically do not experience tremor[4][5]. Additionally, PSP progresses more rapidly than Parkinson’s disease. Most people with PSP develop severe disability within three to five years of symptom onset[4].
Another important distinction is that medications used to treat Parkinson’s disease usually have limited effectiveness in PSP. Healthcare professionals often begin to reconsider the diagnosis when symptoms progress faster than expected or when typical Parkinson’s medications aren’t working[5].
Types of Progressive Supranuclear Palsy
There are four different types or presentations of PSP. They all share similar symptoms, but there are some unique differences[2].
The two most common types are Richardson syndrome (PSP-RS) and the Parkinson’s disease-like variant (PSP-P). Together, these two types make up 75% of PSP cases[2].
Richardson syndrome is the most common form of PSP and typically includes issues with walking and balance, abnormal speech, memory and thinking problems, difficulty controlling eye movement (especially looking down), and a wide-eyed staring facial expression[2].
The Parkinson’s disease-like variant (PSP-P) has similar symptoms to Richardson syndrome but more closely resembles Parkinson’s disease. In this type, tremor is the main symptom instead of balance problems and behavioral changes. People with PSP-P may respond better to medications used for Parkinson’s disease, at least for some time[2].
The other two types include corticobasal syndrome and pure akinesia with gait freezing[2].
What Causes PSP?
Scientists don’t know the exact cause of progressive supranuclear palsy. However, experts know that a protein called tau is involved in the disease process. Tau is an important protein for brain health that occurs naturally in the brain and is usually broken down before it reaches high levels[2][3].
In people with PSP, tau is not broken down properly and forms harmful clumps in brain cells. This buildup causes brain cells in certain parts of the brain to become damaged and die. The areas most affected include the brainstem, cerebral cortex, cerebellum, and basal ganglia—a cluster of cells deep within the brain[1][3].
The condition has been linked to changes in certain genes, but these genetic faults aren’t inherited. The risk to other family members, including children or siblings of someone with PSP, is very low[3].
How is PSP Diagnosed?
There is no single test to diagnose PSP. Instead, diagnosis is based on the pattern of symptoms and ruling out other conditions that can cause similar problems[3][5].
A healthcare professional may suspect PSP rather than Parkinson’s disease if you don’t have tremors, are having many unexplained falls, have little or no response to Parkinson’s medicines, or have trouble moving your eyes, particularly downward[8].
You may need an MRI (magnetic resonance imaging) scan to look for shrinkage in specific regions of the brain associated with PSP. An MRI can also help rule out other disorders that may cause similar symptoms, such as stroke. A PET (positron emission tomography) scan may be recommended to check for early signs of brain changes that might not appear on an MRI[8].
Tests of memory, concentration, and ability to understand language may also be performed. The diagnosis must be made or confirmed by a specialist with expertise in PSP, usually a neurologist (a doctor who specializes in conditions affecting the brain and nerves)[3].
Because many healthcare providers are unfamiliar with the disease, receiving an accurate diagnosis can take a long time and be challenging and frustrating for patients and families[5].
Treatment Options
There is currently no cure for progressive supranuclear palsy, and there are no medications that can stop the disease from progressing. However, treatment focuses on relieving symptoms and helping people with PSP maintain the best possible quality of life[3][8].
Because PSP affects people in many different ways, treatment and care involve a team of health and social care professionals working together. This is known as a multidisciplinary team[3][9].
Medications used to treat Parkinson’s disease, such as levodopa, may be tried to help with muscle stiffness and slowness of movement. The effectiveness of these medicines is limited and usually temporary, lasting about 2 to 3 years in most patients[8][9].
Injections of botulinum toxin (Botox) may be given in small doses into the muscles around the eyes to help with eyelid spasms and improve eye control. The effects usually last for up to 3 months[8][9].
Antidepressants can help with depression and may also assist with pain, bladder and bowel problems, and disturbed sleep[8][9].
Physical therapy is important to help maintain mobility through exercise while avoiding overexertion. A physiotherapist can recommend equipment such as walking frames or specially designed shoes to reduce the risk of slipping and falling. They can also teach breathing exercises to use when eating to reduce the risk of developing aspiration pneumonia[9].
Speech and language therapy can help improve speech clarity and manage swallowing problems. Therapists can teach techniques to make your voice clearer and advise about communication aids. They can also recommend changes to the consistency of food to make swallowing easier[9].
Occupational therapy can provide advice on how to increase safety and prevent falls during daily activities. An occupational therapist can identify potential hazards in the home, such as poor lighting or loose rugs, and recommend modifications[9].
When swallowing problems become severe and put you at risk of malnutrition or dehydration, a feeding tube may be recommended. The main type used is called a PEG (percutaneous endoscopic gastrostomy) tube, which is placed into the stomach through the abdomen during a procedure[9].
Special glasses with prisms may help improve vision problems. A weighted walking tool can help prevent falling backward[8].
Outlook and Life Expectancy
PSP is a progressive disease, meaning symptoms continue to worsen over time. The disease usually worsens rapidly, and most people with PSP develop severe disability within three to five years of symptom onset[4].
PSP can lead to serious complications such as pneumonia (often caused by breathing food into the lungs), choking from swallowing difficulties, or head injuries from falls[1][4].
With good care and assistance, a person with PSP can maintain greater independence and enjoy a better quality of life. However, there will come a point when someone with PSP is at risk of developing serious complications. It’s important to discuss future care preferences with your healthcare team early on[3].
Research is ongoing, with several clinical trials investigating potential new treatments that aim to relieve symptoms and slow down disease progression[11].
