Progressive Supranuclear Palsy (PSP)

Picture of nerve conduction of the brain.

• The life expectancy of someone with progressive supranuclear palsy typically is six to ten years after their diagnosis.
• Progressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) is a rare degenerative disease of the brain (nerve cells).
• The disease impairs movements and balance. Many PSP patients also experience changes in mood, behavior, and personality.
• A decline in cognitive mental processes, such as thinking, memory, attention, and speech, is not uncommon. When these mental changes are severe enough to interfere with everyday activities, they are called dementia.
• The name progressive supranuclear palsy seems like a mouthful, but it accurately describes the disease. PSP is progressive, meaning that it gets worse over time. The disease affects the part of the brain above the nuclei (“supranuclear”), which are pea-sized structures in the part of the nervous system that controls eye movements.
• "Palsy" means weakness, and it is this characteristic weakness in eye movements for which the disease is named (Progressive Supranuclear Palsy).
• PSP is sometimes called Steele-Richardson-Olszewski syndrome, after the 3 scientists who first described it in 1963.
• PSP usually develops in people aged 60 years or older. Symptoms typically become noticeable in the early 60s, although the disease sometimes affects people in their 40s or 50s. PSP is slightly more common in men than in women, but it is a rare disease.
• About 20,000 PSP patients have been diagnosed with the disease.
• Because PSP mainly affects older people and has somewhat similar symptoms, it is often mistaken for Parkinson's disease, a much more common movement disorder. The distinction is important because treatments that help many people with Parkinson's disease do not help those with PSP.
• Unfortunately, we do not yet have an effective treatment for PSP and other related disorders.

The signs and symptoms of progressive supranuclear palsy usually appear very slowly. Many people experience a prolonged phase of symptoms such as:

1. Fatigue (feeling tired)
2. Headaches
3. Joint pain
4. Dizziness
5. Depression

Gradually, some or many people with progressive supranuclear palsy may have more specific symptoms that include:

1. Unexplained balance problems
2. Stiff or awkward steps while walking
3. Very slow movements
4. Frequent falls, clumsiness, and/or postural instability
5. Visual problems - Blurry or double vision, problems controlling eye movements (inability to maintain eye contact), light sensitivity (eye movement problems comprise a strong sign of PSP).
6. Behavior or personality changes - Irritation, grouchiness
7. Memory loss, forgetfulness
8. Apathy (indifference)
9. Slowed thinking, reasoning, and planning
10. Inappropriate laughing or crying
11. Angry or aggressive outbursts
12. Slurred speech
13. Swallowing problems - Solids and liquids
14. Mask-like facial expression (no expression)
15. Muscle spasms
16. Inability to hold urine (incontinence) - Late stages only

The pattern of symptoms varies widely from person to person. Often, friends and family members are more aware of these changes than the affected person.

Most patients with PSP display the characteristic findings of slowness of movement, stiffness, balance difficulty, and eye movement problems. The classic eye movement problem is an inability to voluntarily move the eyes downward, although this problem may take time to develop. Ultimately, all voluntary eye movements may be lost.

Generally, the cognitive and behavioral symptoms are mild and less severe than in other types of dementia, such as Alzheimer's disease and stroke-related dementia. These symptoms are more likely to occur in the later stages of the disease.

The cause of PSP is not known. In a few rare cases, the disease is inherited (runs in families), but usually it does not. No links have been uncovered between PSP and surroundings, occupation, or lifestyle. Research is focusing on genes that may predispose a person to develop the disease.

Researchers have found that people with PSP have abnormal amounts of protein in the brain termed tau. This protein has been found in other neurodegenerative disorders. The tau protein has been associated with microtubules, and tau aggregates interfere with nerve communications. Although this clinical disease rarely occurs within a family, there is no clear genetic link.

Corticobasal degeneration is related to PSP and also involves tau protein. MRI (magnetic resonance imaging) and other neuroimaging methods may help distinguish these cerebral problems.

• Causes, similar symptoms, and signs: Progressive supranuclear palsy and Parkinson's disease both cause similar signs and symptoms of stiffness, movement difficulties, and clumsiness, but progressive supranuclear palsy comes on more quickly and progresses as compared to Parkinson's disease. 
• Axil rigidity: People with progressive supranuclear palsy have "axial rigidity," in which the person usually stands exceptionally straight or on occasion tilts their head backward, which tends to make them fall back. People with Parkinson's disease usually bend forward. 
• Speech and problems with swallowing: Problems with speech and swallowing are more common and severe in progressive supranuclear palsy than in Parkinson's disease, and they tend to show up earlier during the disease. 
• Eye movements: People with progressive supranuclear palsy have abnormal eye movements, and the movements are near normal in Parkinson's disease. 
• Shared symptoms: Progressive supranuclear palsy and Parkinson's disease share symptoms and characteristics, for example, they:
  • Begin in late middle age people
  • Have slow movement or bradykinesia
  • Have rigid muscles
• Tremors: Tremors are very common in people with Parkinson's disease, but are rare in those with progressive supranuclear palsy. 
• Brain cell proteins: Individuals with progressive supranuclear palsy show an accumulation of tau, a type of protein in the brain cells, while people with Parkinson's disease show an accumulation of alpha-synuclein, a different type of protein.
   

Any change in movement, walking, balance, behavior, mood, or personality may signal a problem. A visit to the person’s health care provider is a good idea if the change interferes with any of the following:

• The ability to take care of himself or herself
• The ability to maintain health and safety
• The ability to sustain social relationships
• The ability to work effectively at his or her job
• The ability or interest in participating in activities that he or she enjoys
• The ability to drive or carry out other complex tasks

Many conditions can cause dementia or dementia-like symptoms (for example, Parkinson's disease, metabolic disorders, and Creutzfeldt-Jakob disease) including both medical and psychological problems. Some of these conditions can be reversed, or at least stopped or slowed. Therefore, it is extremely important that the person with symptoms be checked thoroughly to determine if he or she has a treatable condition and to not misdiagnose this progressive disease.

Early diagnosis allows the affected person to plan activities and make arrangements for care while he or she can still take part in making the decisions.

Symptoms and complications of progressive supranuclear palsy can be managed with drug therapy, but the underlying disease is not affected. No known treatment can stop progressive supranuclear palsy. Many different medications have been tried, but with little success. In general, drug therapy helps only a few people with PSP, and the improvement is mild and temporary.

• Antiparkinson drugs: Medications used to treat Parkinson's disease have been tried in progressive supranuclear palsy. These drugs work by increasing the amount of a brain chemical called dopamine or by reducing the level of another brain chemical called acetylcholine. A few patients with PSP have improved with these medications, but the improvement is short-lived and incomplete. One of these drugs may be given to help determine whether a person has PSP or Parkinson's disease. Examples include:
  • levodopa and carbidopa (Sinemet)
  • bromocriptine (Parlodel)
  • ropinirole (Requip)
  • pramipexole (Mirapex)
• Antidepressant medications: Antidepressants work by altering the chemistry of the brain. Two different types are used: tricyclic antidepressants (TCAs) and selective serotonin reuptake inhibitors (SSRIs). These drugs slightly improve symptoms in some people with progressive supranuclear palsy. Nonetheless, antidepressants are usually helpful to treat depression, which commonly accompanies the disorder. Examples include:
  • amitriptyline
  • imipramine (Tofranil)
  • fluoxetine (Prozac)
  • sertraline (Zoloft)
  • bupropion (Wellbutrin)

Individuals with progressive supranuclear palsy should remain physically, mentally, and socially active as long as they are able.

• Daily physical exercise helps keep the body strong and flexible and stimulates the mind. A physical therapist can recommend safe exercises. Walking aids can help keep a person mobile while reducing the danger of falls.
• The individual should engage in as much mental activity as he or she can handle. Puzzles, games, reading, and safe hobbies and crafts are good choices. Ideally, these activities should be interactive. They should be of an appropriate level of difficulty to ensure that the person does not become overly frustrated.
• Social interaction is stimulating and enjoyable for most people with progressive supranuclear palsy. Most senior centers or community centers have scheduled activities suitable for people with dementia.
• A balanced diet that includes low-fat protein foods and plenty of fruits and vegetables helps maintain a healthy weight and prevent malnutrition and constipation.
• An individual with progressive supranuclear palsy should not smoke, both for health and safety reasons.

At present, there is no cure for progressive supranuclear palsy. The goals of treatment are to effectively manage symptoms (such as walking and swallowing problems) and to maximize safety and quality of life.

If you have progressive supranuclear palsy, you and your family caregiver will have frequent visits with your health care team. Your team can help you and your family members adjust to the changing needs brought on by the disease. They will also check you for new or worsening symptoms and complications and will alter your treatment as necessary.

PSP is a progressive disease that may leave you vulnerable to a number of life-threatening complications.

• Difficulty swallowing is a choking hazard.
• Resulting in eating problems increase the risk for malnutrition.
• Walking difficulties and balance problems increase the risk of falling. Head injuries and other injuries from falls can be serious.
• Most people become unable to walk later in the disease. They become confined to a bed and chair. This immobility increases the risk of infections such as pneumonia and urinary tract infections.
• Immobility also increases the risk of breathing problems.

People with PSP usually need a walking aid, such as a cane or walker, within 3-4 years of the first symptoms of the disease. With good care and attention to medical needs, nutritional needs, and safety, a person with PSP can live many years.

The typical lifespan from the first appearance of symptoms is about 6-10 years. The main causes of death are infections and breathing problems.

There is no known way to prevent progressive supranuclear palsy.

The medical care of a person with progressive supranuclear palsy usually involves a team of health professionals. The team provides different aspects of care under the supervision of a leader, usually a neurologist.

• A rehabilitation specialist and an occupational therapist can make recommendations that help keep the person safely mobile and independent for as long as possible. Walking aids such as canes or walkers help many people with progressive supranuclear palsy.
• An ophthalmologist (eye doctor) can manage eye and vision symptoms and rule out other conditions that may cause similar symptoms.
• A speech therapist can help the person speak more clearly or develop other means of communicating. These professionals can also offer suggestions for reducing the risk of choking.
• A dietitian can offer suggestions for eating to maintain proper nutrition while reducing the risk of choking.
• A surgeon can place a feeding tube in the stomach in a simple procedure called a gastrostomy. This is necessary for people who cannot swallow enough food to maintain good nutrition.

Progressive supranuclear palsy is a difficult disease for you and those who care for you. It can be particularly stressful for family caregivers. The condition affects every aspect of life, including family relationships, work, financial status, social life, and physical and mental health. If you are a family caregiver for a person with progressive supranuclear palsy, you may feel overwhelmed, depressed, frustrated, angry, or resentful. These feelings may in turn leave you feeling guilty, ashamed, and anxious.

While understandable, these feelings do not help the situation and usually make it worse. This is why support groups were invented. Support groups are groups of people who have lived through the same difficult experiences and want to help themselves and others by sharing coping strategies. Support groups serve a number of different purposes for a person living with the stress of being a caregiver for a person with PSP.

• The group allows the person to express his or her true feelings in an accepting, nonjudgmental atmosphere.
• The group’s shared experiences allow the caregiver to feel less alone and isolated.
• The group can offer fresh ideas for coping with specific problems.
• The group can introduce the caregiver to resources that may be able to provide some relief.
• The group can give the caregiver the strength he or she needs to ask for help.

Support groups meet in person, on the telephone, or on the Internet. To find a support group that works for you, contact the organizations listed below. You can also ask your health care provider or behavior therapist, or go on the Internet. If you do not have access to the Internet, go to the public library.

For more information about support groups, contact the following agencies:

• Family Caregiver Alliance, National Center on Caregiving - (800) 445-8106
• Society for Progressive Supranuclear Palsy, Inc - (800) 457-4777
• Eldercare Locator Service - (800) 677-1116