Progressive Supranuclear Palsy (Symptoms, Stages, Treatment, and Life Expectancy)

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What is Progressive Supranuclear Palsy (PSP)?

  • 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 disease, a much more common movement disorder. The distinction is important, because treatments that help many people with Parkinson disease do not help those with PSP.
  • Unfortunately, we do not yet have an effective treatment for PSP and other related disorders.

Progressive Supranuclear Palsy Causes: Is It Inherited?

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

Dementia, Alzheimer's, and Aging Brains

PSP Symptoms and Signs

The signs and symptoms of progressive supranuclear palsy usually appear very slowly. Many people experience a prolonged phase of symptoms such as fatigue (feeling tired), headaches, joint pains, dizziness, and depression.

Gradually, some or many patients with the condition may have more specific symptoms that include:

  • Unexplained balance problems
  • Stiff or awkward steps while walking
  • Very slow movements
  • Frequent falls, clumsiness and/or postural instability
  • 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).
  • Behavior or personality changes - Irritation, grouchiness
  • Memory loss, forgetfulness
  • Apathy (indifference)
  • Slowed thinking, reasoning, planning
  • Inappropriate laughing or crying
  • Angry or aggressive outbursts
  • Slurred speech
  • Swallowing problems - Solids and liquids
  • Mask-like facial expression (no expression)
  • Muscle spasms
  • 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 disease and stroke-related dementia. These symptoms are more likely to occur in later stages of the disease.

When Should I Contact a Doctor or Other Health Care Professional If I Have Symptoms of the Disease?

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 to make arrangements for care while he or she can still take part in making the decisions.

Why Am I Fatigued and Tired All of the Time?

Most everyone has been fatigued at some point, and there are numerous causes of it. The majority of diseases known to man often list fatigue or malaise as possible associated symptoms. This complicates things because normal healthy people experience fatigue as a normal response to physical and mental exertion that only lasts a couple of days.

So when is fatigue something to be concerned about?

If you are feeling fatigued and it never goes away (becomes chronic and doesn't go away), or if it becomes extreme it may be a sign or symptom of a medical condition. See your doctor or other health care professional if you think it may be more than just daily physical and mental exertion.

Can Progressive Supranuclear Palsy Be Cured?

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.

How Is Progressive Supranuclear Palsy Diagnosed?

Many different conditions can cause dementia symptoms. According to the National Institutes of Health, there is no blood or imaging test to confirm that you have PSP. However, some researchers suggest that an MRI that demonstrates atrophy of the midbrain tegmentum (shaped like a hummingbird) approaches nearly 100% PSP diagnosis while others report about 70% specificity. Your doctor has the difficult task of finding the cause of your symptoms. This is very important, because some causes of dementia are reversible with treatment, while others are not.

The process of narrowing down the clinical possibilities to reach your diagnosis is complicated. Your health care provider will gather information from several different sources. At any time in the process, he or she may consult an expert in brain disorders (neurologist or psychiatrist).

The first step in the evaluation is the medical interview. You will be asked questions about your symptoms and when they appeared, about medical problems now and in the past, about medications you have taken now and in the past, about family medical problems, about your work and travel history, and about your habits and lifestyle. You may need a family member to help you answer these questions. A physical examination will look for physical disabilities and signs of underlying conditions such as high blood pressure and heart disease. It will include a mental status examination. This involves answering questions and following simple directions. Neuropsychological testing may be done to identify the extent of dementia. Other tests and/or imaging systems may be done to help distinguish PSP from ET (essential tremor or et), Parkinson disease, multisystem atrophy or Alzheimer’s.

Lab tests

No lab test can confirm the diagnosis of PSP. However, blood tests may be done to rule out other conditions that cause dementia symptoms. These include infections, blood disorders, chemical abnormalities, hormonal disorders, and liver or kidney problems.

Imaging studies

Brain scans are not very helpful in establishing the diagnosis of PSP in some patients, but they can rule help out many other neurodegenerative disease conditions that cause dementia in a patient.

  • MRIs and CT scans may be done to rule out other causes of dementia such as stroke. MRIs may also show additional changes in the later stages of the disease. This does not confirm PSP because these changes can occur in other conditions that cause dementia.
  • Positron-emission tomography (PET) or single-photon emission computed tomography (SPECT) scan sometimes is helpful in distinguishing PSP from Parkinson disease and other conditions. These scans are available only at certain large medical centers.

Neuropsychological testing

Neuropsychological testing is the most accurate method of pinpointing and documenting a person’s cognitive problems and strengths. Results vary with the site and severity of damage in the brain.

  • This testing can help give a more accurate diagnosis of the problems and thus can help in treatment planning.
  • The testing involves answering questions and performing tasks that have been carefully prepared for this purpose. Testing is done by a psychologist or other specially trained professional.
  • It evaluates the individual’s appearance, mood, anxiety level, and experience of delusions or hallucinations.
  • It assesses cognitive abilities such as memory, attention, orientation to time and place, use of language, and ability to carry out various tasks and follow instructions.
  • Reasoning, abstract thinking, and problem solving also are tested.

Other tests

Sleep studies may be ordered because sleep patterns are often abnormal in people with PSP. You may undergo a sleep test called a polysomnogram.

Dementia, Alzheimer's, and Aging Brains

Drug Treatment Therapy to Manage PSP Symptoms

Symptoms and complications of PSP can be managed with drug therapy, but the underlying disease is not affected. No known treatment can stop PSP. 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 PSP. 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 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: the tricyclic antidepressants (TCAs) and the selective serotonin reuptake inhibitors (SSRIs). These drugs slightly improve symptoms in some people with PSP. Nonetheless, antidepressants are usually helpful to treat depression, which commonly accompanies the disorder. Examples include:
    • amitriptyline
    • imipramine (Tofranil)
    • fluoxetine (Prozac)
    • sertraline (Zoloft)
    • bupropion (Wellbutrin)

Tips and Lifestyle Changes to Manage Symptoms and Maintain Quality of Life

Individuals with PSP 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 from 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 PSP. 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 PSP should not smoke, both for health and safety reasons.

What's the Prognosis and Life Expectancy for Progressive Supranuclear Palsy?

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

Is There A Way to Prevent Progressive Supranuclear Palsy?

There is no known way to prevent PSP.

Which Specialties of Doctors Diagnose and Treat PSP Symptoms?

The medical care of a person with PSP 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 PSP.
  • 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.

Support Groups and Counseling

PSP 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 PSP, 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
Reviewed on 9/11/2017

REFERENCE:

Nelson, S. "Hummingbird sign in PSP." June 24, 2013.
<https://www.aan.com/residents-and-fellows/e-pearl-of-the-week/June-24-2013/>

Kato, N. et al. "Study of the rostral midbrain atrophy in progressive supranuclear palsy." J Neurol Sci. 2003;210:57–60.
<http://www.jneurosci.org/content/by/year>

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