Definition

What is progressive supranuclear palsy?

Progressive supranuclear palsy, also called Steele-Richardson-Olszewski syndrome, is an uncommon brain disorder that causes serious problems with walking, balance and eye movements. The disorder results from deterioration of cells in areas of your brain that control body movement and thinking.

Progressive supranuclear palsy worsens over time and can lead to life-threatening complications, such as pneumonia and swallowing problems.

How common is progressive supranuclear palsy?

Estimates vary, but only about three to six in every 100,000 people worldwide have progressive supranuclear palsy (PSP)—making it much less common than Parkinson’s disease. Symptoms of PSP begin on average after age 60, but may occur earlier. Men are affected more often than women. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of progressive supranuclear palsy?

The characteristic signs and symptoms of progressive supranuclear palsy include:

  • A loss of balance while walking. A tendency to fall backward can occur very early in the disease.
  • An inability to aim your eyes properly. You may have particular difficulty looking downward, or experience blurring and doubled vision. This difficulty with focusing the eyes can make some people spill food or appear disinterested in conversation because of poor eye contact.

Additional signs and symptoms of progressive supranuclear palsy vary and may mimic those of Parkinson’s disease and dementia. These signs and symptoms worsen as the disease advances, and may include:

  • Stiffness and awkward movements
  • Falling
  • Problems with speech and swallowing
  • Sensitivity to light
  • Sleep disturbances
  • Loss of interest in pleasurable activities
  • Impulsive behavior, possibly including laughing or crying for no reason
  • Difficulties with memory, reasoning, problem-solving and decision-making
  • Depression and anxiety
  • A surprised or frightened facial expression, resulting from rigid facial muscles

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes progressive supranuclear palsy?

The cause of progressive supranuclear palsy isn’t known. The signs and symptoms of the disorder result from deterioration of cells in areas of your brain, especially those that help you control body movements (midbrain) and thinking (frontal lobe).

Researchers have found that the deteriorating brain cells of people with progressive supranuclear palsy have abnormal amounts of a protein called tau. Clumps of tau are also characteristic of other neurodegenerative disorders, such as Alzheimer’s disease.

Rarely, progressive supranuclear palsy occurs within a family. But a genetic link isn’t clear, and most people with progressive supranuclear palsy haven’t inherited the disorder.

Risk factors

What increases my risk for progressive supranuclear palsy?

The only proven risk factor for progressive supranuclear palsy is age. The condition typically affects people around the age of 60, and is virtually unknown in people under the age of 40.

 

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is progressive supranuclear palsy diagnosed?

No specific laboratory tests or imaging approaches currently exist to definitively diagnose PSP.  The disease is often difficult to diagnose because its symptoms can be very much like those of other movement disorders, and because some of the most characteristic symptoms may develop late or not at all. Initial complaints in PSP are typically vague and fall into these categories:

  • Symptoms of disequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness
  • Visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble
  • Slurred speech
  • Various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood.

An initial diagnosis is based on the person’s medical history and a physical and neurological exam. Diagnostic scans such as magnetic resonance imaging may show shrinkage at the top of the brain stem. Other imaging tests can look at brain activity in known areas of degeneration.

PSP is often misdiagnosed because it is relatively rare and some of its symptoms are very much like those of Parkinson’s disease. Memory problems and personality changes may also lead a physician to mistake PSP for depression, or even attribute symptoms to some form of dementia. The key to diagnosing PSP is identifying early gait instability and difficulty moving the eyes, speech and swallow abnormalities, as well as ruling out other similar disorders, some of which are treatable.

How is progressive supranuclear palsy treated?

There’s no cure for progressive supranuclear palsy, so treatment focuses on managing the signs and symptoms.

The treatment options include:

  • Parkinson’s disease medications, which increase levels of a brain chemical involved in smooth, controlled muscle movements (dopamine). The effectiveness of these medications is limited and usually temporary, lasting about two to three years.
  • Onabotulinumtoxin type A (Botox), which may be injected in small doses into the muscles around your eyes. Botox blocks the chemical signals that cause muscles to contract, which can improve eyelid spasms.
  • Eyeglasses with bifocal or prism lenses, which may help ease problems with looking downward.
  • Speech and swallowing evaluations, to help you learn safer swallowing techniques.
  • Physical therapy and occupational therapy, to improve balance.

Researchers are working to develop medication to treat progressive supranuclear palsy, including therapies that may block the formation of tau.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage progressive supranuclear palsy?

To minimize the effects of progressive supranuclear palsy, you can take certain steps at home:

  • Use eyedrops to help ease dry eyes that can occur as a result of problems with blinking or persistent tearing.
  • Install grab bars in hallways and bathrooms, to help you avoid falls.
  • Use a walker that is weighted, to help you avoid falls.
  • Remove scatter rugs or other items that are hard to see without looking downward.
  • Avoid climbing stairs.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: November 8, 2017 | Last Modified: November 8, 2017

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