Overview

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

Progressive supranuclear palsy care at Mayo Clinic

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Symptoms

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

When to see a doctor

Make an appointment with your doctor if you experience signs and symptoms of progressive supranuclear palsy.

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Causes

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.

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Diagnosis

Progressive supranuclear palsy can be difficult to diagnose because signs and symptoms are similar to those of Parkinson's disease. Indications that you have progressive supranuclear palsy rather than Parkinson's disease include:

• A lack of shaking (tremors)
• A poor response to Parkinson's medications
• Difficulty moving your eyes, particularly downward

Your doctor may recommend MRI to determine if you have shrinkage in specific regions of the brain associated with progressive supranuclear palsy. MRI can also help to exclude disorders that may mimic progressive supranuclear palsy, such as stroke.

A positron emission tomography (PET) scan also may be recommended to check for early signs of brain abnormalities that may not appear on MRI.

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Complications

Complications of progressive supranuclear palsy result primarily from hindered muscle movements. These complications may include:

• Falling, which could lead to head injuries, fractures and other injuries.
• Difficulty focusing your eyes, which also can lead to injuries.
• Difficulty sleeping.
• Difficulty looking at bright lights.
• Problems swallowing, which can lead to choking or inhaling food or liquid into your airway (aspiration). Aspiration can develop into pneumonia, the most common cause of death in people with progressive supranuclear palsy.
• Impulsive behaviors — for example, standing up without waiting for assistance — which can lead to falls.

To avoid the hazards of choking, your doctor may recommend a feeding tube. To avoid injuries due to falling, a walker or a wheelchair may be used.

Lifestyle and home remedies

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.

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Coping and support

Living with any chronic illness can be difficult, and it's normal to feel angry, depressed or discouraged at times. Progressive supranuclear palsy presents special problems because it can cause changes in your brain that make you feel anxious or laugh or cry for no reason. Progressive supranuclear palsy can also become extremely frustrating as walking, talking and eating become more difficult.

To manage the stress of living with progressive supranuclear palsy, consider these suggestions:

• Maintain a strong support system of friends and family
• Contact a support group, for yourself or for family members
• Discuss your feelings and concerns about living with progressive supranuclear palsy with your doctor or a counselor

For caregivers

Caring for someone with progressive supranuclear palsy can be difficult physically and emotionally. It's not easy to juggle tasks as you try to adapt to the constantly fluctuating moods and physical needs that accompany this condition. Remember that these moods and physical capabilities may change from hour to hour and are not under the person's conscious control.

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Risk factors

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.

Source: http://www.mayoclinic.com