Steele-Richardson-Olszewski Syndrome / Progressive Supranuclear Palsy
Progressive supranuclear palsy is a brain disorder that causes serious problems with walking, balance and eye movements. Although the cause is unknown, progressive supranuclear palsy results from deterioration of cells in areas of your brain that control movement.
Progressive supranuclear palsy can be difficult to diagnose because some signs and symptoms — such as stiffness, movement difficulties, and changes in mood or personality — are similar to those of Parkinson's disease and dementia. In fact, because of its similarity to Parkinson's disease, it's considered a "parkinsonism-plus syndrome."
Also known as Steele-Richardson-Olszewski syndrome, progressive supranuclear palsy affects one in every 100,000 adults, usually people older than 60, although it can start as early as age 40. No effective treatment for progressive supranuclear palsy exists. The disease is not life-threatening itself, but it can lead to life-threatening complications such as pneumonia and problems swallowing.
Treatment of progressive supranuclear palsy involves approaches to improve some signs and symptoms as well as lifestyle modifications to accommodate the condition.
The cause of progressive supranuclear palsy is not known. However, the signs and symptoms of the disease result from deterioration of brain cells in your brainstem, cortex and basal ganglia. These areas help you control body movements, and their deterioration leads to the coordination and movement problems of the disease.
Although no known cause exists, scientists do have several
theories about what might be behind the disease. These include :
- A virus. Some speculate that a unique virus enters your body, taking many years before it becomes active and starts causing signs and symptoms.
- Genetic mutations. It's possible that random changes (mutations), which may develop in everyone's genes, occur to specific genes to damage the cells involved in progressive supranuclear palsy.
- Environmental exposure. Some scientists think an unknown chemical in the environment could be what causes this disease. This chemical could be in something you eat, drink or breathe. For example, certain tropical fruits have been linked to progressive supranuclear palsy-like symptoms in people on some Caribbean islands.
- Damage from free radicals. As you process food for energy, your body produces substances called free radicals. Free radicals are believed to contribute to aging and certain diseases. They may damage the brain cells involved with the signs and symptoms of progressive supranuclear palsy.
The characteristic signs and symptoms of progressive supranuclear palsy include :
- A loss of balance while walking. You'll likely have a greater tendency to fall backward, not forward. This can occur even early on in the disease.
- An inability to aim your eyes properly. This is particularly true when looking downward, or may occur as a blurring of vision. This difficulty focusing the eyes can make some people appear disinterested in conversation because of poor eye contact or a blank appearance.
Signs and symptoms of progressive supranuclear palsy vary from person to person, however, and may mimic those of Parkinson's disease and dementia. These can include :
- Awkward movements
- Problems with speech and swallowing
- Loss of interest in pleasurable activities (apathy)
- Laughing or crying for no reason
If you are experiencing any of these signs and symptoms, see your doctor.
The only definitive way to determine whether a person has progressive supranuclear palsy is through a brain autopsy. However, through careful evaluation of a person's signs and symptoms, doctors can often correctly identify the condition before this.
Diagnosing progressive supranuclear palsy can be difficult because signs and symptoms are similar to those of Parkinson's disease and dementia. Diagnosis usually involves identifying the key signs — problems with balance and walking and difficulty moving your eyes, particularly downward — and ruling out other similar disorders.
Indications that the condition is progressive supranuclear palsy rather than Parkinson's disease include a lack of shaking (tremors) and a poor response to Parkinson's medications.
Complications of progressive supranuclear palsy result primarily from hindered muscle movements. Such complications include :
- Frequent falling, which can lead to head injuries, fractures and other injuries
- Difficulty focusing your eyes, which can also lead to injuries
- Problems reading or with other tasks requiring hand-eye coordination
- The need for a wheelchair
- Dependence on others for care
- Difficulty sleeping
Another significant complication includes problems swallowing. Swallowing difficulties 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.
No truly effective treatments for progressive supranuclear palsy exist. However, certain medications and other measures can help improve some symptoms.
- Parkinson's disease medications. These include levodopa, amantadine, and the antidepressants amitriptyline and desipramine (Norpramin), which increase levels of dopamine — a chemical that transmits signals between areas of your brain to allow smooth, controlled muscle movements. These medications may help improve slowness, stiffness and balance problems in about 30 percent of people. However, the effectiveness of the drugs is limited and usually temporary, lasting about two years.
- Other antidepressants. These drugs, including fluoxetine (Prozac) and imipramine (Tofranil), may improve symptoms, as well, although how they do this is not known; benefits don't seem to relate to their ability to treat depression.
- Botulinum toxin (Botox). This purified form of the toxin, which if taken in large amounts by mouth causes the food poisoning botulism, may be injected into the muscles or tissue around your eyes. When injected in small doses into specific muscles, Botox blocks the chemical signals that cause muscles to contract, which can improve eyelid spasms and other problems with eye movements.
- Eyeglasses with bifocal or prism lenses. These aids may help alleviate problems with looking downward.
- Speech and swallowing evaluations. Conducted by a speech therapist, these evaluations can help the therapist advise you on safer swallowing techniques.