Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP; also called Steele-Richardson-Olszewski syndrome) is a progressive, rare, parkinsonian -like disorder that can make it difficult to maintain balance, walk or control complex eye movements and may also interfere with cognitive functions. Over time, it can lead to such life-threatening complications as pneumonia and swallowing difficulties. Rarely does PSP occur repeatedly within a family and it usually appears around age 60 or older; the condition is almost unknown in people under the age of 40.

Early in the course of the disease, symptoms may manifest themselves as unexplained falls, a stiff and awkward gait and changes in personality which may include sudden outbursts of laughing or crying for no apparent reason. As the condition progresses, problems with controlling eye movements may appear:

• Trouble voluntarily shifting your gaze downward
• Difficulty controlling the movement of your eyelids 
• Involuntary closing of the eyes
• Prolonged or infrequent blinking
• Difficulty opening your eyes 
• Inability to maintain eye contact during a conversation

The cause of progressive supranuclear palsy is unknown; the symptoms result from the deterioration of brain cells in the areas that help control body movement (typically the midbrain) and cognition (the frontal lobe). Research has shown that the deteriorating brain cells of people with PSP have abnormal amounts of the tau protein; tangles or clumps of tau protein are characteristic of other neurodegenerative conditions such as Alzheimer's disease.

Diagnosis of Progressive Supranuclear Palsy
PSP symptoms are similar to Parkinson’s disease, so to determine whether you are experiencing PSP, our team at Houston Methodist will look for specific signs:

• A lack of shaking or tremors characteristic of Parkinson’s disease
• A poor response to medications used to control Parkinson's symptoms
• Difficulty moving your eyes, especially in a downward direction

We may recommend an MRI scan  to determine if the regions of your brain associated with PSP have started to shrink (as well as to eliminate other disorders which may mimic PSP). Our team may also suggest a positron emission tomography scan (PET).

Treatment Options for Progressive Supranuclear Palsy
Our team will help to alleviate the symptoms of PSP, including stiffness and balance, eye movement control and speech and swallowing difficulties, with several treatment options.

• Parkinson's disease medications, which increase levels of dopamine in the brain, may help with muscle control but the effectiveness of these medications is temporary, usually lasting two to three years.
• Injections of botulinum toxin (Botox®) into the muscles around your eyes can improve eyelid spasms.
• Eyeglasses with bifocal or prism lenses may reduce problems with looking downward.
• Speech and swallowing evaluations can teach you learn safer swallowing techniques so you can avoid choking.
• Physical therapy and occupational therapy may help strengthen muscles and improve balance. As part of physical therapy, we may introduce a walking aid to help you get around more effectively.

Minimally invasive surgery to place a feeding tube into the stomach may be necessary if choking becomes a hazard.