What is PSP?
What is Progressive Supranuclear Palsy?
Progressive supranuclear palsy is a brain disorder that affects movement, vision, speech, and thinking ability (cognition). The signs and symptoms of this disorder usually become apparent in mid- to late adulthood, most often in a person's 60s. Most people with progressive supranuclear palsy survive 5 to 9 years after the disease first appears, although a few affected individuals have lived for more than a decade.
Loss of balance and frequent falls are the most common early signs of progressive supranuclear palsy. Affected individuals have problems with walking, including poor coordination and an unsteady, lurching gait. Other movement abnormalities develop as the disease progresses, including unusually slow movements (bradykinesia), clumsiness, and stiffness of the trunk muscles. These problems worsen with time, and most affected people ultimately require wheelchair assistance.
Progressive supranuclear palsy is also characterized by abnormal eye movements, which typically develop several years after the other movement problems first appear. Restricted up-and-down eye movement (vertical gaze palsy) is a hallmark of this disease. Other eye movement problems include difficulty opening and closing the eyelids, infrequent blinking, and pulling back (retraction) of the eyelids. These abnormalities can lead to blurred vision, an increased sensitivity to light (photophobia), and a staring gaze.
Additional features of progressive supranuclear palsy include slow and slurred speech (dysarthria) and trouble swallowing (dysphagia). Most affected individuals also experience changes in personality and behavior, such as a general loss of interest and enthusiasm (apathy). They develop problems with cognition, including difficulties with attention, planning, and problem solving. As the cognitive and behavioral problems worsen, affected individuals increasingly require help with personal care and other activities of daily living.
Estimates vary, but only about three to six in every 100,000 people worldwide, or approximately 20,000 Americans, have PSP—making it much less common than Parkinson's disease (another movement disorder in which an estimated 50,000 Americans are diagnosed each year).
PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson's disease. It was sometimes referred to as Steele-Richardson-Olszewski syndrome, reflecting the combined names of the scientists who defined the disorder.
Currently there is no effective treatment for PSP, but some symptoms can be managed with medication or other interventions.
Quick Facts of PSP:
- Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that has no known cause or cure
- It affects brain cells that control balance, walking, coordination, eye movement, speech, swallowing, and thinking
- Five to six people in 100,000 have PSP
- Symptoms begin, on average, when an individual is in the early 60’s, but may start as early as in the 40’s
- PSP is slightly more common in men than women, but it has no known geographical, occupational, or racial preference
Symptoms of PSP include:
- Loss of balance
- Changes in personality
- Weakness of eye movements, especially in the downward direction
- Weakened movements of the mouth, tongue, and throat
- Slurred speech
- Difficulty swallowing
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