Progressive Supranuclear Palsy (PSP) Symptoms, Stages, Treatment, and Life Expectancy
What is Progressive Supranuclear Palsy (PSP)?
- Progressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) is a rare degenerative disease of the brain (nerve cells).
- The disease impairs movements and balance. Many PSP patients also experience changes in mood, behavior, and personality.
- A decline in cognitive mental processes, such as thinking, memory, attention, and speech, is not uncommon. When these mental changes are severe enough to interfere with everyday activities, they are called dementia.
- The name progressive supranuclear palsy seems like a mouthful, but it accurately describes the disease. PSP is progressive, meaning that it gets worse over time. The disease affects the part of the brain above the nuclei (“supranuclear”), which are pea-sized structures in the part of the nervous system that controls eye movements.
- "Palsy" means weakness, and it is this characteristic weakness in eye movements for which the disease is named (Progressive Supranuclear Palsy).
- PSP is sometimes called Steele-Richardson-Olszewski syndrome, after the 3 scientists who first described it in 1963.
- PSP usually develops in people aged 60 years or older. Symptoms typically become noticeable in the early 60s, although the disease sometimes affects people in their 40s or 50s. PSP is slightly more common in men than in women, but it is a rare disease.
- About 20,000 PSP patients have been diagnosed with the disease.
- Because PSP mainly affects older people and has somewhat similar symptoms, it is often mistaken for Parkinson disease, a much more common movement disorder. The distinction is important, because treatments that help many people with Parkinson disease do not help those with PSP.
- Unfortunately, we do not yet have an effective treatment for PSP and other related disorders.
Progressive Supranuclear Palsy Causes: Is It Inherited?
The cause of PSP is not known. In a few rare cases, the disease is inherited (runs in families), but usually it does not. No links have been uncovered between PSP and surroundings, occupation, or lifestyle. Research is focusing on genes that may predispose a person to developing the disease.
Researchers have found that people with PSP have abnormal amounts of protein in the brain termed tau. This protein has been found in other neurodegenerative disorders. The tau protein has been associated with microtubules, and tau aggregates interfere with nerve communications. Although this clinical disease rarely occurs within a family, there is no clear genetic link. Corticalbasal degeneration is related to PSP and also involves tau protein. MRI (magnetic resonance imaging) and other neuroimaging methods may help distinguish these cerebral problems.
Medically Reviewed by a Doctor on 5/3/2017
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