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How rare is progressive supranuclear palsy?

About 20,000 Americans — or one in every 100,000 people over age 60 — have PSP.
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What is the most common cause of supranuclear palsy?

What causes PSP? PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with PSP, it isn't broken down properly and forms harmful clumps in brain cells.
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Is PSP a rare disease?

Progressive supranuclear palsy (PSP) is a rare neurological disorder that affects your body movements, walking and balance, and eye movement. It results from damage to nerve cells in areas of the brain that control thinking and body movement.
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How common is progressive supranuclear palsy?

This disorder is believed to affect at least 20,000 people in the United States. According to some reports, PSP is estimated to affect as many as 5-17 in 100,000 people, but recent autopsy studies found PSP pathology in 2-6% of elderly people that had no diagnosis of PSP before death.
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What are the first signs of progressive supranuclear palsy?

Early symptoms
  • sudden loss of balance when walking that usually results in repeated falls, often backwards.
  • muscle stiffness, particularly in the neck.
  • extreme tiredness.
  • changes in personality, such as irritability, apathy (lack of interest) and mood swings.
  • changes in behaviour, such as recklessness and poor judgement.
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Progressive Supranuclear Palsy - Overview, Symptoms, Causes, Diagnosis, Treatments, and Prognosis

What percentage of people have PSP?

Progressive supranuclear palsy (PSP) is a rare neurodegenerative movement disorder, with an estimated annual prevalence of 5–7 per 100,000 persons [1,2] and annual incidence density rate between 0.9 and 2.6 per 100,000 persons [3,4], which both increase with age [5].
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How long can a person live with progressive supranuclear palsy?

PSP typically progresses to death in 5 to 7 years,1 with Richardson syndrome having the fastest rate of progression.
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What famous people died of progressive supranuclear palsy?

PSP is one of several progressive prime-of-life diseases that has claimed the lives of actors Robin Williams and Dudley Moore, and billionaire financier Richard Rainwater.
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Has anyone ever recovered from PSP?

There's currently no cure for PSP and no way to slow it down. But several treatments can help manage your symptoms and improve your quality of life.
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Which celebrity has PSP disease?

A famous person with PSP was Dudley Moore, the actor. He died at the age of 66 with this condition.
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Does PSP run in families?

Most cases of progressive supranuclear palsy are sporadic, which means they occur in people with no history of the disorder in their family. However, some people with this disorder have had family members with related conditions, such as parkinsonism and a loss of intellectual functions (dementia).
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What are the 4 stages of PSP?

The four stages are: Early stage. Mid stage. Advanced stage.
...
End of life stage:
  • Severe impairments and disabilities.
  • Rapid and marked deterioration in condition.
  • Decisions with regard to treatment interventions may be required, considering an individual's previously expressed wishes (advance decisions to refuse treatment).
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What is the average age of onset for progressive supranuclear palsy?

The only proven risk factor for progressive supranuclear palsy is age. The condition typically affects people in their late 60s and 70s. It's virtually unknown in people under the age of 40.
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What is the final stage of supranuclear palsy?

The final stages of PSP are usually dominated by an increasingly severe dysarthria and dysphagia. These features are usually described as being part of a pseudo-bulbar palsy, as brisk jaw and facial jerks may be present.
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What is the sister disease to Parkinson's?

Progressive supranuclear palsy is rare. It may be easily mistaken for Parkinson disease, which is much more common and has similar symptoms. But with PSP, speech and difficulty swallowing are usually affected more significantly than with Parkinson disease.
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How many people in the US have progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) is a rare brain disorder that causes dementia and problems with walking and balance. About 20,000 Americans — or one in every 100,000 people over age 60 — have PSP.
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Is PSP a form of dementia?

Progressive supranuclear palsy (PSP) is a frontotemporal dementia. It is characterised by difficulties with balance, movement, vision, speech and swallowing. It is caused by progressive damage to the cells in the brain that control eye movements.
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How does personality change with progressive supranuclear palsy?

PSP patients typically have deficits in cognitive functioning, difficulties with most daily activities, and present with notable behavioral disturbances—particularly apathy, impulsivity, and irritability.
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What happens in the last stages of PSP?

End-of-life stage: In the end-of-life stage of PSP, individuals may be bedridden and require around-the-clock care. They may have difficulty swallowing, become more susceptible to infections, and experience other complications related to the progression of the disease.
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Is PSP disease painful?

Pain was reported in 58 PD patients (89%), 17 MSA patients (81%), and four PSP patients (25%) (P < 0.01).
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Is PSP caused by stress?

Stress is associated with the development of neurofibrillary tangles via glucocorticoids. Hypertension is associated with an increased risk for PSP by inducing the aggravation of tau pathology and neuroinflammation.
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Do people with PSP sleep a lot?

Patients with PSP may have significant sleep pattern disruptions because the disease affects some neural circuitries that are involved in sleep processing. They may suffer from insomnia, including difficulties with falling and staying asleep.
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What are the traits of PSP?

As originally described, PSP was characterized by progressive supranuclear ophthalmoplegia, gait disorder and postural instability, dysarthria, dysphagia, rigidity, and frontal cognitive disturbance [1].
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Can people with PSP drink alcohol?

There was no significant association between PSP and alcohol drinking, smoking habits, anti- inflammatory agent use or specific occupations.
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Does PSP show up on MRI scan?

MRI scans can also detect changes to the brain that are consistent with a diagnosis of PSP, such as shrinkage of certain areas. Scans that show the build-up of the tau protein in the brain that's associated with PSP are currently under development.
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