What is Progressive Supranuclear Palsy (PSP)?

Progressive supranuclear palsy (PSP) is a rare brain disorder that affects approximately 6 in 100,000 people worldwide. It causes muscle weakness and affects gait, balance and overall movement, as well as the patient’s mood, cognitive skills and behavior.

It was distinguished from Parkinson’s disease in 1963 as the “Steele-Richardson-Olszewski syndrome”. It is a progressive condition that usually shows onset around the age of 60 years and worsens over time.

PSP affects the areas of the brain located above clusters of nerve cell bodies called the “nuclei” – hence the name, “supranuclear” (“above the nuclei”). It is a neurodegenerative disorder classified as a “tauopathy”, as it is associated with pathological aggregation of tau protein in the brain.

Progressive Supranuclear Palsy (PSP) Symptoms

PSP results in weakness of the muscles (palsy) and affects the patient’s ability to walk, maintain balance, and swallow, as well as visual difficulties. It thus commonly leads to falling, lunging forward when moving, or bumping into objects. In particular, the motor neurons controlling the extraocular muscles are affected. This results in the blurring of vision as one of the more distinct symptoms of PSP and is important in diagnosing the condition.

Apart from motor function, the person’s mood and behavior are also affected. This can lead to apathy and depression, as well as slowness of thinking. Speech and swallowing are also affected – it is common for the person to have a slow, slurred speech and experience difficulty swallowing due to muscle weakness, which may lead to malnutrition.

PSP Causes

The mechanism that causes PSP is not fully known. It appears that there are pathological amounts of tau protein aggregating in the brain. This type of protein is also linked to other neurodegenerative disorders including Parkinson’s disease. Particularly the parts of the brain that are involved in controlling movement (the midbrain) and thinking (the frontal lobe) are affected. The only risk factor established so far is advanced age and no evidence has been found so far that PSP is a genetic disease. It is usually a sporadic condition (not inherited), occurring in people with no family history of PSP.

Progressive Supranuclear Palsy (PSP) Diagnosis

When making a diagnosis of PSP, it is important to make a distinction from other neurodegenerative disorders, in particular Parkinson’s disease, as there are a number of overlapping symptoms.

Some distinct symptoms of PSP that aid the right diagnosis include:

  • no tremors (no shaking),
  • difficulty focusing vision, particularly when looking down (downward palsy),
  • very little or no response to medication used in Parkinson’s disease.

An MRI brain scan sometimes followed by a PET scan may be advised in order to determine which areas of the brain are affected by the disease.

PSP Management

No effective treatment is currently possible for those affected by PSP. However, the various symptoms can be managed with the use of medication or other forms of help.

Some of the options include:

  • medication, including that used in the management of Parkinson’s disease
  • botulinum toxin A (botox) injections
  • prescription glasses
  • physical and occupational therapy
  • speech therapy

Making additional adjustments to the lifestyle as well as one or more of a number of useful home remedies may also aid in minimizing the effects of PSP and ensuring the best possible quality of life for the patient.

Sources

Further Reading

Last Updated: Oct 22, 2018

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