What is progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) – or Steele-Richardson-Olszewski syndrome – is a rare neurodegenerative disorder that damages your brain (particularly the basal ganglia, substantia nigra, subthalamic nucleus, pars reticulata and other midbrain structures) and affects how you walk, think, swallow and move your eyes. It may also cause a number of other symptoms. PSP is often confused with Parkinson’s disease. There is no proven cure or disease-modifying treatment.

“Neurodegenerative” refers to the collapse of your nervous system, especially the death of neurons and gia (certain types of cells) in your brain. “Progressive” means that the symptoms get worse over time and “palsy” means that the disorder impairs your eye movements. “Supranuclear” refers to the damage localized above the eye-moving centers (nuclei) in your brain.

Is progressive supranuclear palsy (PSP) fatal?

PSP itself does not directly kill. Unfortunately, PSP symptoms increase the risk of complications such as pneumonia, which can be deadly. Aspiration pneumonia is the most common cause of death in people with PSP. It happens because the muscles in your throat become weak and uncoordinated, and food and drink accidentally trickle down your windpipe and into your lungs.

Another common cause of death in PSP falling. You may suffer fractures and head trauma.

What parts of the brain does progressive supranuclear palsy (PSP) affect?

Eventually PSP will spread to virtually every part of your brain. PSP affects various parts of your brain to a varying extent and at various stages of the disease. Your basal ganglia and brainstem tend to be affected early and more severely. The brainstem is responsible for a large number of vital functions, including your ability to swallow, and posture control. The basal ganglia also help with posture, as well as eye movements, thinking and emotions. Additionally, the basal ganglia are essential to the control of automated movements.

Is there a cure for progressive supranuclear palsy (PSP)?

There is no cure for PSP, but there are ways to manage your symptoms to improve your quality of life. (See the “Treatment” section.) Also, there is extensive ongoing research to find treatments that will halt, slow, or prevent disease progression.

Who is at risk of getting progressive supranuclear palsy (PSP)?

You are at a greater risk of developing PSP if you are age 60 or older. Men are slightly more likely to get PSP than women.

How common is progressive supranuclear palsy (PSP)?

Five to six out of every 100,000 people have PSP. About one per 100,000 gets diagnosed each year.

What are the types of progressive supranuclear palsy (PSP)?

There are many types of PSP but the two most common types are Richardson syndrome and PD-like variant (PSP-P) – which means a Parkinson’s disease-variant. Together, they make up 75% of PSP cases. The symptoms are similar, but there are some unique differences.

Richardson syndrome has many symptoms including problems with walking and balance, abnormal speech, memory and thinking problems, trouble controlling eye movement (especially looking down) and an unusual wide-eyed, staring facial expression. People with Richardson syndrome also have personality changes, forgetfulness, apathy (loss of interest), depression, irritability and problems with attention and concentration. These are the most common symptoms.

PD-like variant (PSP-P) consists of the above symptoms, but also more closely resembles Parkinson’s disease. A tremor (an involuntary muscle contraction) is the main symptom over balance problems and behavioral changes. PSP-parkinsonism responds better to antiparkinson drugs.

What is the difference between progressive supranuclear palsy and Parkinson’s disease (PD)?

PSP frequently resembles Parkinson’s disease, often making it difficult to distinguish these conditions from one another. Both cause difficulty with stiffness and clumsiness. Both cause slow movement and start later in life. And both show damage to the same areas of the brain that affect movement. In particular, PSP-P can be nearly indistinguishable from Parkinson’s disease by clinical examination.

But, PSP and Parkinson’s disease are different in several ways. On average, PSP gets worse quicker than Parkinson’s and doesn’t respond as well to medications. People with Parkinson’s usually bend forward, while people with PSP stand very straight, or even slightly backwards. Problems with swallowing and with speaking appear early with PSP and they are far more severe. A tremor is rare with people who have PSP and common in people who have Parkinson’s. Motor symptoms, such as slowing and loss of dexterity, typically start very asymmetrically in Parkinson’s disease (one side of the body is significantly worse than the other, whereas a more symmetric presentation is typical in PSP even during early stages of the disease).

Additionally, their underlying pathological processes differ at the molecular level. Abnormalities relating to the protein tau and its effect on brain cells is central to PSP pathology. And, when looked at through a microscope, the damaged brain cells in someone with PSP look different than those of people who have Parkinson’s.

What causes progressive supranuclear palsy (PSP)?

The exact cause of PSP is unknown. However, experts have determined that a protein called tau is involved. Tau is an important protein that helps with brain health. It holds together and stabilizes microtubules. (Microtubules help with transporting cells, among other purposes.) Tau also helps preserve the normal structural integrity of neurons, and maintain genomic DNA.

When you have PSP, the tau in your brain aggregates, which means that it clumps together. The clumps damage the neurons (brain cells).

What are the signs and symptoms of progressive supranuclear palsy (PSP)?

If you have PSP, you’ll likely experience the following symptoms that worsen with time. You’ll likely:

  • Lose your balance frequently. You may fall, especially backwards.
  • Lose the ability to aim your eyes. You might not be able to look up or down, follow something that’s moving, or keep your eyes fixed on something still. Your eyelids might retract (pull back), giving your face a surprised look.
  • Have trouble swallowing.
  • Feel stiff muscles and move slowly.
  • Have trouble speaking. Your speech might be quieter and slurred, impairing your ability to pronounce words.
  • Feel mood changes and observe behavioral changes.
  • Experience depression and apathy (loss of interest).
  • Get dementia. Your thoughts will be slow, you’ll have difficulty resisting impulses and you may find problem-solving difficult.
  • Have insomnia (inability to sleep).
  • Feel irritable and agitated.

Are there signs and symptoms of progressive supranuclear palsy (PSP) that are more common, or appear first?

The most common first sign is a loss of balance when walking or climbing stairs. You might feel stiff, awkward when walking and have falls for reasons you can’t explain. The second most common symptom is difficulty looking downward. Then, you might have a hard time finding words, freezing in the middle of walking and have forgetfulness, personality changes, increased irritability and loss of interest. Another very common initial symptom is a “stare” look on your face. Other symptoms that occur at first, but are less common, include slurred speech, difficulty driving and shaky hands.

Is progressive supranuclear palsy (PSP) permanent?

Unfortunately, because there is no cure, you will have PSP for the rest of your life.

Is progressive supranuclear palsy (PSP) inherited?

No, PSP rarely runs in families. If you have PSP, there is very little risk of your other family members having it, and that includes your siblings and your children. Family history of PSP has an almost zero effect on an individual’s lifetime PSP risk, meaning people have about the same risk of developing PSP in their lifetime regardless if they do or do not have someone in their family who had or has PSP.

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