What is Progressive Supranuclear Palsy? [PSP meaning]

After reading what your doctor wrote, you may have wondered: What is PSP?

PSP means “Progressive Supranuclear Palsy”. It is a Parkinson’s Plus Syndrome.

Some diseases are like Parkinson’s disease. But they also have extra symptoms. So, they are called “Parkinson’s Plus Syndromes”.

PSP is the most common Parkinson’s Plus Syndrome. If you have PSP, the extra symptoms will be: (1) Falling (2) Trouble moving eyes.

In the early stages, PSP may be misdiagnosed to be Parkinson’s disease. An MRI may help in the accurate diagnosis of PSP.

Parkinson’s Plus Syndromes

You can read more about Parkinson’s plus syndromes here.

Do read about misdiagnosis here. It will help you understand this article.

Symptoms of PSP

Progressive Supranuclear Palsy (PSP) has the 4 cardinal symptoms of Parkinsonism.

4 movement problems = “Parkinson-ism”
1. Slowness (Bradykinesia, Hypokinesia or Akinesia)

2. Uncontrolled shaking (tremor)

3. Stiffness (rigidity)

4. Unsteadiness & shuffling gait.

All features are not always present. In particular, PSP patients often do not have a tremor.

Parkinson’s Plus syndromes have extra symptoms. So, let us look at the “Plus” symptoms of PSP

PLUS symptoms seen in PSP (Extra)

1) Falling:

Falling can happen both in Parkinson’s disease and PSP.

But, patients with PSP start falling early.

Usually, PSP patients will fall within one year. Also, patients with PSP will fall backwards.

Falling, mostly backwards, is seen in PSP.

If you have PSP, you may walk with your feet wide apart to prevent falls.

Falls are a big problem. They can cause injury. So, it would help if you took all measures to prevent them. Click here to learn how.

2) Trouble moving eyes:

Usually, the doctor notices this problem.

If you have Progressive Supranuclear Palsy (PSP), you will have difficulty moving your eyes. It becomes difficult to look up and look down. Partly because of this problem, you may sit with their head pushed backwards. (hyperextension of the neck).

Look at the video below, posted by Dr. Dusan Pavlovic on youtube.

This patient has trouble looking upwards. He also has some difficulty looking down. When present, difficulty looking downwards is very suggestive of PSP.

 

Here is another video, by Dr. Kathleen Digre at the University of Utah. It is good to know what doctors look for.

But, this video is very technical. It’s okay if you do not understand it completely.

 

 

Other Plus symptoms of PSP

Falling & trouble moving eyes are the main plus signs.

But patients with PSP also have the other additional problems:

1. Speech:

They may need to strain to talk. It may seem like they have a strangled voice. They may stutter or repeat words.

2. Swallowing:

It may be difficult to push food into the stomach.

Food may go into the lungs instead (aspiration). This may cause explosive coughing.

If food goes into the lungs, it causes a nasty pneumonia. This is called “Aspiration”.

Aspiration is a serious issue. It needs urgent attention. Read more about prevention of aspiration here.

3. Thinking:

PSP patients have difficulty controlling their emotions. They may laugh or cry very easily (pseudo-bulbar affect).

PSP patients may become impulsive. They may get up quickly, even though this makes them fall. Doctors call this the “rocket sign.”

What causes Progressive Supranuclear Palsy (PSP)?

Progressive Supranuclear Palsy (PSP) is caused by an accumulation of a waste product called “Tau” in the brain. Remember: Parkinson’s disease is caused by the collection of waste produced called “synuclein.”

Accumulation of a waste product called “tau” in the brain may cause PSP.

Accumulation of tau causes a decrease in dopamine activity in the brain.

The exact reason why “Tau” accumulates in the brain is not known.

How is PSP diagnosed?

PSP is diagnosed clinically. It is diagnosed by the doctor talking to you and examining you.

The most important things are:

– History of frequently falling backwards, especially in the first year.

– Difficulty looking downwards when the doctor examines you.

A detailed conversation with and examination by your doctor is needed to diagnose PSP.

What are the MRI findings of PSP?

These findings are not seen in all patients. In particular, they may not be seen in the early stages of Progressive Supranuclear Palsy (PSP).

In PSP, a particular part of the brain called the”Midbrain” shrinks in size.

When this happens, the following two signs may be seen on the MRI:

  1. Mickey-Mouse sign: When seen from the top, the midbrain may appear like Mickey-Mouse.
  2. Humming-bird sign: When seen from the side, the midbrain may appear like a hummingbird.

Click here to see images: [Menoufia University Journal Article]

Sometimes, these findings are not seen on the first MRI. In those cases, a repeat MRI after 1-2 years may show these changes.

Can an F-DOPA scan or Trodat Scan diagnose PSP?

Yes.

F-DOPA or Trodat scans detect a decrease in dopamine activity in the brain. You can read more about them here.

This is a DAT scan. The scan on the right shows decreased dopamine activity.

Dopamine activity is reduced in Progressive Supranuclear Palsy (PSP), like in Parkinson’s disease. So, these scans are abnormal.

The scan findings in PSP are slightly different than Parkinson’s disease. But these differences are slight.

So, it isn’t easy to distinguish PSP from regular Parkinson’s disease based only on DAT / FDOPA studies.

What is the treatment of PSP?

You can read more about the treatment of PSP in another article. [Click here]

 


Caution:
This information is for educational purposes. It is not a substitute for professional medical diagnosis & treatment. Do not change your medications, supplements or other treatments without your doctor's permission.

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Dr. Siddharth Kharkar

Dr. Siddharth Kharkar is a board certified (American Board of Psychiatry & Neurology certified) Neurologist. He is a Epilepsy specialist & Parkinson's specialist in Mumbai, Maharashtra, India.

He has trained in the best institutions in India, US and UK including KEM hospital in Mumbai, Johns Hopkins University in Baltimore, University of California at San Francisco (UCSF), USA & Kings College in London.

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