PSP is the short-form of a disease called Progressive Supranuclear Palsy.
Diseases that produce signs and symptoms that are similar to Parkinson’s disease, but have additional features are called “Parkinson’s Plus Syndromes”.
PSP is the commonest “Parkinson’s Plus” syndrome.
So, Progressive Supranuclear Palsy is a “Parkinson’s Plus” syndrome in which the following characteristics are seen:
- Frequent falling, especially backwards.
- Problems moving eyes, especially to look downward.
Other subtle features may also be seen:
- They may have trouble opening their eyes.
- Problems with speaking or swallowing.
- They may make involuntary sounds such as groaning or humming.
- Problems with impulse control, such as getting up suddenly from a chair. This is also called the “rocket sign,” reflecting the rapidity with which some patients get up from their chairs.
What Causes Progressive Supranuclear Palsy (PSP)?
PSP is caused by an accumulation of a waste product called “Tau” in the brain. Remember: Parkinson’s disease is caused by accumulation of a waste produced called “syncucelin.”
The exact reason why “Tau” accumulates in the brain is not known.
How is Progressive Supranuclear Palsy (PSP) Diagnosed?
Progressive Supranuclear Palsy is diagnosed based on the presence of the typical symptoms described above. Also, small things like the slowness of eye movements may be observed by a neurologist.
An MRI can help in the diagnosis. In PSP, a particular part of the brain called the “Midbrain” (shown below) shrinks in size. This shrinkage can be seen on the MRI. The appearance of this shrinkage on MRI is called the “hummingbird sign” because a shrunken midbrain looks like a hummingbird when viewed from the side. It may also be referred to as the “Mickey Mouse sign” because that is what the midbrain looks like on the MRI when viewed from the top.
If an F-DOPA scan (Click here to know more about an F-DOPA scan) is done for patients with PSP, it also shows a decrease in dopamine activity in the brain. Although some features such as asymmetry may help in distinguishing between Parkinson’s disease and PSP, usually an F-DOPA scan is not very useful for this purpose.
What is the Treatment of Progressive Supranuclear Palsy?
The main treatment of PSP is the same as Parkinson’s disease. The most effective medication to improve movement is Levodopa. The Dopamine agonists may help with movement.
As noted before, one of the challenging things about PSP, and all Parkinson’s Plus syndromes, is that the response to Levodopa can be incomplete and may decrease with time. The response is unpredictable. Some patients show a more marked and sustained response than expected.
A key feature of PSP is falling. Avoidance of injury due to falls is an important and often overlooked part of PSP treatment. This problem should be addressed in 3 ways:
1. Treat other causes of falls:
- Testing for problems in the nerves & spinal cord. A whole spine MRI and the following blood tests may be reasonable – B12, folic acid, TSH, VDRL, HIV, vitamin E levels, and copper levels.
- Testing for Vitamin D level.
- Properly functioning eyes and ears help to prevent falls. Make sure you are wearing the correct glasses and if you have problems like cataracts, get them treated. Treat any ear problems. See an Opthalmologist & ENT doctor regularly.
2. Make sure your bones are strong:
Falling can lead to broken bones, as bones become brittle with age. I recommend measuring the bone density with a DEXA scan in all patients who are at risk of falling, including patients with PSP. If the bones show to be brittle, there are effective medications to make them strong again.
3. Physical Therapy:
Physical Therapy, especially balance training, is important to decrease the risk of falling. Make sure you mention to your physiotherapist that you want to focus on your balance even more than increasing your muscle strength. Controlling impulsiveness, especially when getting up, may be achieved through sustained counseling by physiotherapists. Some patients may require professional counseling.
Dr. Siddharth Kharkar
Dr. Kharkar is a Neurologist, Epilepsy specialist & Parkinson’s disease 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.