What are the”Parkinson’s Plus” Syndromes?

It is best to think of the “Parkinson’s Plus” syndromes as the sisters of Parkinson’s disease.

Although many diseases are similar to Parkinson’s disease, the changes they produce and their symptoms are slightly different. It’s why we think of these diseases as the sisters of Parkinson’s disease. Another name for them are the “Parkinson’s Plus” syndromes.

 

PSP, MSA, CBD, and other “Parkinson’s Plus” diseases are like the sisters of Parkinson’s disease.

There are three common Parkinson’s Plus syndromes.

Parkinson's Plus Syndromes
1. Progresive Supranuclear Palsy (PSP) 2. Multiple System Atrophy (MSA) 3. Cortico Basal Degeneration (CBD)

These are identified as: PSP, MSA, and CBD, and should be identified correctly before any treatment begins. However, this can be difficult. Because of how hard it is to tell PSP, MSA, and CBD apart, doctors sometimes claim the patient has a “Parkinson’s Plus” syndrome without noting the exact disease.

What Symptoms May Indicate a Parkinson’s Plus Syndrome?

If you are showing any of the following symptoms, you may have a Parkinson’s Plus syndrome. These symptoms are red flags. Meaning that if you have one, you must be careful.

 

Red flag. Your doctor will be extra careful if you have any of the following symptoms.

'Red Flags' suggesting that you may have a Parkinson's Plus Syndrome
1. Lack of response to Levdopa. 2. Frequently falling down, especially backwards. 3. Severe talking or swallowing problems. 4. Problems with eye movement. 5. Impotence. 6. Blurring of vision. 7. Blacking out when rising from a seated position. 8. Problems with thinking or memory loss. 9. Personality changes. 1o. Symptoms only in the legs while the arms and hands are completely normal. 10. Rapid progression of the disease

What is the Treatment for a Parkinson’s Plus Syndrome?

The treatment for a Parkinson’s Plus syndrome is the same as the treatment for Parkinson’s disease itself.

Many patients respond to levodopa. However, what’s challenging is that sometimes levodopa does not have a dramatic effect. It may also happen that this effect may not last for many years. Therefore, the response to treatment is unpredictable, although some patients with Parkinson’s Plus syndromes do respond positively.

Once it enters the brain, Levdoopa is converted into Dopamine.

It is very important to figure out which kind of Parkinson’s Plus syndrome you have so that the specific problems associated with that disease can be treated. For example:

  1. In PSP, the patient frequently falls backwards because the areas of the brain responsible for balance are not functioning well. In this case, balance training can help.
  2. In MSA, the patient can fall because of a drop in blood pressure when they get up. In this case they should drink enough water and try to get up slowly to prevent falls. Some medications can also prevent falls.

What is Progressive Supranuclear Palsy (PSP)?

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.

Falling, especially backwards, may indicate you have PSP.

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.

    Patients with PSP can get up suddenly like a rocket. This impulsive tendency needs to be controlled to prevent falls.

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. In addition, small things like slowness of eye movements may be observed by a neurologist.

Looking carefully at how quickly and how much your eyes move when you look up and down helps your neurologist diagnose PSP.

An MRI can help in the diagnosis. In PSP, a particular part of the brain called the “Midbrain” shrinks in size. This appearance on the MRI is called the “hummingbird sign.”

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 a 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.

    Get your cataracts removed. Wear proper glasses. Prevent falls.

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.

A DEXA scan measures bone density. If your bones are not dense enough they can break easily.

Medications for brittle bones ('Osteoporosis')
1. Calcium & Vitamin D 2. Bisphosponates (for example: Alendronate) 3. Teriperatide 4. Calcitonin 5. Zolendronic acid 6. Your doctor can choose the appropriate medication for you based on your test results, age, gender and other characteristics.

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.

Physical therapy, especially balance training, can help to prevent falls.

What is Multiple System Atrophy (MSA)?

Multiple System Atrophy is a “Parkinson’s Plus” syndrome in which a component of our nervous system called the “Autonomic Nervous System” is also affected.

The Autonomic Nervous System helps us do things that we don’t thing about consciously. Thus, it helps us in:

  • Preventing a drop in blood pressure when we stand up from a chair or bed.

    The Autonomic Nervous System helps us maintain our Blood Pressure.

  • Maintaining heart rhythm
  • Having an erection
  • Sweating
  • Passing urine and stool

The following symptoms can be seen because of this system dysfunction:

  • A sudden drop in blood pressure can cause sudden dizziness, blurred vision, or loss of consciousness if you stand up abruptly.

    In MSA, patients may feel lightheaded, have blurred visions, or experience loss of consciousness if they stand up suddenly.

  • Heart rhythm problems (these are uncommon).
  • Impotence
  • Reduced sweating
  • Loss of bladder or bowel control or severe constipation

There is a form of MSA called MSA-C: The C stands for cerebellum. The cerebellum is the part of the brain that helps with coordination while walking or doing something physical. Therefore, these patients may have difficulty coordinating their movements and may sway from side-to-side while walking.

What Causes Multiple System Atrophy (MSA)?

MSA is caused by the accumulation of the same waste material as in Parkinson’s disease (synuclein).

It is not known why the accumulation of synuclein causes Parkinson’s disease in some and Multiple System Atrophy in others.

What is the Treatment of Multiple System Atrophy (MSA)?

Like all Parkinson’s Plus syndromes, the mainstay treatment is Levodopa. As noted before, the patient’s response to levodopa is unpredictable and sometimes may not be excellent.

Just like all Parkinson’s Plus syndromes, and Parkinson’s disease itself, many of the manifestations can be effectively managed. For example:

  • Drop in blood pressure: Maintaining a good fluid balance and taking medications such as pyridostigmine, fludrocortisone, midodrine may be helpful.
  • Heart rhythm problems: These can usually be controlled with medications. In rare cases a pacemaker may be needed.

    Very rarely, a pacemaker may be needed to make sure the heart beats regularly.

  • Impotence: There are many effective medications for impotence.
  • Bladder & bowel control: A mixture of behavioral changes, physical supports (diapers), and medications are usually helpful.

What are Other Types of Parkinson’s Plus Syndromes?

There are many other Parkinson’s Plus Syndromes. Since it’s not possible to describe each of these in detail here, I will mention their names and certain key characteristics.

  1. Corticobasal Degeneration: Characterized by difficulty using autmoatic movements of an arm or hand.
  2. Dementia with Lewy Bodies:  Characterized by hallucinations & disorganized behaviour.
  3. Frontotemporal dementia (“Pick’s disease”): Characterized by personality changes, including impulsivity and dis-inhibition.