What are the”Parkinson’s Plus” syndromes?

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

Some diseases are very similar to Parkinson’s disease. But the changes they produce inside the brain, and the symptoms they produce are slightly different. These diseases are like the “Sisters” of Parkinson’s disease. These diseases are called 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)

Ideally, the precise disease should be identified e.g. PSP, MSA, CBD etc before treatment. But sometimes, identifying the precise disease is difficult. Therefore, sometimes doctors may just mention that the patient has a “Parkinson’s Plus” syndrome, without noting the exact disease.

What symptoms may indicate a Parkinson’s Plus syndrome?

If any of the following symptoms are present, you may have a Parkinson’s Plus syndrome. These symptoms are called “Red Flags” – which means that if these are present, one has to 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 on getting up from a seating position.
8. Problems with thinking, memory or personality change.
9. Symptoms only in the legs. Arms/hands completely normal.
10. Rapid progresion of the disease

What is the treatment of Parkinson’s Plus Syndromes?

On the whole, the treatment of Parkinson’s Plus syndromes is the same as the treatment for Parkinson’s disease itself.

Many patients respond to levodopa. However, the challenging part is that sometimes levodopa may not have a dramatic effect. It may also happen that this effect may not last for many years. The response to treatment therefore is unpredictable – some patients with Parkinson’s plus syndromes can have a good response.

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 peculiar problems associated with that disease can be treated. For example:

  1. In PSP, the patient frequently falls backwards because the brain areas 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 he/she gets up – In this case, drinking enough water, getting up suddenly and some medications can prevent falls.

What is Progressive Supranuclear Palsy (PSP)?

Progressive supranuclear palsy is a “Parkinson’s Plus” syndrome in which the following characteristic features are seen:

  • Frequent falling, especially backwards.
  • Problems moving eyes, especially to look downwards.

Falling, especially backwards, may indicate that you have PSP.

In addition, other subtle features can also be seen:

  • Sometimes they may have trouble opening their eyes.
  • Problems with speaking or swallowing
  • Occasionally 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 chair.

    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 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 such as slowness of eye movements may be observed by the neurologist.

Looking carefully at how quickly and how much your eyes are move when you look upwards and downwards helps your neurologist to 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 & 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. In addition, 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 certainly show a more marked and sustained response than expected.

In addition, a key feature of PSP is falls. Avoidance of Injury due to falls is an important, 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 of the 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:

A bigger issue than falling is if any bones break due to a fall. Bones become brittle with age. I recommend measurement of Bone Density with a DEXA scan in all patients who are at risk of falling, including patients with PSP. If your bones indeed are brittle, there are very effective medications to make them strong again.

A DEXA scan measures bone density. If your bones are not adequately dense, 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. Many others… 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 especially 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 muscular strength. Controlling impulsiveness, especially while getting up, may be achieved through sustained counselling by physiotherapists. Some patients may require professional counselling.

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 particular component of our nervous system called the “Autonomic Nervous System” is also affected.

The Autonomic Nervous System helps us in doing things that we do not thing consciously about: Thus, it helps us in:

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

    The Autonomic Nervous System helps us to maintain our Blood Pressure.

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

Therefore, the following symptoms can be seen due to the dysfunction of this system:

  • Sudden drop in blood pressure producing lightheadedness, blurred vision or loss of consciousness if you stand up suddenly.

    In MSA, patients may feel lightheaded, have blurring of vision or even lose 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 us with coordination while doing things and walking. Therefore, these patients can have difficulty in coordinating their movements and may sway from side-to-side while walking.

What causes Multiple System Atrophy (MSA)?

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

It is not known why in some patients accumulation of this material produces Parkinson’s disease, while accumulation in other patients produces Multiple System Atrophy.

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

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

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

  • Drop in Blood Pressure: Maintaining a good fluid balance, and medications such as pyridostigmine, fludrocortisone, midodrine etc may be helpful.
  • Heart rhythm problems: These can usually be controlled with medications. Rarely, a pacemaker may be needed.

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

  • Impotence: As is widely known, there are many (very effective) medications for impotence.
  • Bladder & bowel control: A mixture of behavioral changes, physical supports such as 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 is not possible to describe each one of these in detail here, I will just mention their names and certain key characteristics:

  1. Corticobasal Degeneration: Characterized by difficulty using, marked stiffening or automatic 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 change including impulsivity and disinhibition.