Multiple System Atrophy (MSA)

What is MSA?

MSA is a neurological in which multiple brain areas are affected by a degenerative process resulting in shrinking or atrophy. As parts of brain are involved in different functions, sometimes they are described as multiple systems, like the cortical system (e.g., motor control), the extrapyramidal system (e.g., balance and movements), or the cerebellar system (e.g., gait and dexterity). These parts are all interconnected and influence each other. In MSA, more than one of these systems are affected resulting in a variety of symptoms. In comparison, in Parkinson disease, one of these systems may be affected.

 

What causes MSA?

It is considered a degenerative disorder, implying that brain cells die or degenerate. This may be due to collection of an abnormal form of a protein called alpha synuclein in brain cells. Same protein is involved to cause Parkinson disease but in MSA, it is accumulated in many areas of brain, especially in many of its deep parts. Exact reason for its abnormal form, or why is it accumulated is unknown.

 

What are symptoms of MSA?

Symptoms vary in different patients depending upon which brain system is more involved or involved first. Following list of symptoms and findings provides an outline of problems in MSA:

1. Extrapyramidal (or Parkinson) type: Rigidity, gait disorder, tremor, decreased movements.
2. Cerebellar type: Abnormal gait, abnormal speech, abnormal eye movements, tremor, loss of dexterity.
3. Autonomic type: Dizziness, low blood pressure, cardiac arrythmias, urinary retention or incontinence, lack of sweating, erectile dysfunction.
4. Cortical type: Dementia, usually of frontotemporal type, aphasia.
5. Other: REM sleep behavior disorder, central sleep apnea, laryngeal stridor (high pitched sound from larynx due to blockage caused by vocal cord spasm), axial dystonia (spasm of trunk muscles)

 

How is MSA diagnosed?

It is diagnosed based upon its clinical features or patient’s symptoms and signs on examination. Imaging may not be helpful in early stages. There are detailed clinical diagnostic criterion developed by professional societies. As far as labs are concerned, skin biopsy to check for alpha-synuclein, and CSF analysis to check for alpha-synuclein and neurofilament light chains are helpful tests.   

 

Are there different types of MSA?

As described above, neurologists may divide MSA in different types based upon the system involved, or what is affected first.

 

How quickly MSA progresses?

In comparison to Parkinson disease or Alzheimer dementia, it is a relatively quickly progressive condition. Many patients are severely disabled or using a wheelchair in 5 years, and bedbound by 8 years. Average survival is 7-9 years; some variant cases may survive many more years, while a rare form may be fatal in less than 3 years. Falling may be an indicator of shorter survival.

 

Is MSA a terminal illness?

Patients usually do not die directly from MSA, they succumb to one of its complications. Complications of MSA include falls and related injuries, aspiration, pressure sores, cardiac or lung failure, other infections such as UTI, or choking.

 

What conditions may look like MSA?

All conditions with Parkinson-like symptoms, spinocerebellar ataxia, paraneoplastic syndrome, multiple strokes, vasculitis, Fragile X tremor-ataxia syndrome, multiple sclerosis, etc.

 

How is MSA treated?

There is no specific medicine or measure available to slow down, stop, or reverse the abnormal process of MSA. Medicines and many measures are used based upon symptoms.

1. Parkinson symptoms: With medicines used for Parkinson disease (Please read the section on Parkinson disease for more detail)
2. Low blood pressure: With meds and other measures (please read the section on orthostatic hypotension for more detail)
3. Urinary symptoms: Fluid management, meds, Botox, catheterization
4. Erectile dysfunction: Meds may be helpful but may also cause low blood pressure.
5. Sleep disorders: Depends upon the nature of sleep disorder.
6. Behavioral: Mood stabilizers, SSRI (small dose), antipsychotics (small dose)
7. Stridor: Urgent ENT evaluation, CPAP, tracheostomy.
8. Drooling: Meds, Botox.
9. Dystonia: Botox
10. Gait disorder and falls: Walker, wheelchair

Following measures may help all patients:

1. Learn about this condition. This article is written for that purpose.
2. Make appropriate changes in living situation and lifestyle to minimize falls and injuries. This may include having one floor living space.
3. Avoid stairs.
4. Remove loose rugs.
5. Keep nightlight on.
6. Consider a walker or a wheelchair for safety.
7. Install handicap type features in the house and especially in the bathroom.
8. Take sitting showers.
9. Have a will, health proxy and financial power of attorney papers done.
10. Consider early retirement.
11. Minimize driving. Don’t drive at night, during rush hours, in bad weather, and long-distance.
12. Try speech therapy to improve and maintain speech.
13. While drinking, do it slowly with smaller sips, in erect sitting or standing position.
14. While eating, do it slowly with smaller bites, in erect sitting or standing position. Avoid food that may seem difficult to chew or swallow. A different formulation of the same food item may help to avoid choking. For example, if eating meat, avoid steak or bigger pieces, try minced meat or burger.
15. Avoid large meals and take smaller more frequent meals.
16. Avoid alcohol and drug(s) of abuse.
17. If speech is affected, consider a microphone and a speaker for better communication. In more severe cases, consider electronic keyboard for communication.
18. If living at home, consider getting couple of hours of help during most days to help with cleaning and meals.
19. For patients with excessive blinking causing eye closure, consider Botox injections.
20. Physical therapy can help to improve strength and balance.
21. Occupational therapy may help to make appropriate changes in day to day living, especially to avoid falls and injuries.
22. If available, consider joining a support group. If not, at least contact Elder Care services for any help.
23. When coming for appointments, either bring the list of all medicines you take, or the prescription bottles. Also, write down the questions you may have for the doctor.

 

Who do I need to see if I have MSA type symptoms:

Other than your PCP, consult a neurologist. MSA is a neurological disorder under the category of Movement Disorders. Some neurologists specialize in this type of diseases, and some patients may benefit from consulting a movement disorder specialist. You may need multiple type of expertise to manage different aspects of this disease.

 

Is MSA a genetic disorder?

Probably yes, but its genetic basis has not been established. There is no evidence that it is a familial condition.

 

How is MSA distinguished from Parkinson disease?

In early stages it may be difficult to differentiate between the two. Following tests may help:

A: Autonomic testing, like the Tilt-table test may help to confirm autonomic issues that favor MSA.

B: Urodynamic study: Detrusor-sphincter dyssynergia in MSA.

C: MRI brain: Not in early stages but in later stages there are distinct MRI findings that may help to differentiate between the two, and the type of MSA.

 

Where can I get more information about MSA?

American Academy of Neurology

International Parkinson and Movement Disorder Society

Mission MSA