What Is Multiple System Atrophy?

Overview

Multiple System Atrophy (MSA) is a rare illness that affects the autonomic nervous system, causing progressive deterioration of the brain's nerve cells.1 The autonomic (unconscious) nervous system regulates involuntary bodily functions, such as blood pressure, heart rate, and respiration.⁵ 

The primary areas that are affected by MSA are the brain stem (the connection between the cerebrum, spinal cord, and cerebellum),⁶ the cerebellum (involved in motor movement regulation and balance control)⁷ and the basal ganglia (it takes part in motor control, motor learning, executive functions, behaviours, and emotions).⁸

Cell damage from  MSA can result in problems related to balance, movement, and other autonomic body functions.² Multiple System Atrophy symptoms are often mistaken for symptoms of Parkinson's disease. Unlike Parkinson's, MSA progresses faster and responds less effectively to medication.²    

Types of multiple system atrophy

There are two main subtypes of MSA:

• Cerebellar type MSA (MSA-C). This type of affects a patient’s ability to coordinate movements, resulting in ataxia (a group of disorders that affect balance, coordination and speech).⁴ Patients with MSA-C may also experience autonomic dysfunction and falls.⁴    
• Parkinsonian type MSA (MSA-P). The term Parkinsonian refers to parkinsonism, characterised by movement abnormalities, including bradykinesia (slowness of movement), tremors, muscle rigidity, inability to hold posture, and automatic nervous system dysfunction.³ 

Causes of multiple system atrophy

The exact cause of  Multiple System Atrophy is unknown. The majority of the cases can occur randomly. Notably, MSA is distinguished by the buildup of alpha-synuclein protein in the glia (supporting cells for the nerve cells in the brain).² This distinguishes  MSA from Parkinson's, where the accumulation of protein cells occurs directly in the nerve cells.² 

Although MSA is not considered a hereditary disease, there are reports suggesting that certain genetic variants may influence the development of MSA. These genetic variants include inflammation and oxidative stress.2 Some theories also propose that environmental factors, in combination with genetic factors, are the potential causes for the development and progression of MSA; however, further studies are needed to obtain evidence.² 

Symptoms of MSA 

The symptoms of MSA vary depending on which part of the brain is affected by the disease. The most common symptoms are:^1,2 

• Muscle rigidity or stiffness
• Bradykinesia 
• Ataxia
• Loss of bladder control 
• Speech difficulties 
• Sexual dysfunction, including erectile dysfunction and decreased libido 
• Cold feet and hands 
• Low blood pressure, provoking fainting, blurred vision and dizziness 
• Pain in the neck or shoulders
• Sleeping problems
•  Respiratory difficulties, including difficulty breathing and swallowing
• Cognitive and emotional changes
• Anxiety, depression and panic attacks 

Diagnosing multiple system atrophy 

The diagnosis of MSA can be a little complicated, especially during the early stages, due to the similar features to Parkinson’s disease. Many tests should be performed to diagnose the disease. There are:^1,2

• Individual’s medical and family history 
• Neorigical examination
• Autonomic testing (which includes heart rate control and blood pressure)
• Examination of bladder function 
• Brain scans, including
  • Magnetic resonance imaging (MRI), which  might identify changes in the brain that suggest MSA or another disease
  • Positron emission tomography (PET) is used to oversee the metabolic function in specific parts of the brain 
  • Dopamine transporter (DAT), which evaluates the dopamine distribution and activity in the brain 

Some doctors might treat a patient with medication for Parkinson’s disease to determine if it is Parkinson's or not.² If the patient does not respond to the treatment, then it proceeds to become a potential case of MSA.²  

Management and treatment 

Currently, there is no known treatment or cure for MSA and no way to delay the degeneration of the nerve cells.¹ However, there are management and treatments that help to deal with the symptoms of the disease. The treatment will also be according to the gravity of the symptoms:

• Usually, some specialists will prescribe medication to help improve motor function, including regulating blood pressure and improving bladder control.¹   
• Use compression stockings or abdominal binders to help control the blood pressure. Also, add extra salt to the diet and increase water intake.¹ 
• Some doctors may recommend botox injections to mitigate abnormal muscle postures.¹
• Problems with swallowing can be addressed with speech therapy.² 
• The inclusion of physical therapy is crucial to treat possible contractures and reduce muscle spasms and abnormal posture.² 

If the symptoms of the disease are severe, the patient may need:²

• A tracheostomy to help preserve breathing ability.
• Tube feeding
• Colostomy 
• Urostomy 
• The use of walkers and wheelchairs will be necessary if there is a significant decline in a person’s mobility. 

Risk factors & complications

The complications and risk factors that take place in MSA are  due to the progression of the disease:^1,2 

• As mentioned before, while the disease progresses, the patient loses control over the mobility of the body, and ataxia, bradykinesia, and muscle rigidity start to appear. Some of the individuals eventually will become wheelchair-bound or bedridden. 
• Due to the loss of balance and postural stability, MSA patients are likely to experience falls and injuries, resulting in bruises, fractures and head trauma. 
• Due to swallowing problems, the likelihood of choking episodes and aspiration pneumonia increases. 
• MSA increases the tendency to develop sleeping disorders, with patients experiencing insomnia and REM sleep behaviour disorder.
• Some patients may develop dysarthria, a  difficulty in speaking due to the weakening of the muscles used to speak.
• The progression of MSA can be traumatic for patients and their family members, causing feelings of frustration, anxiety, depression and isolation.
• MSA can result in severe urinary and bowel problems, including incontinence, difficulty emptying the bladder, and constipation. 

FAQs

How can I prevent MSA?

Currently, there are no sufficient studies showing that any medication or treatment can prevent MSA from developing.² 

How common is MSA

According to a study made in the United States, around 15,000 to 50,000 people are affected by the disease in that country.¹ 

What can I expect if I have MSA?

Usually, patients with MSA initially experience movement-related issues, which then progress to other symptoms. With time, patients might use a cane, walker or wheelchair to aid their mobility.² 

When should I see a doctor?

You should seek the help of medical experts when you start experiencing movement-related issues, sexual dysfunction, sleep disturbance, dizziness and passing out.¹ Unfortunately, MSA is a deadly disease, and the average survival time is 6 to 10 years;  in some cases, patients with less severe symptoms may survive up to 15 years.² 

Summary

Multiple System Atrophy (MSA) is a rare illness affecting the autonomic nervous system, causing progressive deterioration of the brain's nerve cells. Multiple System Atrophy symptoms are often mistaken for  Parkinson's disease, but MSA progresses faster and does not respond as well to medication. There are two subtypes of MSA: Cerebellar type MSA  and Parkinsonian type MSA. The exact cause of  Multiple System Atrophy is unknown. The majority of the cases can occur randomly. A distinguishing feature of  MSA is the buildup of alpha-synuclein protein in the glia. Unlike Parkinson’s,  in MSA, the accumulation of protein cells is directly allocated to the nerve cells. The symptoms of MSA vary depending on which part of the brain is affected by the disease. Currently, there is no known treatment or cure for MSA, nor to delay the degeneration of the nerve cells. However, there are management and treatments that help to deal with the symptoms of the disease. The treatment will also be according to the gravity of the symptoms. Seeking the help of medical experts early is crucial when symptoms like movement-related issues, sexual dysfunction, sleep disturbance, dizziness, and passing out arise.