Atypical Parkinsonisms: What is Multiple System Atrophy (MSA)?

A man is in front of a cement set of stairs. There are many stairs and a banister. They are outside. The man is in a motorized wheelchair. He has a mostly shaved head with a small amount of black hair at the top. He is wearing a black winter coat.

There are multiple conditions that are similar to Parkinson's. These conditions are sometimes referred to as parkinsonisms, atypical parkinsonisms, or parkinson-plus syndromes. Multiple System Atrophy (MSA) is one example.

the symptoms of MSA

The symptoms of MSA and Parkinson's are similar, so distinguishing between the two conditions can be challenging. For example, people living with MSA often have prominent rigidity and bradykinesia, but they infrequently experience a resting tremor. However, absence of rest tremor is not a certain indication of MSA, since as many as 31% of people living with Parkinson's do not experience rest tremor at the time of their diagnosis.

People living with MSA tend to have earlier onset of autonomic symptoms than people living with Parkinson's. Examples of autonomic symptoms include orthostatic hypotension, incontinence or other urinary issues, unusual sweating, sexual dysfunction, and issues with vision.

Other signs that suggest a diagnosis of MSA include cerebellar issues, which can cause gait dysfunction or abnormal eye movements; earlier falls than would be typical for someone with Parkinson's; difficulty speaking; and myoclonus: sudden, brief jerking movements, which especially affects the fingers.

Like Parkinson's, MSA has subtypes. MSA is conventionally thought to have just two: MSA-P and MSA-C. MSA-P is characterized by prominent bradykinesia, postural instability, rigidity, and postural and action tremors. MSA-C is characterized by more significant inability to control body movements, difficulty speaking, and eye movement disruption. Early autonomic symptoms are prominent in both subtypes of MSA, and cognition is typically unaffected by both subtypes.

How MSA differs from Parkinson's

Both Parkinson's and MSA feature pathophysiology related to alpha-synuclein, but there are differences in which cells and parts of the brain are most affected.

MSA usually progresses more quickly than Parkinson's. Significant autonomic dysfunction is common within three years of diagnosis, and most people living with MSA require use of a wheelchair within five years of diagnosis.

On average, people live six to ten years after diagnosis with MSA. According to a 2017 study published in JAMA Neurology, people living with MSA-P experience a six-year decrease in lifespan. The same study found a one year decrease in lifespan for people living with Parkinson's.

MSA is much rarer than Parkinson's. A 2019 study found that in people over 40 years old, the rate of MSA diagnoses is 7.8 per 100,000 people. In contrast, a 2022 estimate found that among people over 45 years old there are between 47 and 77 Parkinson's diagnoses per 100,000 people.

How MSA is Diagnosed

Because of the similarities between MSA, Parkinson's, and other parkinsonisms, diagnosing MSA is often difficult. An autopsy is the only way to confirm an MSA diagnosis. A 2015 study of 134 people clinically diagnosed with MSA, only 62% were found by an autopsy to have an accurate diagnosis.

There are ways to improve confidence in an MSA diagnosis. Critically, recent developments in alpha-synuclein testing have promise for improving accuracy of diagnosis, and a study from April of this year confirms that alpha-synuclein associated with MSA can be distinguished from alpha-synuclein associated with Parkinson's. At present, however, the standard practice is that MSA is diagnosed by clinical examination.

In 2022, the Movement Disorder Society released a description of criteria for the diagnosis of MSA. The document describes four levels of diagnostic certainty:

1.) Neuropathologically Established MSA

This is the most certain diagnostic level, and it requires an autopsy.

2.) Clinically Established MSA

This is the second most certain diagnostic level. It has four sets of criteria:

At least one of the following autonomic issues:

  • Unexplained difficulty emptying your bladder
  • Unexplained urinary urge
  • Neurogenic orthostatic hypotension

At least one MRI finding. Note that some of the relevant findings in an MRI are subtle and may be missed by those unfamiliar with the relevant signs.

At least one of the following:

Parkinsonian Symptoms That Respond Poorly to Levodopa Cerebellar Symptoms
Bradykinesia Poor motor control of gait
Rigidity Poor upper limb motor control
Tremor Difficulties with speech
Repetitive, uncontrolled motion of the eyes

 

Two Supportive Motor or Non-Motor Features:

Motor Features Non-motor Features
Moderate to severe postural instability within three years Stridor
Severe speech impairment within three years Inspiratory sighing
Head or neck dystonia induced or worsened by levodopa Cold, discolored hands and feet
Swallowing difficulties Pathologic laughter or crying
Jerky postural or kinetic tremors
Postural deformity
Abnormal Babinski reflex

3.) Clinically Probable MSA

Clinically Probable MSA has similar requirements to those for Clinically Established MSA.

At least two of the following:

  • One type of autonomic dysfunction from the list above
  • One type of cerebellar disturbance from the table above
  • Parkinsonism

At least one motor or non-motor features described in the chart above.

An abnormal MRI finding is not required for a Clinically Probable MSA diagnosis.

4.) Possible Prodromal MSA

Possible prodromal MSA is a diagnostic category devised by the MDS to help identify those people who may develop MSA but whose symptoms do not yet clearly establish the diagnosis. Criteria for possible prodromal MSA include autonomic symptoms or RBD sleep disorder and having subtle parkinsonian or cerebellar signs of possible MSA.

There are several important symptoms that would exclude someone from being diagnosed with possible prodromal MSA:

  • Unexplained loss of sense of smell
  • Abnormal cardiac testing
  • Dementia within three years of symptom onset
  • Visual hallucinations

Treatments for MSA

Today, there is no treatment proven to slow or stop the progression of MSA. There are, however, treatments that can help manage the symptoms.

For example, although most people's MSA motor symptoms respond poorly to levodopa, studies have found that up to 65% of people with MSA get some positive effect from taking levodopa. MAO-B inhibitors, dopamine agonists, and amantadine may also be helpful.

Other treatments commonly used by people with Parkinson's can be helpful, too. These include botulinum toxin for dystonia and melatonin or clonazepam for sleep issues. Sleep apnea is common in MSA, and can be managed with a continuous positive airway pressure (CPAP) machine, although as MSA progresses, many people find the device intolerable to wear.

Seeing a speech-language pathologist can also help you learn techniques to address difficulties with your speech, breathing, and swallowing.

Physical therapy is also important because higher levels of activity can help work against some of the heart and lung issues associated with increased mortality in MSA. A physical therapist can help you remain active as long and as safely as possible.

Remaining active can help lessen orthostatic hypotension. It is important to manage this symptom because it can contribute to falls. In addition to exercise, increasing water and salt intake can help, as can compression stockings and elevating your head 10 degrees while laying down. There are also medications for orthostatic hypotension.

Urinary symptoms of MSA can be treated in multiple ways, and it is important to manage these symptoms--and the hygiene issues associated with them. Urinary tract infections are common in MSA and increase mortality. There are lifestyle modifications, medications, and surgical procedures that may help.

Living Well With MSA

Living with MSA is hard. It tends to progress quickly, and the common symptoms can have significant impacts on quality of life. Still, by taking action today, you can take steps to live well.

Many of the things you can do to live well with MSA involve prioritizing palliative care. Know that this does not only mean hospice care. There are neuropalliative care specialists that work to provide whole-person support, and they can help you address the specific challenges you face by helping connect you with the right supportive care providers.

Some ways you can help improve your quality of life with MSA include the following:

  • Work with an occupational therapist to develop strategies to improve the safety of your home and your ability to contribute to daily activities.
  • Receive care from an MSA expert.
  • Seek mental healthcare support.
  • Prioritize the well-being of your care partners.
  • Participate in clinical trials. If you are able to participate in a trial, you will likely have more time with expert care providers, and many people who participate in trials report an increased sense of well-being from having contributed to improving understanding and advancing new treatment strategies. Clinicaltrials.gov is the most comprehensive resource to search for trials, but the Fox Trial Finder allows you to search for MSA trials, too.

The most important thing you can do to live well with MSA is to avoid isolation and rely on your care partner, friends, and family. Social isolation is associated with worsened quality of life, so prioritize your relationships, find an MSA-specific support group through the organizations listed below, and connect with our Living with Parkinson's Meetup--people living with Parkinson's and MSA face many of the same issues, and we are here for you.

Additional Resources

MSA Association

Defeat MSA Alliance

National Organization for Rare Disorders

The Differential Diagnosis of Parkinson's

Strategies for Speech and Communication Improvement in MSA

Managing Orthostatic Hypotension

Genetic Testing for Parkinson's

A Primer on Clinical Trials

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