Multiple system atrophy: Insights into a rare and debilitating movement disorder

Article Reviews
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Read Full Text: Multiple system atrophy: Insights into a rare and debilitating movement disorder (This is a paid article, but we thought it was still important to cover.)
Journal: Nature Reviews Neurology
Year Published: 2017
Ranked 20th on our 2016-2021 list of the 100 Most Influential OT Related Articles
This week’s article tackles a challenging diagnosis: multiple system atrophy (MSA).

MSA is a rare and aggressive type of terminal movement disorder. These patients face many challenges, including autonomic dysfunction and progressive motor system degeneration.

In this article, you’ll find a great condition-specific assessment, as well as some recommendations for supporting patients and families as they come to terms with an MSA diagnosis.

Perhaps most importantly, you’ll be reminded of the sobering reality that there is no cure for MSA. This means occupational therapy is one of the few options available to help patients navigate increasing challenges with dignity. By better understanding this rare condition, we can walk alongside patients and families, providing support and care when they need it most.

Let’s dive in.

What was the intent of this paper?

This evidence review provides a substantial update regarding what we know (and don’t know) about multiple system atrophy (MSA).

It is an extremely useful read for anyone looking to understand this diagnosis on a deeper level than what a standard Google search would yield. There is a lot of hardcore science in this paper, so I’ve done my best to pull the most salient points relevant to our work as OTs.

What is multiple system atrophy (MSA)?

Multiple system atrophy (MSA) is an adult-onset movement disorder with an aggressive clinical course. MSA patients can expect to live about 9 years from the point of symptom emergence.

The first symptoms of MSA usually occur when patients are in their 50s. Multiple neuronal pathways degenerate over the course of the disease, which creates a complex and multifaceted clinical picture. (Hence the name multiple system atrophy.)

Autonomic dysfunction is a hallmark of this disorder, as is motor system degeneration. The types of motor degeneration are divided into two main subtypes:

  • MSA with predominant parkinsonism (MSA-P) subtype
  • MSA with predominant cerebellar ataxia (MSA-C) subtype

Symptoms of MSA

The authors state that “disability develops relentlessly.” The combination of symptoms is varied and evolves over time. But, below are some of the most common clinical characteristics.

Autonomic dysfunction

Lightheadedness and urinary incontinence are some of the first clinical signs experienced by patients. Autonomic dysfunction can present in a wide variety of ways, but two hallmarks of MSA include:

Orthostatic hypotension

  • Recurrent syncope
  • Dizziness
  • Nausea
  • General weakness
  • Tremulousness
  • Headache
  • Pain, often in the nuchal “neck region,” particularly coat hanger pain

Urogenital dysfunction

  • Erectile dysfunction
  • Reduced genital sensitivity
  • Urinary urgency and frequency
  • Incomplete bladder emptying
  • Urinary incontinence

Parkinsonism symptoms

Cerebellar ataxia symptoms

Other MSA related symptoms

Cause of MSA

Our understanding of what causes MSA is incomplete, but the evidence seems to suggest the dysfunction of a protein called alpha-synuclein.

Dysfunction of the alpha-synuclein protein also seems to be a component of Parkinson’s disease and dementia with Lewy bodies, which may explain the similarity in these three diagnoses.

Diagnosis of MSA

Diagnosis is tricky. There are no lab tests or imaging that can confirm MSA, so it’s often diagnosed based on clinical presentation.

The clinical diagnosis of probable MSA requires the following features:

  • Sporadic, progressive, adult onset (>30 years old) of symptoms
  • Autonomic failure involving either urinary incontinence or an orthostatic blood pressure decrease within three minutes of standing (by ≥30 mmHg systolic or ≥15 mmHg diastolic)
  • Either poorly levodopa-responsive parkinsonism or a cerebellar syndrome

One of the biggest challenges of diagnosing MSA is ruling out Parkinson’s disease and Lewy body dementia. There is significant overlap in symptoms, which makes accurate diagnosis challenging.

Assessment of MSA

The article mentioned one assessment tool that is specific to MSA. This assessment might be a helpful tool for you, considering the many systems impacted by MSA:

Symptomatic treatment of MSA

There are no therapies that change the relentless course of MSA. So, as a therapist, your focus is on helping patients manage their symptoms.

The article specifically mentions occupational therapy’s role in preventing falls, and the authors discuss how patients can better manage orthostatic hypotension.

** Preventing falls**

Occupational therapy is mentioned (along with PT and walking aids) as the best treatment option to prevent falls. The article cites this older, but very specific, article: Occupational therapy in multiple system atrophy: a pilot randomized controlled trial.

Managing orthostatic hypotension

It is widely accepted that non-pharmacological should be the first line of treatment for autonomic failure in MSA.

Here’s the specifics mentioned by the article:

  • Patient education regarding physical countermeasures to orthostatic hypotension
  • Large meals should be avoided
  • Patients should adhere to a high-salt diet and ensure sufficient fluid intake
  • Venous compression by use of stockings and binders may be helpful
  • Nighttime head-up tilt may alleviate hypotension in the early morning

The article does not mention this, but I did find a guide for occupational therapists treating MSA. This guide was put together by the MSA Trust, which is based out of the UK.

Connecting with others with MSA

The article ends with a description of how important it is to get all hands on deck. There is a clear need for a globally coordinated effort to research MSA. The authors did also mention specific charities that can serve as a connecting point for MSA patients.

Here’s a video from the MSA Coalition that really helped me put a face to the diagnosis:

Takeaways for OT practitioners:

(These are my own personal takeaways and were not mentioned specifically in the research.)

1. This article provides concrete ways to support those with MSA.

You might only see one or two patients with MSA across all the years you practice. So, between the infrequency of seeing these patients and the cascading number of difficulties such patients face, it might feel difficult to know where to start.

But, this article really showcases the importance of what you have to offer. Strategies for managing autonomic dysfunction should certainly be considered, as should fall prevention and recovery. And, as always, it is of the utmost importance to listen to the specific needs of each patient.

2. If you treat patients with POTS, dementia, or Parkinson’s disease, it’s crucial to be aware of MSA.

If a patient’s current diagnosis does not seem to fully match their symptoms, it’s critical that you understand similar and/or related diagnoses. You might need to talk to your team about deeper exploration of symptoms, especially as new ones emerge.

And, from a broad perspective, I think it is important to watch how our understanding of these related conditions develops. Personally, I feel hopeful that advances in the upcoming decades will provide new treatment options—not just for mainstream conditions, but also for their more rare counterparts.

Here’s the full APA citation for this article:
Krismer F, Wenning GK. Multiple system atrophy: insights into a rare and debilitating movement disorder. Nat Rev Neurol. 2017 Apr;13(4):232-243. doi: 10.1038/nrneurol.2017.26. Epub 2017 Mar 17. PMID: 28303913.

What questions/thoughts does this article raise for you?

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Thank you for such a thorough article and treatment guide out of the UK, Sarah. This will be very useful where I work and help me to discern the symptoms of some of our participants with movement disorders. We do have a couple that don’t seem to truly “fit” either PD or Lewy Body Dementia.

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Very interesting article. There are so many varieties of movement disorders which affect aging people. As OT’s, it’s important that we familiarize ourselves with treatment interventions, resources that could help our patients with even one aspect of self care so they can have some level of independence. It begins with listening as all OT’s are well qualified followed by solutions.

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Thank you for sharing this article. I currently have a new patient that presents with some of the listed symptoms. With this guidance I can be looking at some of the other listed symptoms to get some baseline measures. With the changes occurring differently for each sub type for MSA patients it is especially important for the IDT to be communicating to catch the possibility of an MSA dx.

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Thank you for this informative article. It made me think of past patients that I’ve treated and they had no diagnosis for their symptoms of syncope and weakness . I think there may be a lot more people with this then we realize . I like the link to assessments ! Thank you!

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This is my first introduction to MSA. As a recent graduate with two fieldworks in OP settings, I haven’t had any experience with MSA through my clinical rotations or through my OT graduate education. That being said, I really appreciated your point about being aware of MSA (or other diagnoses for that matter) when a client’s diagnosis doesn’t quite “fit”. While not totally related, my younger brother was diagnosed with Asperger’s (in 2004, I recognize this diagnosis no longer exists!) and ADHD as a child. My mother, a former SLP, kept advocating for new diagnosticians because she felt my brother boxed in. In the end, he was re-diagnosed with executive functioning disorder. I recognize this situation is very different than what was mentioned above, but it’s something I always remember when meeting new clients: 1) a diagnosis doesn’t define an individual; 2) a diagnosis is a label and sometimes not even the correct one!

This article also made me think of the role of OTs in hospice care. My uncle passed away about a year ago from a long (read; 12+ year, go Geary!) battle with lymphoma. When he was near end-stage and entered hospice care, I advocated for hospice OT but that setting for OT isn’t as prevalent up here. Sarah, you mentioned the importance of showcasing all the roles an OT can take, and I would really like to see more hospice OTs. I think it’s an area that OTs can make a LOT of impact and allow clients to live with dignity through their end days.

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Thank you for this article, Sarah. Honestly the timing could not have been better as I have just taken on a client in the community with MSA-P and I have a client with suspect MSA-C. As an OT with only 3 years experience, it has been a bit of a daunting task as I have been unsure of how to progress. Having read this article it is amazing that my ideas of taking a falls prevention and education approach have been validated by the research that is out there.

I found this article especially interesting as I have those two clients with MSA, or MSA symptoms, but both are at completely different stages. The guide for Occupational Therapists for MSA clients is seemingly an amazing tool for me to follow to build a solid foundation with my clients and their families to ensure they are getting the most out of their days as possible.

For any other Australian OT’s, I have found an MSA group based here that may provide useful to yourself, the client, or their family: https://www.msadownunder.org.au/

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This article evokes a lot of emotions in me. I have lost 2 friends in the recent past-one to MSA and the other to complications from progressive supranuclear palsy.
I am glad to see that both these diagnoses are covered in the fact sheet presented in this article which will help many OTs find important info to guide their interventions.
I lost my dear friend Naseem (name changed to protect privacy) to MSA a few years ago. I met Naseem when she had early symptoms of MSA and was admitted to our inpatient rehab hospital. She was an MD (internal medicine) and her husband who I work with is a neurologist (look at the irony of this situation). She had the MSA-P subtype with Parkinsonian symptoms-stridor, antecollis, rigidity, weakness. Leva-dopa was tried but she did not respond to it. I have seen Naseem go from an energetic, fun-loving, classy lady to one whose neck had to be tied with a bandana to her w/c to prevent her head from falling forward. I was not aware of this diagnosis at that time and had to research it to know what was going on. Naseem’s MSA progressed rapidly and we lost her about 3 years ago due to respiratory complications.
I had assisted her husband to find adaptations for communication and mobility which helped Naseem maintain some control of her life. Her symptoms were so confusing, they looked so much like Parkinson’s but had other variations. Towards the end, her stridor became so bad that I could hardly understand her on the phone.
Our other friend with progressive supranuclear palsy started off with the classic symptoms of problems with walking, balance and eye movements, and within a few years succumbed to swallowing complications. Working in Neuro, I was so perplexed with these little-known diagnoses that were life-threatening and with no cure. This article rightly points out that since there is such an overlap in symptoms, accurate diagnosis is challenging.
The article and its accompanying resources provide a nice basis for OTs on what to look for and how to approach these debilitating conditions.
" If you treat patients with POTS, dementia, or Parkinson’s disease, it’s crucial to be aware of MSA." Thank you for this important takeaway. I am hopeful too, that advances in new treatment options will prevent people like my young friends from leaving us prematurely.

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Wow, @Geordie I always feel like you are doing such important work as a new practitioner. I’m glad these resources arrived at the right time for you.

Thank you so much for sharing the Australian based group! I actually added it to the article so it will be easy for others to find in the future.

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This is such an underserved diagnosis… Thank you for bringing awareness to this condition, Sarah. I’m glad you mentioned POTS, because it truly has so many similarities in management given the degree of ANS involvement. Combining our understanding of treating the whole person, our knowledge of movement disorders, and our skillset in activity analysis and adaptation, we truly are the perfect discipline IMHO to serve these folks and improve quality of life for the whole family.
PWR! training is now offered online and could be a helpful resource for anyone looking to learn more/ take CEUs. Additionally, there will be a POTS lecture at the upcoming AOTA conference by myself and another OT that provides some details about managing the autonomic nervous system. We have such an awesome opportunity to fill this need and make a difference for these folks in the midst of a truly devastating diagnosis.

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Hey @emily12! I’m so glad to see you on here! I was thinking of your work as I was working on this article! Have you had patients with MSA referred to you because of your specialization in the ANS? I’m curious as to whether the majority of patients with MSA are getting the ANS support this article recommends.

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Oh, @sanchala, I’m so sorry for the loss of your friends. Hearing stories of this diagnosis is really important to understanding how complex and straight up hard it is.

I hope to see OTs getting involved in the charities mentioned above, as as rare disease research efforts are often underfunded.

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Sarah,
You are so right about this. When I had asked my friend’s husband, the neurologist, about why there was not much info/research on MSA, he had said that since it is not common like PD, it is considered rare and is underfunded. What a shame!

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I have learned so much more about POTS through your responses/podcast on the topic - so much more than ever learned in OT school.

What is the PWR! Training? I’ll be attending the Aspire conference and am looking forward to your lecture on POTS to continue learning more about this diagnosis.

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I am just now seeing this, Allison, so I apologize for the delay. PWR! Is Parkinson’s Wellness Recovery and it’s a beautiful model to use for exercise and approaching treatment for movement disorders. Becky Farley (PT) founded it and previously was a part of creating LSVT program (I believe?) so it’s similar in a lot of ways with BIG movements but it’s not the rigid structure that LSVT requires so you can incorporate it in any treatment setting. It’s also much more functional in my opinion.
Sarah - yes, these folks are absolutely underserved and the ANS piece may be missed if providers don’t have the knowledge on that part, so I could assume yes but don’t know enough stories to be sure. I have one individual in my practice who has MSA, previously thought to be POTS. So much more to learn and to research.

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