Part II: Trends and Relationships in the Progression of MSA
On the MSA Coalition site, there is a compelling blog posting that speaks directly to the idea that each patient presents their own unique path. Belinda Franklin (2022) wrote of her unique journey with MSA, offering that her “case of MSA is not as linear as is typical.” Her experience underscores the themes and variations of neurodegenerative diseases and reminds us to live the day fully.
In 2002, Watanabe, and his colleagues published their findings from a study of 230 Japanese patients’ progression in MSA. Their study suggested that many symptoms and factors are involved in the progression of MSA but, most importantly, the interval from the initial symptom to the combined motor and autonomic dysfunction can predict functional deterioration and survival in MSA. This sounds kind of obvious, right? But as Belinda’s story told, as well as other researchers' findings regarding the inability to predict with MSA, Watanabe’s team suggested that their research pointed toward a ‘perfect storm’ like scenario. The perfect storm is the merging of motor dysfunction with autonomic dysfunction and the pace at which this storm forms can inform the rate of functional deterioration of the patient. ,
Now I am not trying to be flippant. In fact, it didn’t even strike me until this moment as I wrote that paragraph. I met with one of my neurological expert team members last week and we did the six-month review. He remarked how some of the autonomic symptom features seem to be now as prevalent as the previously observed motor features. As we concluded, he asked if I have taken the time to ‘get my affairs in order’ and if I had considered MAID.
Regardless as to whether or not I read too much into this event, the reality is, I need to keep my focus on living into this disease, push the ever-redefining boundaries, and be fully present. Sorry for the sidebar and let’s get back on topic.
Eighteen years after the Watanabe (2002) study, a European team of researchers led by Alexandra Fouler-Samier (2020) published their findings arising from a cohort of 261 patients. Their conclusion reflects Watanabe’s finding but they were able to offer some distinctive relationships. The following chart shows these.
Event | Predicted Progress |
The shorter the interval between initial symptom onset to combined motor/autonomic dysfunction | Suggests an expected more rapid disease progression (confirms the 2002 study). |
Autonomic dysfunctions as initial featured symptoms | Implies a more rapid disease progression |
The greater the severity of orthostatic hypotension | Predicts often the rapidness of progression. |
The greater the motor reserves | Suggests a slower motor dysfunction initially but a more rapid progression when it occurs. |
The later age of symptom onset | Suggests a more rapid disease progression |
I wish to reiterate that these are research findings and not rules. The Fouler-Samier team was able to offer these inferences or generalizations, which are meant as information and not a prognosis.
Now I am going to turn this inward and utilize the question a graduate school professor kept asking me about anything we read: “So what?”
What can I take away from this? Like you, I can look down the left column and recognize some of those characteristics that apply to me and others that do not. One that does is about the motor reserves. I have always been very active and continue to be. I work hard five days a week at the gym, building and maintaining strength and working on my physiotherapy exercises. I wobble and shake and laugh at myself as I move from machine to machine. Twenty months ago I had to give up skiing and bike riding because a drunk-like guy on skis or a bike is dangerous. But last week, I picked up a recumbent trike (more about this in another blog soon) and reaffirmed Kyle Bryant’s call to action to live into these diseases.
If the research is correct then maybe one day I may hit the wall of rapid progressive motor dysfunction, but until then, I’m living into today with all I got. The answer to “So What?” my dear professor … this knowledge lets any of us turn the inevitabilities of the right column into powerful motivation to live today more fully than we may ever have imagined.
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Fanciulli, A., & Wenning, G. K. (2015). Multiple-system atrophy. The New England journal of medicine, 372(3), 249–263. https://doi.org/10.1056/NEJMra1311488 Foubert-Samier, A., Pavy-Le Traon, A., Guillet, F., Le-Goff, M., Helmer, C., Tison, F., Rascol, O., Proust-Lima, C., & Meissner, W. G. (2020). Disease progression and prognostic factors in multiple system atrophy: A prospective cohort study. Neurobiology of disease, 139, 104813. https://doi.org/10.1016/j.nbd.2020.104813
Watanabe, H., Saito, Y., Terao, S., Ando, T., Kachi, T., Mukai, E., Aiba, I., Abe, Y., Tamakoshi, A., Doyu, M., Hirayama, M., & Sobue, G. (2002). Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain: a journal of neurology, 125(Pt 5), 1070–1083. https://doi.org/10.1093/brain/awf117
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