In this podcast, Dr Coyle reviews the diagnosis and clinical course of progressive multiple sclerosis (MS), pearls for distinguishing it from its differential diagnosis, and the importance of multidisciplinary care in the long-term management of progressive MS. She recently discussed this topic at the 2020 Consortium of Multiple Sclerosis Centers (CMSC) Virtual Meeting.
Additional Resources:
Coyle PK. Progressive MS: diagnosis, clinical course and long-term management. Presented at: CMSC Virtual Meeting; May 27, 2020.
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Patricia K. Coyle, MD, is professor and interim chair in the Department of Neurology at Stony Brook University in New York, and director of the Stony Brook MS Comprehensive Care Center.
TRANSCRIPT:
Dr Coyle: My name is Patricia K. Coyle. I'm professor and interim chair in the Department of Neurology at Stony Brook University in New York, and I direct the Stony Brook MS Comprehensive Care Center. My topic today is on progressive MS diagnosis, clinical course, and long-term management, and we have a couple of questions that I was given to cover.
The first involves, what are some pearls for diagnosing progressive MS and distinguishing it from its differential diagnosis?
There are 2 forms of progressive MS, 2 distinct clinical phenotypes. When we say progressive MS, this represents the neurodegenerative phase of MS injury to synapses, axons, neurons. The clinical expression is gradual, slow neurological worsening. You can have a progressive form of MS from onset that's primary progressive–that's typically the progressive form that we would be diagnosing. Quite unusual, only about 10 to 15%. There’s a second progressive phenotype, secondary progressive MS. Every secondary progressive MS is relapsing, but a relapsing MS patient is at risk, particularly in midlife, to transition to gradual, worsening disease. Those individuals, you're not typically diagnosing. They're already diagnosed with relapsing MS, and then it's just a determination that their phenotype has switched.
So, I think really the issue is having a suspicion. If somebody in midlife, 40s, 50s, men affected equally to women in this unusual primary progressive phenotype of MS, is describing slow deterioration and their ability to walk and slow development of weakness of their legs to recognize that that could be primary progressive MS, and then to do a thorough workup following the 2017 McDonald revised diagnostic criteria. To meet the diagnostic criteria for primary progressive MS, you need to have at least a year of worsening, either retrospective or prospective, and to have 3 other features: a lesion in the brain that's 3 millimeters or larger that looks like MS in specified areas that are common to MS, or 2 MRI lesions in the spinal cord, or abnormal spinal fluid showing positive CSF-specific or oligoclonal bands.
So, what this indicates is that you should do a rigorous workup. If you're working somebody up for primary progressive MS, you should do selected bloodwork, image their entire CNS, brain, cervical, thoracic cord, including the conus at the very bottom. That's all central nervous system, and we would routinely do a spinal tap, looking in particular for all the good oligoclonal bands. So, clinical suspicion, followed by a thorough workup.
I was asked to briefly give an overview of the clinical course of progressive MS. Progressive MS is more severe. It means inevitable disability. We don't have any way to prevent that at the current time. That having been said, the pace of worsening is different, and it can be very slow, or it can be moderate, or can be more significant. The good news is that patients don't tend to change. So, you can get a sense early in the course with regard to how quickly the person is worsening. In addition, it's not necessarily inevitably downhill continually, since patients can seem to clinically stabilize for extended periods of time. This may represent the degree of brain reserve that they have, and there were even descriptions of modest improvement that can last for a period of time. But in general, it's gradually downhill, although the rate of slow progression doesn't tend to change over time.
Now, another question that I was asked was, what is the role of multidisciplinary care in the long-term management of progressive MS?
Well, it's very important. Progressive MS patients tend to be older, and they tend to be more disabled, and with age, you tend to see more comorbid conditions. So, it becomes very important, for example, that rehabilitation medicine be there to help individuals that are having difficulty with walking or difficulty with using their hands for PT and OT, and perhaps speech therapy. Comorbid conditions like depression and anxiety–very important to identify them and make sure that they are being optimally managed.
Progressive MS patients may have more in the way of symptoms–very important to have symptomatic management, and you're also looking at the aging MS individual, and so things like cognitive function testing may be very important. And certainly, there's an important role for the nurse, for physician extenders, nurse practitioners, or physicians’ assistants, as well as the neurologists. So really, a multidisciplinary care role can be very, very important for the progressive MS patient
My final question was, what might be key takeaways to leave with neurologists and other clinicians who are treating progressive MS?
And, I think key takeaways are, make the diagnosis accurately. Be thorough diagnostically early on to make sure you're properly labeling people. I think that that's a critical piece. Because progressive MS is in the setting of aging, it's important to try to have the central nervous system age as well as possible. You really want to emphasize the value of a wellness program in every progressive MS patient in particular. You want to help their CNS be able to withstand insults and to age better, so there needs to be a really profound discussion about that and appropriate recommendations made.
Be on the lookout for comorbid conditions. Make sure they're identified and optimally manage because if you don't, it's going to hurt the neurologic process for the MS individual, particularly the progressive MS individual. Make sure symptoms are being appropriately treated. That's really a quality of life issue, to have the patient be managing as well as possible.
And then, don't ever give up hope. We are now getting treatments for progressive MS. This has been a tough nut to crack, but we're now getting treatments for progressive MS. There are very promising clinical trials in CNS repair strategies where you could take an MS individual with fixed deficit and perhaps offer them the hope in the future of improvement in that fixed deficit. And so, I think there's a lot to be optimistic about because we have very smart minds that are now really turning to progressive MS and looking at how we can get better treatments, better management, and truly help this very significant form of MS.
I want to thank you very much for listening to me today.