Stiff Person Syndrome
Justin Abbatemarco, MD, discusses the the diagnosis and management of stiff person syndrome.
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Stiff Person Syndrome
Podcast Transcript
Neuro Pathways Podcast Series
Release Date: June1, 2024
Expiration Date: June 1, 2025
Estimated Time of Completion: 27 minutes
Stiff Person Syndrome
Justin Abbatemarco, MD
Description
Each podcast in the Neurological Institute series provides a brief, review of management strategies related to the topic.
Learning Objectives
- Review up to date and clinically pertinent topics related to neurological disease
- Discuss advances in the field of neurological diseases
- Describe options for the treatment and care of various neurological disease
Target Audience
Physicians and Advanced Practice providers in Family Practice, Internal Medicine & Subspecialties, Neurology, Nursing, Pediatrics, Psychology/Psychiatry, Radiology as well as Professors, Researchers, and Students.
ACCREDITATION
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CREDIT DESIGNATION
- American Medical Association (AMA)
Cleveland Clinic Center for Continuing Education designates this enduring material for a maximum of 0.50 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Participants claiming CME credit from this activity may submit the credit hours to the American Osteopathic Association for Category 2 credit.
- American Nurses Credentialing Center (ANCC)
Cleveland Clinic Center for Continuing Education designates this enduring material for a maximum of 0.50 ANCC contact hours.
- Certificate of Participation
A certificate of participation will be provided to other health care professionals for requesting credits in accordance with their professional boards and/or associations.
- American Board of Surgery (ABS)
Successful completion of this CME activity enables the learner to earn credit toward the CME requirements of the American Board of Surgery’s Continuous Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABS credit.
Credit will be reported within 30 days of claiming credit.
Podcast Series Director
Imad Najm, MD
Epilepsy Center
Additional Planner/Reviewer
Cindy Willis, DNP
Faculty
Justin Abbatemarco, MD
Mellen Center
Host
Glen Stevens, DO, PhD
Cleveland Clinic Brain Tumor and Neuro-Oncology Center
Agenda
Stiff Person Syndrome
Justin Abbatemarco, MD
Disclosures
In accordance with the Standards for Integrity and Independence issued by the Accreditation Council for Continuing Medical Education (ACCME), The Cleveland Clinic Center for Continuing Education mitigates all relevant conflicts of interest to ensure CME activities are free of commercial bias.
The following faculty have indicated that they may have a relationship, which in the context of their presentation(s), could be perceived as a potential conflict of interest:
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Imad Najm, MD |
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Glen Stevens, DO, PhD |
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The following faculty have indicated they have no relationship which, in the context of their presentation(s), could be perceived as a potential conflict of interest: Cindy Willis, DNP
CME Disclaimer
The information in this educational activity is provided for general medical education purposes only and is not meant to substitute for the independent medical judgment of a physician relative to diagnostic and treatment options of a specific patient's medical condition. The viewpoints expressed in this CME activity are those of the authors/faculty. They do not represent an endorsement by The Cleveland Clinic Foundation. In no event will The Cleveland Clinic Foundation be liable for any decision made or action taken in reliance upon the information provided through this CME activity.
HOW TO OBTAIN AMA PRA Category 1 Credits™, ANCC Contact Hours, OR CERTIFICATE OF PARTICIPATION:
Go to: Neuro Pathways Podcast June 1, 2024 to log into myCME and begin the activity evaluation and print your certificate If you need assistance, contact the CME office at myCME@ccf.org
Copyright © 2024 The Cleveland Clinic Foundation. All Rights Reserved.
Introduction: Neuro Pathways, a Cleveland Clinic podcast exploring the latest research discoveries and clinical advances in the fields of neurology, neurosurgery, neurorehab and psychiatry.
Glen Stevens, DO, PhD: Fluctuating muscle rigidity and painful spasms can severely impact mobility and quality of life of those with stiff person syndrome. Those same characteristics can also make the condition challenging to diagnose. In this episode of Neuro Pathways, we're diving into the perplexing and often misdiagnosed topic of stiff person syndrome, familiarizing listeners with the symptoms, latest diagnostic criteria and treatment options for this rare neuroimmunologic disorder. I'm your host, Glen Stevens, DO, PhD, neurologist/neuro-oncologist at Cleveland Clinics Neurological Institute, and joining me for today's conversation is Dr. Justin Abbatemarco, MD. Dr. Abbatemarco is a Neurologist in the Mellen Center for Multiple Sclerosis within Cleveland Clinics Neurological Institute. Justin, welcome back to Neuro Pathways.
Justin Abbatemarco, MD: Glenn. Thanks for having me.
Glen Stevens, DO, PhD: So, we've had you here before and we're going to be delving into a different autoimmune neurology topic today. That of stiff person syndrome, which those listening can't help but be somewhat familiar with in that Celine Dion was diagnosed with this in late 2022, which has brought it really to the forefront. And I remember an article saying one in a million. In this case, one in a million, not such a good thing. It's considered a rare diagnostic disorder. But before we get into stiff person syndrome, why don't you just tell us a little bit about yourself, how you made your way to Cleveland Clinic, what you do on a day-to-day basis?
Justin Abbatemarco, MD: Absolutely. And I will just say I think it's incredible what a celebrity endorsement does, increasing awareness, research. So, it's definitely been a positive in that light and we appreciate that kind of awareness around it. But yes, I work at the Mellen Center. I work with neuroimmunological diseases, specifically autoimmune diseases that affect the brain and spinal cord. So MS, neuromyelitis optica spectrum disorder, which we did an episode on, MOGAD and then autoimmune encephalitis and related disorders, which includes stiff person disease. Did my training here, came up, did second fellowship in University of Utah and then have been here for the last three years.
Glen Stevens, DO, PhD: Well, welcome. Always good to have you back. So as you mentioned with the neuromyelitis optica being a spectrum disorder, when I trained they didn't have spectrum disorders, but now everything is, they learn more, becomes a spectrum disorder. When I trained it was a stiff man syndrome. Now, of course, stiff person syndrome and others would call it stiff person spectrum disorder. So it really probably is a spectrum of disorders. Can you start by laying out the spectrum and the different types of phenotypes that we see?
Justin Abbatemarco, MD: Maybe as a quick aside, so again, that was first described as stiff man syndrome. Now we know it predominantly affects women, 70%/80% of the population. So maybe shows us some of our bias within the medical field. But as we've come along, I think we've really seen that there is this whole spectrum of disease. Stiff person syndrome is definitely the predominant phenotype, but we now know it can affect just one limb. And then there are more severe presentations like PERM, progressive encephalomyelitis with myoclonus and rigidity, which is probably the most extreme end of this disease and this whole spectrum of disorders. And then I think to make things even more complicated, there are other GAD65 related autoimmune diseases. We now know temporal lobe epilepsy, cerebellar ataxia, limbic encephalitis. And so I think it really creates this confusing world for I think neurologists, patients to delve into and understand.
Glen Stevens, DO, PhD: Is the epilepsy syndrome associated with the spectrum disorder or it's on the fringe and something different?
Justin Abbatemarco, MD: It's something that's associated with the GAD65 autoimmunity, and then we've seen other antibodies now associated with it. So it's a world that we're still understanding, but yeah one we put under the umbrella of GAD65 autoimmunity.
Glen Stevens, DO, PhD: And I'm sure we'll get into the GAD65 here in a little bit. But in terms of the different phenotypes that you mentioned, obviously makes it challenging, a little more difficult to reach the correct diagnosis. But take us through a diagnosis for a patient that comes in that you suspect might have one of these disorders. How do they present? What's the workup?
Justin Abbatemarco, MD: Absolutely. I think if we think about this in face value, right? If we talk about spasms and pain, that's a really common neurological concern that many of us see. And so how do we delve into this a little more to know when we should be thinking about it and testing for this disease? Maybe demographics could be a helpful start. So we're usually seeing this, again, 70%/80% of women, reproductive age, so around 30 to 40 years old is the most common phenotype. And then something maybe a little bit more unique is that 30, 40, maybe even 50% of these patients will have non-neurologic autoimmune diseases. So type 1 diabetes being one of the most common, autoimmune thyroid disorders, vitiligo. So other autoimmune diseases in that population would at least raise my at antenna that maybe we could be thinking about that.
When we talk about the symptoms that we'll see on this disease, so we do, we hear about spasms and rigidity. To help break that down, though, so the rigidity usually involves the trunk, so usually starts in the abdomen, low back region. Sometimes that rigidity can involve both sides and so it can create this hyperlordosis with this board-like feeling on abs and then it spreads into the extremities and so they'll have some rigidity usually in the lower extremities. On top of that, these patients will then experience these spasms. Again, it can involve trunk or appendicular, and these can be triggered by loud sounds, sometimes by touch, sometimes emotional triggers and they can be extremely painful. But that's the clinical phenotype that we see. It's a progressive disease, so it can start involving one area and then spread to others over months to years.
Glen Stevens, DO, PhD: Before we get into the diagnostic testing, average time? Do you have any idea of how long it takes for people to get diagnosed with this disorder? I'm sure it's not, "My symptoms started Wednesday and I was a diagnosed Thursday." I suspect it's months, years.
Justin Abbatemarco, MD: Definitely, right. I think the diagnosis is so challenging both ways. Misdiagnosis and then getting to the correct diagnosis. But, months. And again, these symptoms are insidious and so patients may write them off a little bit initially, and so it makes some challenges around this diagnostic procedure.
Glen Stevens, DO, PhD: So a person comes in, the phenotype sounds suspicious for one of these spectrum disorders. What are the tests that you're going to run on these patients?
Justin Abbatemarco, MD: I mean we start with the exam, right? There are a few clear exam features that will come up. So one of those elements would be seeing that stiffness, spasticity on examination, we usually see some increased tone. Again, checking around the abdominal and lower back region, which is not something we always do. Sometimes you can even see that hyperlordosis on examination. Patients usually will also have hyperreflexia and their gait will be really unsteady. So those are some key neurological examination features that could maybe point us in that direction.
Glen Stevens, DO, PhD: Do most of these people end up having imaging? I think everybody gets the image these days. So I suspect they probably have spine imaging, brain imaging to look for, do they have a posterior fossa abnormality affecting their gait?
Justin Abbatemarco, MD: We're dealing with such a rare neurological disease rolling out things that are more common, spondylosis, multiple sclerosis, something else along those lines that can mimic this. So the vast majority will eventually get it and it should be normal. So MRI brain, spinal cord, there's not usually any abnormalities on imaging which can create a little bit of a challenge. I think it leaves people guessing. Which part of the nervous system are we dealing with here?
Glen Stevens, DO, PhD: Is EMG helpful for this?
Justin Abbatemarco, MD: It's a controversial topic. I think we will use it. One, we can rule out other pathology. Is there a upper motor neuron type disorder? With patients with diabetes that always confounds the neurological examination, maybe their hyperreflexia is being masked. There are two or three specific EMG findings that we can see, but they can be seen in other neurological disorders. So two things that have been talked about in the literature are this co-contraction of agonist and antagonist muscles and in this inability to relax in a paraspinal regions, this continuous muscle activation in that region. Again, not entirely specific for SPS, can be seen in other diseases, can be voluntary in some patients, but these are things that have been described in the literature that may point us down that road that maybe we should be thinking about this.
Glen Stevens, DO, PhD: So is there a silver bullet with the blood test or no?
Justin Abbatemarco, MD: Definitely not. I think this creates the most confusion, right? We wish that there was just one test, the answer and we would be done. And again, when we have these nonspecific kind of symptoms to some degree? It makes it even more challenging to start putting together this puzzle.
Glen Stevens, DO, PhD: But what is the most common blood test that is done in this disorder?
Justin Abbatemarco, MD: So GAD65 and we've known about this since the 80s, so it's a test we've known about for a long time. We can see this positive though – 8-10% of healthy controls and in many other patients with autoimmune diseases, type 1 diabetes being the poster child, we've known about that for decades as well. I think when we talk about this antibody in terms of like SPS, we usually talk about very high titers. To make it even more confusing, it depends on how the test is done. There are a few ways that we can test for this each coming with their own units and how to interpret those. But I think high, tighter serum GAD is usually seen in 60-70% of these population.
Glen Stevens, DO, PhD: And CSF? Helpful, not helpful?
Justin Abbatemarco, MD: Super helpful. I think I get it on all of my patients that were considering this. There are a couple of things we can look for there. So we're dealing with an inflammatory disease, so just looking for inflammatory markers. An elevated IGT index, oligoclonal bands are really helpful markers. Maybe 40-60% of our patients will have those elements. We don't usually see a pleocytosis. CSF protein is always nonspecific, so I'm not sure always how to interpret that. But these are things have been reported. And then one of the more helpful features is to see CSF GAD positivity in the CSF. And so we can run those tests to see if the antibody is there. Some folks even asked or recommended to see intrathecal synthesis. So using that similar algorithm we use for an IGT index, using that albumin ratio to see if there's intrathecal GAD65 production. Could he be a little more specific? But again, that hasn't been proven on a prospective level, which is something that we've thought about as a field to help with this nonspecific diagnostic eval.
Glen Stevens, DO, PhD: But you would think that if you saw increased synthesis in the spinal fluid it would be pretty diagnostic.
Justin Abbatemarco, MD: It's helpful.
Glen Stevens, DO, PhD: You'd feel pretty comfortable.
Justin Abbatemarco, MD: So much more. I think CSF positivity as a starting point is really helpful. My suspicion will increase when we see that because we think it's way more specific than it is in serum.
Glen Stevens, DO, PhD: Now I guess it's complicated by the fact that you mentioned that you see other autoimmune disorders in these patients. So these patients may have diabetes and diabetes you can see the GAD65 antibodies. So a bit of an overlap, right?
Justin Abbatemarco, MD: Definitely, and then you can even have very high titers associated with the diabetes, so confusing to kind of parse these things apart.
Glen Stevens, DO, PhD: And do you have any sense for if we check somebody's titer and then we check it a month later, does it stay fairly similar or it fluctuates or do we not have data on that?
Justin Abbatemarco, MD: No. Some of the treatment trials that we've done, which are small, some retrospective. I think in general we haven't seen clear correlations with any of our autoimmune diseases. When we talk about NMDA, AQP four, we don't usually always see a treatment response. We don't always see the antibodies going away. And even if we do, it doesn't always respond to clinical response. And I think the same thing for this disease. It's not something that helps me on a clinical basis, usually.
Glen Stevens, DO, PhD: So are any of the governing bodies, whatever those may be, have definitive, possible, probable, could be, might be, don't think it is, does this exist?
Justin Abbatemarco, MD: There was a paper published in the Clinical and Translational Neurology journal. It was out of the Mayo Clinic group that had some suggested diagnostic criteria kind of talking about these same elements that we're talking about some demographics that make us more interested in this disease, clinical features that are more specific and in how to think through these diagnostic elements, but nothing that's been proven on a prospective basis or enrolled in multi-center kind of trials. So it's definitely something that's challenging and a little bit expert opinion based.
Glen Stevens, DO, PhD: Well I think it's always difficult because these trials will always have individuals that don't have the antibodies yet clinically everybody thinks they have the disorder. So then the real question is why don't they have the antibody? But as you learned with the neuromyelitis optica, it's a spectrum of disorders and there's probably a very similar look-alike disorder that has an antibody that we don't understand yet.
Justin Abbatemarco, MD: We definitely, actually we've seen that just recently. So glycine has come about now commercially available to test for. So we used to think about like 30-40% of patients were "antibody-negative", now seeing a portion of those patients with a glycine. Maybe one other antibody that folks could be aware of is amphiphysin, one that's been around for a long time. But we see that as a paraneoplastic kind of a company, usually associated with an OB kind of cancer. So breast cancer being the most common. So I do. This world is always evolving and we're going to see our testing I think become more specific and seeing other antibody associations as we go.
Glen Stevens, DO, PhD: It's amazing how we get smarter as better tests become available.
Justin Abbatemarco, MD: It's way easier that way, right?
Glen Stevens, DO, PhD: It's way easier as it moves forward. In your experience, would you say that you're making the diagnosis more commonly on your history and exam than you are in the CSF or the blood work or they're so intricately related that it really requires both?
Justin Abbatemarco, MD: When I talk to patients, right, and they're coming in for that second or third opinion, I use that puzzle analogy that each one of those puzzle pieces helps me put it together. Again, demographics, their symptoms, exam findings, then all the testing that we talked about - blood, CSF, EMG, and then things to rule out other disorders. It truly is trying to put together all of those different elements, but I think everything comes down to, do they fit that clinical description? And then we could think about treatment trials, the response there to help us confirm that diagnosis.
Glen Stevens, DO, PhD: So we feel comfortable making the diagnosis with a patient. So now they're going to come to you and say, Justin, so now what? What's my treatment? What's the algorithm?
Justin Abbatemarco, MD: A challenge. We thought the diagnostic algorithm was challenging, I think the treatment algorithm is just as challenging to navigate. When we talk about some of these autoimmune diseases, we've had great success, NMDA encephalitis, when we talk to patients, I tell them, I expect us to make a meaningful recovery. We're going to get this treatment going. It's going to be a little bit of time. For some reason, stiff person syndrome is a challenging diagnosis and can have a progressive course. It doesn't have this monophasic course of some of the other autoimmune diseases and it also doesn't respond as well to immunotherapy. And many, many agents have been tried. There have been a few prospective trials looking at this. I think IVIG has served as the backbone for this kind of treatment. And so seeing how patients respond there, but many patients will have only a partial response there that can wane over time. Again, this kind of progressive disability accrual we can see. Other medications that have been described, CellCept, azathioprine, rituximab, and some patients have even undergone autologous stem cell transplant.
Glen Stevens, DO, PhD: If IVIG doesn't work, do you do plasma exchange on these patients?
Justin Abbatemarco, MD: I think we definitely will consider that, especially for the more severe variant like PERM in the hospital. I don't use that as much as a long-term agent, but acutely trying to get someone out of a initial relapse or initial diagnosis is absolutely fair. But I think IVIG definitely serves as the backbone for my initial treatment.
Glen Stevens, DO, PhD: So steroids?
Justin Abbatemarco, MD: Less often because dealing with a long-term neurological disease, so just trying to avoid those kind of complications. Maybe I should also mention that benzodiazepines here, specifically Valium, has definitely been shown to be really helpful. Maybe a little less evidence for baclofen or tizanidine, but sometimes we augment that. So using some of those agents, immunotherapy and then at our institution we're really lucky to have a great PM&R group, so we think about botulinum toxin injections and even intrathecal baclofen pumps. Trying to think about this as holistically as we can along with that rehabilitation approach to treat them from every angle that we can because we know it's a challenging kind of disease to treat.
Glen Stevens, DO, PhD: Back in the Stone Age, when I used to treat some of these, it was really Valium-based.
Justin Abbatemarco, MD: And really if those patients are not responding to Valium or benzodiazepines, that should be a red flag in our minds that maybe we're not dealing with the right diagnosis. I think the same for IVIG. If they're not responding as we think they should, maybe to go back to the drawing board and think again about the diagnosis.
Glen Stevens, DO, PhD: Can we use titers as a marker for treatment? I mean obviously we're looking at the patient and we're just making an assessment. Are they better or not? Are titers helpful or not?
Justin Abbatemarco, MD: Not usually. It's a little bit based on symptoms. I think going to our physical therapists and hearing from them as they spend more and more time with the patient is always helpful from my end. But no, titers, in general, don't help with monitoring treatments or adjusting treatments moving on either.
Glen Stevens, DO, PhD: And realistic goal for patients, what are patients looking for?
Justin Abbatemarco, MD: I think their symptoms can interfere with all aspects of their life. I think improved quality of life. Sometimes these symptoms can be so severe, it can cause this anxiety about going out into social settings, work. And so how do we start reducing the impact on their activities of daily living? And again, we definitely talk about that multidisciplinary approach. Mental and physical wellness. Medications are definitely a piece to this. Having the right diagnosis, it's a real challenge, but I think specifically spasms, gait impairment and then maybe those specific phobias can be big domains that patients can be affected by.
Glen Stevens, DO, PhD: You mentioned that you can have a stimulus response. Can you desensitize patients with some type of de-stimulating program to touch or sound?
Justin Abbatemarco, MD: Yeah, we include our health psychologists involved with this. Sometimes psychiatry, I think anxiety and depression are really common comorbidities, have even been discussed. Is that part of the disease, right? We're dealing with this hyper-excitability disorder, so is that affecting those domains as well? I think that would make sense to me and I think having those programs involved are absolutely a part of the treatment plan for patients.
Glen Stevens, DO, PhD: And if I can just go back, if you're going to give someone IVIG, how frequently are you giving it?
Justin Abbatemarco, MD: I think there are different protocols out there. Some people will start with a relatively high dose of IVIG, like two grams per kilogram of ideal body weight every four weeks. But it is definitely a personalization as we go. In my practice, I'll usually try to start a little bit lower, see how they respond and adjust based on their symptoms. But again, emphasizing that holistic treatment paradigm that we have. So maybe starting monthly with like 0.4 or 5 grams per kilogram and then seeing how their symptoms respond. Is it wearing off? Are we having good response? I think the inverse is just as important. If patients are questioning their response or we think they've made some significant improvement, having a drug holiday, reducing the dose, playing with it and seeing how they respond to that. Trying to find the lowest effective dose, I think, is the most important thing rather than a starting dose. But having that constant conversation with patients over the months and years of this treatment.
Glen Stevens, DO, PhD: How long do they need to be on treatment before you would say you're not going to be responsive?
Justin Abbatemarco, MD: I tell patients that this is probably going to be a lifelong neurological disease and as they go through different phases of life, we have to think about that. If IVIG is effective, they can be on that lifelong, again, we could think about those other agents. There's been some talk about rituximab being that next line. Others have thought azathioprine or some of the oral agents could be maybe more effective. It's not clear. And that second line agents based on patient preference comorbidities and how those medications fit with their life.
Glen Stevens, DO, PhD: Do you see spontaneous remissions in this disorder?
Justin Abbatemarco, MD: We definitely can. It's a little unusual. If we had a paraneoplastic and we were able to remove that cancer, maybe that would be something we could see. But in general, it's usually a lifelong neurological disease and that's how I describe it to most of my patients.
Glen Stevens, DO, PhD: I think that's a good point that you raise. That's important for the audience is that a percentage of these patients will actually be a paraneoplastic disorder as opposed to a pure autoimmune, unrelated to a cancer. What's the breakdown on that?
Justin Abbatemarco, MD: I don't know the specific numbers, but I definitely think considering that diagnosis in the right patient population, if they have any risk factors for breast cancer, if they haven't been screened for mammography, making sure they're up-to-date on the age appropriate cancer screening is important. I think the inverse there as well. GAD65-related autoimmune disease, stiff person included, are not usually paraneoplastic. It’s just that amplifies then if that's coming up, that should definitely send us down a totally different ...
Glen Stevens, DO, PhD: Right. That would certainly concern me if that was positive.
Justin Abbatemarco, MD: Exactly. Totally different kind of treatment path that we have to think about.
Glen Stevens, DO, PhD: What about swallowing? Do you see swallowing difficulties with these patients? Do any of them have to get feeding tubes or weight loss, those types of things?
Justin Abbatemarco, MD: I think sometimes those spasms can involve, again, the truncal muscles and it can include the neck, speech therapy and optimizing medications in that end. We really try to avoid feeding tubes and things like that if we can.
Glen Stevens, DO, PhD: Do people become non-ambulatory?
Justin Abbatemarco, MD: It can definitely be something we think about and see, especially as the disease progresses. I think one of our big treatment focuses is going to keep people ambulatory with rehabilitation. Again, sometimes thinking about these alternative treatment regimens, like botulinum toxin injections, intrathecal baclofen, thinking outside the box again to manage these symptoms, because they're not always as responsive to immunotherapy.
Glen Stevens, DO, PhD: I'm interested in the possibility of the intrathecal baclofen. It seems like something that would hold a fair amount of potential promise if you could titer it well enough.
Justin Abbatemarco, MD: Our group did a little bit of retrospective work to see how that was. Again, a little bit of a mixed response, but some patients had a remarkable response to that treatment.
Glen Stevens, DO, PhD: So what's on the horizon? What's out there that not commonly being used or is being looked at?
Justin Abbatemarco, MD: I think there's still challenges around the diagnosis, which, I think, we talked about this in the beginning, but the awareness now around this disease. And so with that comes funding, research projects to look at this and how we have better biomarkers to make this diagnosis and follow it. We've talked about the challenges of just following titers, right, is not always enough. Making sure that people are getting the right diagnosis of they're not responding to treatment. And then we're seeing a whole new slew of more targeted immunotherapies for other diseases that we could apply here, right? NMOSD is that perfect example where we've discovered what's going on with underlying pathophysiology and targeting it. I think we need to still get there. We don't understand what's driving this disease to have a more targeted immunotherapy, right? We've just talked about things we've known about for decades. IVIG, azathioprine, imuran, rituximab. We have a ways to go.
Glen Stevens, DO, PhD: Well, it's true in that with rare neurologic disorders, the most important thing is probably a strong advocate. And this is not going to be the water bucket challenge of ALS from several years ago. But certainly in the general population, this is entered into the lexicon and people are starting to talk about and understand it and certainly hope that more funding will come into these rare neurologic disorders as a group.
Justin Abbatemarco, MD: Definitely.
Glen Stevens, DO, PhD: And it doesn't have to be private. It can be from the public sector as well, hopefully.
Justin Abbatemarco, MD: It's so important, right? Awareness for both patients, caregivers alike, it's so important.
Glen Stevens, DO, PhD: So it takes a village. Other resources for these patients besides some that you've mentioned. Probably social workers need to help these patients.
Justin Abbatemarco, MD: Definitely, right. Especially with disability as that accrues and difficulty with work, accommodations, things along that line. I think we've talked about that. But, again, really taking a step back and looking at that as a whole person, right. Mental health resource is super important. Our rehabilitation folks, primary care doc, especially when we're dealing with these immunosuppressive medications to keep them up to date on vaccines, cancer screening measures, it truly is a disease that requires a whole community to help them with.
Glen Stevens, DO, PhD: So, once again, Justin, I appreciate your coming here and shedding some light on a rare neurologic disorder that certainly affects, if not length of life, certainly quality of life in patients. And we look forward to hearing from you in a couple of years with some updates and new treatments and managements and better quality of life for patients with these disorders. So thank you for your time today.
Justin Abbatemarco, MD: I appreciate the opportunity.
Closing: This concludes this episode of Neuro Pathways. You can find additional podcast episodes on our website, clevelandclinic.org/neuropodcast, or subscribe to the podcast on iTunes, Google Play, Spotify, or wherever you get your podcasts. And don't forget, you can access real-time updates from experts in Cleveland Clinic's Neurological Institute on our Consult QD website. That's @CleClinicMD, all one word. And thank you for listening.
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A Cleveland Clinic podcast for medical professionals exploring the latest research discoveries and clinical advances in the fields of neurology, neurosurgery, neurorehab and psychiatry. Learn how the landscape for treating conditions of the brain, spine and nervous system is changing from experts in Cleveland Clinic's Neurological Institute.
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