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International Parkinson and Movement Disorder Society
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Special Series: Approach to patients with acquired choreas

May 20, 2024
Dr. Ruth Walker and Dr. Molly Cincotta discuss causes of acquired chorea and approach to the work-up of a patient with an acquired chorea.

[00:00:04] Dr. Ruth Walker: Hello, and welcome to the MDS podcast, the official podcast of the International Parkinson and Movement Disorders Society. I'm Professor Ruth Walker from the James J. Peters Veterans Affairs Medical Center in the Bronx and Mount Sinai School of Medicine in New York City, and I'm the guest editor for this special series on Chorea.

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Today, I'm interviewing Dr. Molly Cincotta from Temple University in Philadelphia, Pennsylvania in the United States. She will be discussing causes of acquired chorea and her approach to the workup of the patient with an acquired chorea. So Molly, thank you so much for joining us. today. So I just want to start off with, when do you start thinking about an acquired cause of the patient's chorea as opposed to like a genetic cause?

[00:00:52] Dr. Molly Cincotta: Well, thank you very much for having me and for that particular question, I think a lot of times the, the biggest tell is going to be the time [00:01:00] course. So, for a lot of acquired choreas, you're going to see more of a, a subacute onset or an acute onset of symptoms versus a lot of the genetic choreas, which tend to be a little bit more slow in their progression and and have like a longer Ramp up time to when they come to medical attention.

In addition to that I would say that the distribution of the chorea can give you some clues. We found that when a chorea is significantly. asymmetric, so one side of the body is much more affected than the other, or unilateral, that's typically going to be much more of a clue that we might be dealing with an acquired chorea versus the genetic ones, which tend to be a little bit more symmetric in their onset.

[00:01:49] Dr. Ruth Walker: So what about in the neurological examination? Any other particular features apart from the distribution of chorea which can give you clues as to the, [00:02:00] the etiology or the fact that it's an acquired chorea?

[00:02:03] Dr. Molly Cincotta: So I do think the distribution can be very helpful.

And there's certainly distribution features that might also suggest that it is not an acquired chorea. So, making sure that you look at their facial involvement forehead involvement is something we think about more on Huntington's disease, or if there's a particular. Eating dystonia that is very associated with chorea acanthocytosis or VSP13A disease. But in the absence of those types of features I would look for other neurologic signs and symptoms that might suggest that there's something going on that would explain it. 

 So, for example, if you would see someone with an infarct causing chorea, they may have neighborhood signs related to where the stroke happened or sensory findings that correspond with the side of the body that is having the abnormal movement. You [00:03:00] may also see cases where there's associated neurologic symptoms or even.

You can see patients who have physical symptoms, medical symptoms that would point you in a certain direction. So for example, hyperthyroidism, they may have symptoms like weight loss fast heart rate or be sweating more. Someone with a polycythemia vera may be complaining of headaches or have some flushing.

But I also think it's just important to take that really good history as far as what, their background is because typically the clues are going to be in things that are happening around the onset of symptoms that you might associate with. increasing their risk of developing one of these acquired choreas.

[00:03:47] Dr. Ruth Walker: Yeah, absolutely. And also, you know, as with any good thorough medical history, we're going to talk about like, medications and things like that. That's obviously Something we never want to forget. So I just wanted to touch [00:04:00] briefly on COVID, which has obviously been in all of our minds for the past few years.

What's the evidence that COVID or COVID vaccination can cause chorea?

[00:04:12] Dr. Molly Cincotta: There are certainly some cases out there where we've seen COVID itself or the COVID vaccine lead to new onset chorea. I will say on a population scale, especially given how many people have developed COVID 19 infection, as well as how many people have received the vaccine. This is a very, very small number of people that we're seeing this in.

And Obviously, some of that literature is limited by just how many case reports are coming out, but there was also a lot of attention being paid to this particular issue. So I do think when it was showing up, there were a lot of individuals writing about it. Particularly, there were a couple of cases with new onset chorea after the vaccinations.

And this did. Span the different types of [00:05:00] vaccinations that were available. It didn't seem to be just 1 particular manufacturer or brand. These seemed to be potentially. an immune mediated process, some of them responded to things like steroids and they didn't seem to continue on lifelong or get worse over time.

But the follow up on this is obviously still ongoing. When it comes to both the COVID vaccine and COVID induced chorea, though, we do have to keep in mind that there are often other things that are going on with the disease medications, other exposures, secondary infections that can. Kind of cloud the picture.

So people, when they're hospitalized with these diseases especially when they're very ill can develop hospital induced or illness induced delirium and sometimes abnormal movements related to that they can have renal failure, which can lead to uremia. So it's always important to keep in mind. [00:06:00] Other potential confounders as well as medications they may have been exposed to.

And then beyond that particular issue it's also important to keep in mind that some of these individuals may just have an underlying disease or vulnerability to chorea in the same way that you might see in someone developing chorea Gravidarum or another illness induced type of Korea and just the timing of things may either unmask that or make mild symptoms worse or even just happen to coincide at the same time.

So I do think it's always important to look for other potential causes when you're suspecting a COVID 19 or vaccine induced chorea.

[00:06:45] Dr. Ruth Walker: Thank you. That's very interesting. So I want to focus a little bit more on a particular entity, which has been related to COVID 19 infection, which is the development of diabetes and the relationship of diabetes to [00:07:00] COVID infection. And certainly one of the best recognized causes of asymmetric chorea is the non ketotic diabetic hemi chorea hemibolismus syndrome.

So can you tell us a little bit more about that and, what Why on earth is it classically asymmetric?

[00:07:19] Dr. Molly Cincotta: So, first of all, I do think that name is such a mouthful and in the literature, there doesn't seem to be like a standardized way that we abbreviate that complicated, but very important name.

But yes looking through the literature, I definitely haven't been able to find one perfect explanation for why this is so asymmetric. There's a lot of theories that, I think if you take it together might suggest this is related to something about the blood flow to the basal ganglia and the thought that there's probably some asymmetry.

In how the smaller blood vessels [00:08:00] are able to access this deep structure. This is a little bit hard to pin down because we're talking about the blood vessels that you're not going to be able to see easily on a cTA or even invasive angiogram imaging, it's sometimes you can see a more proximal stenosis and we do know that stenosis in vessels can lead to chorea in some cases, but these individuals who are getting hemichorea from hyperglycemia, typically they're older.

They have obviously a lot of vascular risk factors of the diabetes being one and typically because it's It's generally a poorly controlled diabetes if they're ending up in this situation. Not always, but you would presume that they, they're having difficulty keeping their blood sugar at a normal level.

So they may have accumulated some of the consequences of this over time. Atherosclerosis, many of them will [00:09:00] also have high blood pressure, high cholesterol, the typical vascular risk factors that we associate with strokes and vascular issues. So it does seem like there may be some sort of hypo perfusion occurring and then whether that hypo perfusion is being exacerbated by increased viscosity from the high sugar, or just. Changing the metabolism within the basal ganglia in a way that affects 1 side more than the other because of that relative difference in perfusion.

I think that's probably the best explanation. Although again, it's hard to really say we don't fully understand exactly why this occurs at all. There's some. Thoughts about these, these changes in metabolism, but it, it does continue to be an ongoing area where we're trying to uncover the pathophysiology and there's a lot of sort of contradictory information out there.

And there are some cases where people get [00:10:00] bilateral symptoms from this, but it does tend to be either very asymmetric or completely unilateral. I did also see in some paper that I'm, I always have trouble refinding it, but they were talking about the possibility that we just have a dominant side when it comes to these deeper structures, the same way that, you know, we have a dominant side when it comes to language and that potentially that was part of what's contributing.

I haven't seen much in the way of conformational studies on that, but I do think it's an interesting concept that, you know, we may have part of our basal ganglia that is. Doing more work than than the other side. And therefore one or the other sides would be more vulnerable to changes in metabolism.

[00:10:42] Dr. Ruth Walker: Now that's interesting. Yeah, I've not come across that paper, but that's, yeah, definitely makes sense. And also I was just thinking just in terms of the circuitry, maybe it doesn't take a lot for like, one side of the circuitry to be kind of flipped into a state which generates much more amplified [00:11:00] hyperkinetic movements than the other side, but,

[00:11:03] Dr. Molly Cincotta: It does seem like there's, I mean, there's obviously a threshold that's getting passed. And so the idea that that threshold wouldn't be the same on both sides or would affect one side more than the other, doesn't seem that. Crazy, but it is interesting because of how unilateral it often presents. And the fact that the imaging also tends to back this up.

You'll see 1 side very clearly defined on your T1 MRI and the other side looks pretty normal. So I think that's a really interesting aspect of this particular disorder.

[00:11:36] Dr. Ruth Walker: There's certainly a lot of work to be done in these people. Another thing that, seems to be in the literature is that often when people have autoimmune or paraneoplastic choreas, it's often quite asymmetric. Do you think that's kind of a, a similar, thinking behind that asymmetry as well?

[00:11:56] Dr. Molly Cincotta: I haven't been able to find, again, much that really looks into [00:12:00] this question. I actually think that asymmetry in hyperglycemic chorea is occasionally addressed, but typically in all these cases, it's a bit glossed over when, when people are talking about the pathophysiology. I do think a similar asymmetry in blood flow could account for some of this.

As could asymmetry in dominant basal ganglia being more vulnerable to these antibodies with the autoimmune disorders you know, we do often see asymmetry, but not always and not as consistently as we see it in hyperglycemic. Syndromes and also they often have more facial involvement in certain 1, particularly, I'm thinking in NMDA there tends to be a little more facial involvement.

We also see this asymmetry in, sydenham disease, which is thought to also be autoimmune just as a Perry infectious a manifestation. So, you know, you would [00:13:00] wonder if there's something about how these antibodies are being delivered to the blood brain barrier. That may be asymmetric either at baseline or again, if there's 1 side, that's going to be more active or less active if, if there's some vulnerability there,

[00:13:19] Dr. Ruth Walker: So, I know now that, when I see people in clinic and I've seen, recently seen a number of older people with a very clearly asymmetric chorea these are definitely not people I would think of, as having late onset Huntington's or anything like that, there's definitely more room for systematic evaluations, but I do feel like, the sense that we got from the literature that these are metabolic etiologies paraneoplastic autoimmune is, I feel like that's, certainly potentially informative.

You also mentioned briefly, and I'm going to kind of throw this in you mentioned chorea gravidarum and, what do you think about the role of Sydenham's anti ASO, [00:14:00] et cetera, antibodies in chorea gavidarum.

[00:14:03] Dr. Molly Cincotta: so it does seem like and definitely historically individuals who had Sydenham as a child and then went on to become pregnant. We're at higher risk of developing chorea gravidarum these days, particularly in the U. S. where Sydenham disease is relatively uncommon. Chorea gravidarum is seen more in individuals who have other types of risk factors.

Usually systemic auto immune diseases that might put them at higher risk of developing chorea. There does seem to be a relationship between. The dopamine system and estrogen that may be somewhat protective in diseases like Parkinson's disease, but then leads to more vulnerability for individuals with hyperkinetic movement disorders like chorea.

So the literature also talks about even in Huntington's disease women or those with higher levels of estrogen have more chorea, even [00:15:00] throughout their disease course in comparison to, male patients. So, I do think that there's some evidence for that.

And that. The vulnerability seems to have occurred when they had that initial insult, whether that was the ASO anti antibodies in childhood with Sydenham disease or some other immune. Mediated process later in life that maybe broke down that blood brain barrier a little bit more.

[00:15:28] Dr. Ruth Walker: Interesting. So can you tell us a little bit about how you would approach, the individual with a, an asymmetric chorea in terms of your workup, diagnostic testing?

[00:15:40] Dr. Molly Cincotta: Yeah, I, I would say that even if they don't have an asymmetric chorea and if they don't have a very strong history that suggests, Huntington's disease is high on the differential or very likely, I think there's a lot of utility and making sure that anything potentially treatable is ruled out [00:16:00] 1st and typically this involves blood tests.

So I try to get as much of that. Completed within reason as early as possible, because if there's something we can reverse, we definitely want to do that initially. Now, the presentation, I think also really will influence this. So if they're coming to the ER with relatively acute onset, chorea, really at the top of my differential are going to be things like hemichorea from hyperglycemia.

or potentially infarct or vascular issues. So that is going to be a relatively quick to rule out or rule in as a potential cause and should be, attended to expediently. But in the clinic setting, typically people have had symptoms for long enough that we're not necessarily thinking this is an acute stroke or that their glucose is going to be so high that they would need, to be admitted for an insulin drip without Potentially, a few other symptoms to to include there.

So, in those cases, I'm going to be sending blood [00:17:00] tests for other potential reversible causes, essentially and that mostly is acquired causes, but I do also look for some of the more easily tested Genetic causes, particularly things like ceruloplasm and serum copper for Wilson's disease because even though that is a genetic disorder, it is treatable and wouldn't be something we want to miss.

So I as many of us do have a list of tests that I, I just sort of bring up and go through what seems reasonable. And that'll include some basic testing. Thyroid, occasionally, if depending on the timeframe perineoplastic antibodies or NMDA and inflammatory markers, things of that nature.

And then I typically would always try to get an MRI of the brain because again, lesions For a symmetric, any sort of localizing, exam, you certainly would want to see if there's something you can structurally identify that could help get to the answer and [00:18:00] then, depending on the situation, a lumbar puncture might be appropriate.

I often will wait to see what the initial serum tests show, particularly the sort of lower hanging fruit, you know, things that will come back within a week or two. Before I jumped to doing invasive testing, particularly because our perineoplastic antibodies from the serum are a pretty good test and do often come back positive when people have an autoimmune or perineoplastic etiology and the logistics and patient reticence to getting a lumbar puncture can often be a barrier, but I would have a relatively low threshold to do that for anyone with a fairly rapid onset, or over the course of a couple months, developing a new onset asymmetric chorea particularly if the most obvious things like a medication change or or high glucose or something of that nature doesn't come back with anything revealing.

[00:18:58] Dr. Ruth Walker: And it's also, I mean, it's worth mentioning [00:19:00] that, perineoplastic panel is only as complete as the tests which are done. There are still probably a significant number of. Autoimmune antibodies, which are yet to be discovered. And there are often patients who remain undiagnosed.

And so you know, certainly do you always do like the imaging as well? Does that guide you?

[00:19:23] Dr. Molly Cincotta: Yeah, for sure. I would definitely get imaging in, in most cases of, of new onset chorea because I do think it can help to narrow your differential and guide your treatment. And also, depending on what you see on an MRI scan, if there's. Signs that could be more suggestive of an autoimmune disorder, even if your antibody panels are coming back negative, or you're waiting for them

you don't want to wait on a potentially life saving treatment. So if. If enough of the exam history align or enough starts to align, but then your, [00:20:00] your MRI really pushes you in a certain direction, empirically treating someone while you're waiting for that additional testing to come back is quite appropriate.

And I was certainly wouldn't want to hold off on that simply because the panels didn't reveal the answer as we know, every kind of test, you have false positives, false negatives, and just things we don't know to look for yet. And since autoimmune encephalitis is a still very rapidly growing field and also because we keep realizing that some of these disorders have, presentations that we didn't recognize or symptoms that were not previously described. I think it's good to have a broad differential when approaching that.

[00:20:41] Dr. Ruth Walker: Great. Yeah, absolutely. And what about like whole body imaging and PET scanning to look for occult malignancy?

[00:20:49] Dr. Molly Cincotta: So this is not something I've done very much clinically. I do think there are probably circumstances where it is a good idea to really search for a [00:21:00] potential. malignancy well, I guess I should take that back. I, I. Frequently have asked to get a chest and abdomen pelvis CT scans to look for possible occult malignancies.

Unfortunately, this isn't the most sensitive way to look for these things, especially because in a lot of cases, a malignancy is going to be very small and potentially, potentially even be suppressed by some of these antibodies, which is an interesting trait. Of this particular disorder. So when thinking about more specific imaging, whole body pet scans and things of that nature those can be a little bit challenging to obtain, but certainly do have a role in looking when you have enough clinical suspicion, because ultimately, Their recovery is going to be dependent on identifying the malignancy and treating that directly.

So you may be able to reduce symptoms with things like immune modulating drugs. But if the malignancy remains [00:22:00] untreated not only will that eventually cause issues just by the fact that it is cancer, but also their, their neurologic symptoms are not going to remain in remission.

[00:22:12] Dr. Ruth Walker: Great. I mean, we've got lots of material here. I don't know if you want to, I asked, you know, I don't know if you want to get into therapies as well. Say a couple of words about therapies.

[00:22:21] Dr. Molly Cincotta: Yeah, I mean, I think therapies actually kind of get a little more simple again, in some ways. I mean, ultimately, you want to be able to reverse the underlying cause if you can identify one. But after that there's a relatively short list of medications that have a good effect on controlling the symptoms of chorea.

And I also strongly recommend treating to the patient's needs rather than treating just because the chorea is there. So if it's mild, and it's not impairing to the patient, but potentially, they don't need treatment at that particular time. And since many of these treatments can have side effects, [00:23:00] it's good to minimize that as much as possible.

But obviously, chorea can cause a lot of impairment. So, in those situations, you want to try to look for ways of relieving it. I do think that the The sort of broad answer is usually to look to dopamine blocking agents either neuroleptic medications or VMAT2 inhibitors and these can be very effective and occasionally in some of these disorders can address other symptoms particularly the, the dopamine blocking medications can treat psychiatric symptoms that may be comorbid with some of these disorders.

On the other hand they can have side effects and for any diseases. That have overlap with other movement disorders like Parkinsonism and some of these certainly do you can either bring on Parkinsonism or exacerbated and sometimes those side effects are not necessarily worth the the reduction in chorea.

So I do think that [00:24:00] balancing that is very important and then there's some less specific medications that can potentially help out. So benzodiazepines, particularly clonazepam, can help to alleviate some symptoms. Occasionally propanolol may be able to alleviate some symptoms. And then in some cases you might be able to use something like amantadine, which can both help with Parkinsonism and hyperkinetic movements.

So I will occasionally use that, particularly for. Folks with tardive dyskinesia, because they often have multiple things going on at once these medications tend to be a little less robust in their ability to suppress the Korea, but occasionally help you kind of walk the line between side effects and relief.

And then if it's a very focal area, again I'll bring up tardive dyskinesia. Sometimes Botox injections can help to reduce certain movements to either reduce the amount of dopamine blockade they need or you know, even get them off of a medication they might be having side effects [00:25:00] to.

[00:25:00] Dr. Ruth Walker: So that's really helpful. So thank you so much, Molly. It's been a really interesting discussion and we look forward to learning more about the asymmetric acquired careers going forward. And it's been really nice talking to you.

[00:25:14] Dr. Molly Cincotta: It's been great talking to you two, and thank you very much for having me on.


Special thank you to:

Dr. Molly Cincotta
Assistant Professor of Clinical Neurology,
Temple University 

Dr. Ruth Walker 

James J. Peters Veterans Affairs Medical Center in the Bronx and Mount Sinai School of Medicine

New York City, NY, USA

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