Melanie Cole (Host): Welcome to UAB Med Cast. I'm Melanie Cole. Today, we're exploring the management of spinal vascular lesions with Dr. Jesse Jones. He's an Assistant Professor and an Interventional Neuroradiologist at UAB Medicine and Dr. Will Meador, he's an Associate Professor and Neurologist at UAB Medicine. Gentlemen, thank you so much for being with us today. Dr. Meador, I'd like to start with you. Can you tell us a little bit about spinal vascular lesions, the etiology? Just talk a little bit about spinal vascular disease and what symptoms would bring attention to a provider.

Will Meador, MD (Guest): Sure. So spinal vascular disease is typically first evaluated by neurologists with patients who have symptoms localizing to the spinal cord. About 80% of those lesions are dural AV fistulas, or malformations of arterial or venous tree. And in that situation, patients typically present with episodic or stepwise progression. They may develop neurologic symptoms like weakness, stiffness, or spasticity, loss of bladder and bowel control that come and go, especially when they are under strain or walking for longer distances in a form called neurogenic claudication. And those patients, they're often worked up for other spinal cord pathology, such as transverse myelitis and other conditions, but ultimately need evaluation for possible dural AV fistula, cavernous malformations or other forms that can commonly present with spinal cord vascular lesions, and those also need to be considered in the initial workup.

Jesse Jones, MD (Guest): Dr. Meador, can you talk a little bit about distinguishing vascular lesions. I think my understanding is they're quite rare, versus the much more common garden variety say, a compressive myelopathy, or even like a vitamin deficiency. Is there a difference in presentation between these various spinal pathologies, the common versus the zebras if you will?

Dr. Meador: Absolutely, again, getting back to that claudication aspect of the presentation. So with compressive lesions, those tend to be progressive and constant over time, whereas the vascular malformations tend to have these fluctuating courses. And so we see patients who come in and they're often normal on exam or have very minimal findings, but they report, if they walk for longer distances, they have these profound symptoms.

And so that's a big clue when we're worried about something such as a dural AV fistula. With vitamin deficiencies, with other causes of myelopathy, those tend to be slowly progressive. So for a subacute combined degeneration, for example, those may present with several weeks or months of progressive deterioration.

Host: So then Dr. Meador, how is a patient with suspected disease worked up and eventually diagnosed? Tell us how important early diagnosis is to being crucial to improving the outcome prediction.

Dr. Meador: So working these patients up adequately is vital because they can sustain irreversible disability if it's not managed appropriately. And so we need to consider these things early in the workup, which begins of course, with a thorough neurologic examination, where we're assessing for reflexes and signs of myelopathy, such as spasticity. We're asking questions about neurogenic bladder and bowel, which are common in these patients. And we also need to consider the time course heavily when we're thinking about their history. Again, listening for those kinds of claudication like events. But beyond that, we usually start with imaging.

We may proceed with lumbar puncture for CSF analysis, to look for inflammatory causes of such symptoms. We would do blood work to look for common vitamin deficiencies and other causes of myelopathy, such as B12 or copper levels. And then we would proceed with more advanced imaging after that.

Dr. Jones: So by advanced imaging, you're talking about for instance, like an MRI?

Dr. Meador: Yeah. And so an MRI angiogram can be done of the spinal canal and those vary, I think in quality, based on the facility, that's performing them. But that may be an option, also DWI sequences, which are not commonly done on MRI of the spinal cord can be obtained as well, especially in the acute setting. And then further on to that, I would refer them to someone like yourself, Dr. Jones.

Dr. Jones: Gotcha. Yeah, reading these studies, it can be, sometimes very obvious to diagnose lesions where there's very large vessels. For instance, a spinal arterial venous malformation or another entity called a perimedullary fistula and arteriovenous fistula of the spine. These typically have very prominent blood vessels and they're quite obvious.

And other lesions, as you mentioned, dural fistulas, can be very difficult to diagnose, even with MRI, typically, you know what we're going to see is this some edema or swelling typically within the conus or the bottom of the spinal cord. Just because humans are upright and mainly we're upright walking, sitting, and the edema tends to develop in the dependent portions of the spinal cord being the conus.

But beyond that, you may not see an obvious flow void or blood vessel. And it really comes down to either, as you mentioned, a spinal angiogram or some of the more specialized studies we do here UAB such as a CT pigtail angiogram to really diagnose these dural fistulas.

Host: Dr. Jones, is there anything exciting in neuroradiologic imaging, anything that excites you that other providers might not know that you're doing at UAB? You just started to mention one.

Dr. Jones: So I think, given the challenges of diagnosing some of these spinal vascular lesions that really don't have a really obvious correlate on MRI, but the patient is obviously has symptoms as Dr. Meador alluded to either it's a claudication with activity or unexplained myelopathy. What we'll typically move on to is a study called a pigtail CT angiogram.

And that's something that is done at UAB. Where the patient has a catheter, which is a long, skinny tube placed into their aorta. And this catheter is kind of like, an irrigation hose that has a lot of little small openings in it. And contrast is injected through all these tiny openings and fills the aorta.

And as it fills the aorta, all of the spinal arteries and there's typically 31 of these, coursing from the aorta into the spinal canal are opacified. And we can actually see and interrogate each spinal artery, one by one, and look for a subtle sign of a dural fistula. This has proven to be very helpful to diagnose difficult or challenging cases.

Dr. Meador: And thinking about Dr. Jones, when we refer patients over to you for evaluation for such advanced imaging, are there any specific tests or things that we should consider and rule out before we refer them to your group?

Dr. Jones: I think, as you mentioned, just the thoroughness of the exam on your side, in terms of your neurologic exam and the, and the notes. I typically refer to those when I'm working up a patient and the MRI is very important to give us any kind of clue. Like I said, either some edema in the conus, which would lead us towards a vascular lesion, such as a dural fistula. Oftentimes the difficult cases that end up seeing me or my colleagues after years of a fruitless workup. And we're not really sure what's going on with these people. They don't really have cord edema. They may have more of a focal lesion, like a transverse myelitis type picture. But they don't have any of the more classic findings of a spinal vascular lesion. I think that's where the spinal angiogram can be very important in terms of being a gold standard.

Host: So which one of you would like to discuss treatment options now? Dr. Jones, I think that would be you tell us a little bit about the parameters for treatment modalities.

Dr. Jones: Once a spinal vascular lesion is found, really comes down to what the specific lesion is. And this is going to come to the realm of a sub-specialized provider to counsel patients appropriately. And that's either going to be a vascular neurosurgeon or an interventional neuroradiologist.

For instance, the dural fistula, these are typically treated surgically where that vein that receives this fistula's blood flow, can be exposed and clipped during a surgery. And, leading up to this, the spinal angiogram can be very helpful for the surgeon to identify exactly where the draining vein is located and really facilitate their surgery.

Some of the more intrinsic cord lesions, spinal vascular lesions that are actually within the parenchyma of the spinal cord, pose a real treatment challenge. You can't get to them easily because they're buried within an otherwise functioning spinal cord and they pose a lot of operative risk or perioperative morbidity. And so a lot of times what is done with these is they're observed. We try to minimize patients' use of any kind of anticoagulation that would predispose them to having bleeding and try to control their blood pressure. And if they do have unfortunately, an event where they have a bleeding episode, then we typically would go in and do a combination of an open surgery combined with an endovascular procedure to close off some of the vessels feeding into these lesions.

Dr. Meador: And once Dr. Jones's team is finished, hopefully, resolving the vascular malformation, of course, then we will manage subsequent spasticity and neurogenic bladder, gait disorder, et cetera, within the neurology clinic following up from that.

Dr. Jones: And Dr. Meador, what's your experience with like I say, once these have been diagnosed and, hopefully treated thoroughly, what's the prognosis in terms of, does rehab play a role, inpatient versus outpatient or are there other kind of adjuvant things that can be done to facilitate these people's recovery?

Dr. Meador: We really approached them pretty aggressively from a rehabilitation standpoint. And if patients are diagnosed with these vascular malformations in the inpatient setting, we would definitely push for inpatient rehabilitation, but most of the rehab, will actually occur as an outpatient, in the ambulatory setting. Assuming that they are ambulatory, at least can get to and from physical therapy. If they do have gait disorder to the point that they have difficulty with transportation and to getting into those appointments, we will try for inpatient rehabilitation. I think there's a lot of benefit to that, the biggest thing from my perspective is early recognition and early treatment because as you know, a lot of these deficits will be irreversible and if we can catch it early and treat it early, with the help of experts like yourself, then we will at least prevent any further disability accumulation.

Dr. Jones: And what's the best way for physicians out there that are suspecting a spinal vascular lesion or just something with a spinal cord that they're concerned about. What's the best way to get these people the care they need?

Dr. Meador: I think it's really important to try to get them evaluated soon, because neurology access is a problem nationwide and especially in our region, unfortunately, but trying to get these patients evaluated sooner rather than later by a neurologist, as you mentioned, earlier for that kind of detailed neurologic exam and detailed history to see if we suspect this condition. And then if we do, then neurologists should refer them to a center that can perform these advanced imaging studies, to rule that out very promptly.

Host: Well, thank you for telling us about the importance also of the multidisciplinary approach, certainly in the post-treatment modalities. So, Dr. Meador please talk briefly about ischemic myelopathy. You have some things you'd like to discuss that you would like other providers to know about. Please talk about those now.

Dr. Meador: I think when thinking about vascular disease in the spinal cord, we really need to also think about ischemic myelopothy or spinal cord stroke. Somewhere between one and 2% of all ischemic strokes in the United States are spinal cord strokes. And so it's often under-recognized. I think people learn in medical school about the anterior spinal artery infarct, which is the classic presentation, but it's actually quite rare that the patients present with such a classic presentation, where they have sparing of the dorsal columns. They can have, hemi-cord effects. They can have complete or partial transverse myelitis like presentations. Many of these patients have pain associated at the site of the lesion, which is a bit atypical for ischemic lesions and makes it a bit unique. And these are often proceeded with TIAs.

So these patients often have some transient ischemic attack, like event before that would localize to the cord. And then it went on to full on stroke later on. So I think that needs to be considered in these patients. And we need to consider that in any patient with a myelopathy because I do think it is under-recognized in the community.

Dr. Jones: That's very interesting Dr. Meador. And is it believed that these spinal cord strokes are primarily the result of atherosclerosis in those vessels, like in the aorta and the spinal arteries or is it more of an embolic phenomenon?

Dr. Meador: So most of these are likely atherosclerosis, from small penetrating arteries in the spinal cord, typically coming off of the anterior spinal artery because you have two posterior spinal arteries, right? So you have some redundancy and you have collateral flow there a little bit better than the anterior spinal artery. So most of these tend to be central core where the gray matter is, which has highest demand for blood flow, of course, but also anteriorly. And so these are thought to be primarily atherosclerotic. Most of the embolic disease that enters into the posterior circulation, will of course go north, if you will, to the brainstem or to the posterior cerebral arteries.

Dr. Jones: Well, that's something very interesting and also important, I think for providers of spinal angiography to be aware of as well. These are people that certainly, if it's highly suspected should not be going on to spinal angiography, given the risk associated with catheter placement in a diseased artery like that.

Dr. Meador: Absolutely.

Host: We'll do either review have anything you'd like to add as a final thought for other providers about what you're doing at UAB Medicine, Dr. Jones, why don't you start for us and give us your final thoughts.

Dr. Jones: Spinal vascular lesions are rare entities, but they're very important to be diagnosed because they're treatable. And if they're caught early, patients can have a remarkably good outcome in terms of functional improvement. Whereas if patients who are worked up and neglected and not get the MRI that they need or not get the neurology referral that they need; they unfortunately get to the point where they're diagnosed and there's really no useful treatment options. So I think working these people up early is extremely important, whenever it's suspected.

Host: And Dr. Meador, last word to you, what would you like other providers to take away from this fascinating interview today?

Dr. Meador: I think awareness the biggest element that we need to be thinking about this condition. And if we hear symptoms from patients that are suggestive of it, as Dr. Jones alluded to, we really need to get them worked up quickly and promptly and completely, because we really want to avoid disability or troubles accumulating.

Host: Thank you both so much for joining us. What an interesting interview. And a physician can refer a patient to UAB Medicine by calling the mist line at one 800-UAB-MIST. Or by visiting our website at UAB medicine.org/physician. That concludes this episode of UAB Med Cast. For updates on the latest medical advancements, breakthroughs and research, follow us on your social channels. I'm Melanie Cole.