The following is a transcript of a Q&A session at Spinal CSF Leak: Bridging the Gap 2024. Please note that the transcript been edited for length and clarity to center on the participant questions, whether put forward via online or in-person submission.
Question: Given the difference in cranial spinal elastance that Dr. Simy Parikh mentioned in her presentation, how do you measure compliance in the dura? And what is the risk of RIH in a CSF leak patient who has minimal or no orthostatic headaches?
And I actually do have another question: from my reading I’ve understood that superficial siderosis is a potential complication for untreated CSF leaks over time, and this is particularly so for ventral CSF leaks. So, I really wanted to understand what are the risk factors of developing superficial siderosis in CSF leak patients, like especially in terms of symptomology – like is there a correlation to the types of symptoms that patients have?
Answer:
Dr. Wouter Schievink: Yeah, so we’ve been able to follow some patients who we saw 20-25 years ago before we had advanced myelography to pinpoint the site of a leak. So, we would treat those patients with blood patches and then oftentimes they were feeling much better or they felt they were totally cured; their symptoms had completely resolved – but they were still leaking. That’s a not uncommon type of presentation. And then we contacted them or at some point they came to see us again and we found out that for patients who have a ventral leak, after 15 years there’s about a 50% chance of having developed superficial siderosis.
But you do have to be aware that some of those patients had no symptoms of superficial siderosis – it was just an MRI finding. And among patients with superficial siderosis, ventral leaks are the most common, but you also can get it from, for example, CSF-venous fistulas. You can get it in the setting of dural ectasia in, for example, Marfan syndrome. You also can get it with large pseudomeningoceles after spine surgery. But certainly, for ventral leaks, that’s a real concern.
And as far as elastance is concerned, that’s not something we usually measure where we work, but I think Jürgen might have some more information on that.
Dr. Jürgen Beck: I try to answer – it’s not easy. You do have a lot of data with lumbar infusion testing before and after surgical procedures and there is a change, but unfortunately, it’s not as straightforward as we had thought in the beginning. So, it’s very logical and you can analyze the data if you reach the patient early and if you treat the patient early – but after 3 months or even after one year, it’s getting really complicated and I still do not know what’s causing this. There is a change in the parameters from lumbar infusion testing but it’s really complicated in complicated cases. So do not have a straightforward answer for that.
But coming to the first problem, I would really stick to that what Wouter Schievink was telling you. I think a ventral leak is a chronic or the most significant chronic risk factor for developing siderosis, which is really toxic and you cannot reverse the symptoms 10 or 20 years later. So, I would opt for closing a leak despite having no symptoms at the time.
Moderator: Thank you so much. This is a question that might be a good one for both Dr. Schievink and Nicole Frost. What are the long-term physical limitations for somebody who’s had a CSF leak closed? Thinking back to the video we had of someone who had a surgical leak closed who seemed like a young active person who wanted to kind of get back to things but was understandably worried. In the long-term chronic period, would you have recommendations and restrictions and activities, and would that change if you were suspecting they may have an underlying connective tissue disorder?
Answer:
Dr. Wouter Schievink: I generally tell my patients that in the long term there are zero restrictions to what they can do except no neck manipulation. And I used to tell them no roller coasters but I don’t tell them that anymore. But you know, for everybody that’s a little bit different, right? So, I mean some patients don’t really want to take the risk of having another spinal fluid leak and they limit their activities and that’s reasonable, but I don’t give them any long-term restrictions.
Nicole Frost: I think it’s another one of our big unknowns and it has to be looked at very individually, and I think as Dr. Schievink – the risk benefit that the patient has to weigh up together with their team. Definitely co-occurring conditions like connective tissue disorders would come into the equation.
The level of function the person had before – ideally, I think we need to aim to get people back to full function – but if there is a history of recurrent leaking, significant connective tissue fragility or other factors, it may vary that in some situations and it just has to be weighed up very individually.
Question: Is there any national or even international connection forum or whatever for physicians that we as patients can gently suggest that they go to when we feel like they need updated information?
Answer:
Peter Kranz: I would say that currently there are aspirations to create that sort of forum and those efforts are underway. So, at the moment, I would say that if there are people out there who want to talk about CSF leaks, most of the people who spoke today love to talk about that with other physicians and would be happy to do it. And aspirationally, I hope that we will have that sort of environment that really promotes best practices and promotes research and promotes education specifically among healthcare providers, and hopefully we’ll have that soon.
Moderator: There’s some talk about GLP-1 medications as a viable option for rebound intracranial hypertension. Perhaps Dr. Carroll, you can speak to this as a potential – is there, do you see any potential there or promise there?
Answer:
Dr. Ian Carroll: There’s good animal data that GLP-1 agonists reduced intracranial pressure in animals, and they were doing a randomized control trial in the UK that I think – they closed because they were – I’m not sure for the reasons, but it was closed without completing the trial, I believe. There is a published case report that I participated in of somebody who felt that it was helpful for them. I’ve had two other patients that felt it was helpful for them. That’s anecdotal information.
In general, the GLP-1 agonists are medications that not only seem to reduce weight but improve glucose control without causing hypoglycemia and appear to be associated with increased longevity in patients taking them for things like diabetes. So that would suggest that, generally speaking, they are safe medications for many people. But I think with regard to data on their efficacy, we really have no idea and there’s no funding forthcoming to do the large randomized trial that would be needed to look at that.
Question: I am a caregiver to my young adult daughter, and my question is for Dr. Ruhoy. She does have multiple syndromes going on, and we’re in about our seventh year of chasing – probably the last two years we’ve gotten some answers to a lot of those questions. But we’re starting the long road of disability and considering that, and you mentioned that in your talk and I wondered if you had any direct work with your patients to narrow down what their primary condition would be, which is kind of what the paperwork asks for, and how would you counsel that?
Answer:
Dr. Ilene Ruhoy: That’s a hard question to answer because it really is individual, and I think a lot of the work that we do is really about trying to answer that question for a specific patient, and it can sometimes depend upon what the test results show. So, in fact, it very often depends upon that. I don’t obviously know your daughter’s case and I don’t know what’s been done or what hasn’t been done – but certainly the first-tier workup that I do is a lot of labs, a lot of imaging. Sometimes imaging has already been done but oftentimes not, or at least not the right imaging. So, I do that first and I sort of see what comes back and I take it from there and I think about the symptoms and I think about the history because I’ve taken a long history.
And then the next tier is – if I don’t have answers from that set of labs, sometimes I do follow-up labs based on the first set of labs, sometimes I do follow-up imaging based on the first set of imaging, and then I go down the route of looking at other kinds of – like I said, I do a lot of genetic kind of genetic workups, do targeted panels, do whole genome sequencing, and so I try to sort of put pieces of a puzzle together for an individual patient.
Because I realize we talk about the Septad patient and all these comorbid diagnoses but there isn’t yet really a clear intersection on how they all relate. There are lots of theories abound for sure, and sometimes the theories make sense, at least to me, and I don’t pretend that I’m the expert in everything, but sometimes I think well that doesn’t make a whole lot of sense, at least not for this patient. That’s sort of how I see it, you know, it doesn’t – those pieces of the puzzle don’t connect for this particular patient.
So, I just do a lot of workups and I try to sort of narrow it down to really like a sound theory on what I think the prominent diagnosis is and why I think that is and what could be related to it and how we’re going to go about treating it because that’s what the paperwork requires, right? Sort of this diagnosis, what you’re going to do about it, how long is this going to take, and then there’s all those questions about can this patient ever go back to work kind of thing.
Because I’m speaking specifically for that paperwork, that’s sort of how I do it because I know I have to be succinct and I have to be clear and it has to be somewhat objective and it has to have reasons for what I’m saying and so that’s how I approach that. But with regards to just overall treatment and management of patients and not worrying about filling out governmental paperwork, then there’s a lot more like I sort of go outside the box a little bit. But obviously that doesn’t apply when you’re filling out paperwork that has very specific answers to very specific questions.
Question: I’m the caregiver for [a leak patient]. For somebody with kind of complex, I would say, comorbidities, is there a better place or somewhere you would recommend over one of the top institutions for looking into a leak? And then my second question is – Dr. Carroll was referencing historical data. I’m an AI engineer so it’s very interesting how you guys are using artificial intelligence to look at all the data and if you are or aren’t.
Answer:
Dr. Peter Kranz: I’m going to take the second question first. I think that there actually are a lot of efforts to try to apply AI, and that’s true in lots of fields in medicine right now. AI is being applied to a lot of things, but in particular analysis of brain imaging and spine imaging and things like that are definitely ongoing topics of great interest. You know, as it applies to AI. I think because of the complexity of a lot of these different questions that we have and our limitations as human beings in terms of recognizing patterns and things like that, there’s a lot of potential for AI to sift through that massive amount of data and all these different symptoms and symptom profiles and clusters of symptoms and all those sorts of things to try to simplify that in a way that the human mind may not be able to do.
And then – I’m not 100% sure I understood the first question. Are you saying for patients who have multiple comorbidities, like are there places to go for that – for complex non-leak related issues?
Question (Continued): Yes, that is correct, but they present – there’s so potentially with hypermobile EDS there could be a leak present, so how do you choose the right center for somebody with those comorbidities?
Dr. Andrew Callen:
I think it really depends on the specific patient and the specific constellation of symptoms. It’s very hard to make a generalization, I think, for that, unless you can find where Dr. Ruhoy is and hope that there’s good concurrent leak treatment there too.
Moderator: Thank you for your questions and for attending Spinal CSF Leak: Bridging the Gap 2024.