Slides
Transcript
Well, thank you very much, and I just again would like to introduce a little bit where we are placed in the world close to the Swiss border. We are not Switzerland. We are not Bern. We like them, but we are not Bern. We are Freiburg, Germany. And this is the team, and we are so happy. I think I sneaked in from the side as a neurologist. I work now in the department of neurosurgery handling the CSF patients, and it’s so important to have this team around. We are so happy to have now a CSF nurse with us. We have our OR nurses outside and here within the group showing how we work, and it’s all based on teamwork. Otherwise, this field cannot evolve.
This is our hospital. This is the view on the neuro center, and on the side we have this beautiful graffiti just showing two black boxes, and I think probably that’s what I’m going to talk about now. I will mainly talk about what I don’t know, and I hope that you will tell me after that what we need to change.
So first I would like to introduce a little bit the concept of headache and neck pain and talk a little bit about what I think we might need to do, which is detangling what is actually orthostatic headache in SIH or orthostatic symptoms in SIH. And also I will share a little bit of what we do with headaches after leak closure.
We mainly regard the trigeminal system as the messenger of headache and neck pain. And what is pain-sensitive here? It’s the meninges, it’s the arteries, it’s the veins, and it transduces via the brainstem. Also, we have some input from the upper cervical nerve root. So it’s C1, C2 is also involved — the occipital nerve part in pain perception in general. I stole this from my brain, but in all the headache systems we talk about the trigeminal activation.
Now, we look into SIH. It would seem really obvious — we have a meningeal enhancement. So hyperemia of the meninges, we have hematoma, we have this smooshed brainstem. So we have an affection of venous system, arteries, and the meninges. It would seem logical that this might play a role in headache perception in SIH, but as Peter Kranz’s group and others — and I think that’s probably what we all share from our clinical perspective — it doesn’t match headache intensity.
If we look at our patients, sometimes I open up an image and I see this full-blown bilateral subdural. I think I really need to check on that patient — is it up, how he is, do we need to do emergency evacuation, or whatever, calling for the neurosurgeons obviously. And then the patient walks across in the hall and says, “I’m absolutely fine. I have this little bit back there, but I just came anyway because I did have the appointment.” And I think that’s what the data also showed — it doesn’t resolve headache intensity.
We’ve been a little bit curious about this, because how can this be? We might just find something else in there. So I looked into a cohort of our patients — very typical female dominance, typical leak — and they were all very much affected by headache and mainly orthostatic headache. And we looked at a lot of things like age, does it matter? Gender, does it matter? Do different subsections of the Bern score matter? Does it matter how the brain is sagged? For example, does it matter which type of leak is it? Can we somehow resolve orthostatic pain in SIH or pain in general?
And also, I will not go into detail with that, but it’s one of my main research interests. I look into non-invasive assessment of intradural dynamics — so the dynamics of the CSF within the cranio-cervical junction. And what we found is that none of the investigated parameters resolved orthostatic or non-orthostatic pain in those patients. But the intradural dynamics, and it’s very important that what we see here is a glimpse into compliance actually. So we measure time, we solve velocities, and it gives us some idea what the compliance of the system is like. It’s not an absolute measure of pressure. It will never be that. But we have an idea — how stiff is the system and how does it react to every pulse.
And we can see in the early phase, again in the patients with dural tears, we can highly resolve headache impact by these measurements. We have a very, very good lineage of low compliance in the system would lead to higher headache impact, but only in the early phase. If the disease lasts a little bit longer, then the correlation is much less. It’s still significant, but it’s much less. And if the duration of the disease is more than a year, I again cannot resolve headache intensity.
So what are we left with? The same thing we know from other studies — somehow the CSF circulation adapts over time. And if we measure pressure, if we measure resistance to outflow, although the patient does have orthostatic headache, the clinical presentation is the same. We cannot resolve it by the dynamic test. So the question is what happens after that? What changes? What actually causes the orthostatic symptom in SIH after the disease has lasted? And so I would rather call this that we get into an orthostatic dysfunction. It’s not necessarily the low volume that causes the symptoms. It’s something that happens maybe even outside.
And we already discussed a lot about this — about venous systems, what actually helps us to raise our heads, to stand up. And I think what we know is that we don’t know how the system actually balances itself. And probably we need to go into this some more also to get better in treatment options.
You’ve already seen this image — this is Florian’s work with a headache impact — and it has already been pointed out we see this tremendous effect of headache resolution after surgical repair for leak. But also we see those patients, like a quarter of them, remaining with persisting headaches, and we need to take care of this.
So what do we do after leak closure? I think again here we need to talk to patients. We cannot just do science in the back of the hospital. We need to talk to patients, and we need to get a good story again. And I think one of the major questions is: has your headache changed now? Is it still the same, or has it changed? And then go into detail and maybe get a good idea what else does the patient present with.
Orthostatic, remaining orthostatic headache after leak closure — first, have you done follow-up? We’ve already discussed this. You need to follow up. You need to make sure that the leak is actually sealed, no matter what you did. Do adequate imaging, know which type of leak is what. Lalani was already telling us about that — fistulas can reoccur, so we need to check for that.
Also, we did quite a lot of post–dural puncture headache, and this is to Ian here. We need to think that we did this during the procedure quite many times, and we closed the leak, so we decreased compliance. We might think about that. We might have caused a leak after that.
What can we do? Literature is really scarce, and what’s really funny is that we try to recommend caffeine in the first place, maybe later on, to increase CSF production, but the data actually says that caffeine given acutely reduces CSF production and increases pain sensitivity. So we might need to have this in mind. On the good side, if you are a coffee drinker or black tea drinker and you just continue that long enough, your CSF production will increase.
There is some RCT data out on gabapentin, but let’s be honest — very, very low numbers, and it derives from post-dural puncture headaches. But it was in favor of treating with gabapentin for this type of headache.
And I really like the greater occipital nerve blocks because you don’t do systemic treatment, and there are some case reports out of that. But if you talk about the neck and head pain, the greater occipital nerve block is something worthwhile trying. And also there are some case reports on sphenopalatine block.
What else? We might talk about caffeine withdrawal. Does the patient still take acetazolamide or topiramate — something that actually lowers the pressure? When you have that in mind, and POTS, there is data out that you cannot discern POTS from SIH, but yet the patient might have a Postural Tachycardia Syndrome. And the question is, is it really orthostatic? Worsening in the second half of the day might just be hypertensive. So not everything that is better lying down — maybe the patient can just stand the pain better. So it’s really worthwhile listening to what might be the change in symptoms here.
That leads me to rebound hypertension. We already discussed a lot about this, and we heard from Lalani’s and Andrew’s work, I think with the collaborators also from Freiburg, that they saw about like high-end 20% numbers of rebound hypertension. That has been described by about Wouter Schievink as well and also by Frederico Cagnazzo with his team in Montpellier. They looked into this. So we get about a third of rebound hypertension, usually very short, very short-lasting, and you can treat it with acetazolamide. We taper — we usually go very low, maybe just on an individual basis having one or half a pill, and usually it’s gone after two weeks.
Keep the side effects in mind. It’s a nasty drug. You need to tell the patient about it. They just need to be aware that nothing is destroyed if they take it. So it’s just a tingling, and it’s worth checking for the potassium. And maybe some patients just lose a little bit of potassium with it.
What if the symptoms prolong? And I will not go over the entire slide. Or if they’re very severe, what do we need to think about? I think one of those major parts is underlying IIH. Do we have an underlying hypertensive disorder with all the differentials that come with it? And also usually there’s no need to do another lumbar puncture. We know we closed the leak, we expect that pressure rises, and if we do a good enough, well enough differential, we should be clear here and don’t need to necessarily do invasive diagnostics.
What about the other headaches? So an easy step would just be treat the phenotype. Listen to the phenotype. Treat the phenotype. Also migraines, headaches have a high prevalence in the normal population. One-third here in the room should have migraine, and the likelihood is that our SIH cohort comes with some migraine that is maybe triggered due to the CSF circulation disorder. It’s important to talk to the patient about this, and then we treat the migraine. That’s at least a little bit we can take off.
A couple of weeks ago I caught the hemicrania continua. So that’s the part where the neurologist can do a little bit of good in this disease and come in and add to the treatment. And also we need to talk about medication overuse, but in my experience that’s not really common in SIH patients.
What else? It’s still human beings, so if somebody is in pain, the neck muscle, the craniomandibular muscles, they work with that, and just do whatever you can to improve pain perception in those patients.
So the first outtake is that orthostatic headache is not a low volume headache. So it’s not the easy explanation: you lose the CSF and that’s what causes the headache. It’s more complicated, especially in the long run. I would propose that we see it as an orthostatic dysfunction.
And what can we do after leak closure? I’m really happy to hear what you do. I think we do the best we can to improve whatever this specific patient presents to us.
What else can we do? We can raise awareness. We need to level out our resources, and we need data. And this was already coming up earlier. We need to align our data, get better, not only looking into our retrospective cohorts. And that’s why we are now running a registry, and we started this with a very easy approach where we have a lot of data, comprehensive data, tailored to SIH patients — not only looking into headache but quality of life and what do they bring prior to the disease. And we assess this in an electronic survey. So it’s really easy handling of data, and we add the typical imaging parameters and treatment outcomes for those level B or C centers who are actually capable of doing so. So assessing demographics of an SIH patient is something that probably a level A or community hospital even can fulfill, and then the high-level diagnostics and therapies — those need to be aligned obviously. And we’ve started this registry in November. We are currently at close to 100 SIH patients and 31 chronic post-dural puncture headache patients just in our center, and we’re now working on connecting with other centers as well to build this network and to learn and actually get better data.
And I’m so happy that [muffled], who’s here, and Frederico Cagnazzo from OPG, and of course the Danish headache center in Bern — we are currently discussing together with Lalani Carlton Jones how we can align this. And I’m — oh sorry, I did not know this, this is sorry. Coming here, I forgot to get this out. And with this I would love to thank you and hear your thoughts later on in the discussion session, I think. And thank you for listening.