TRANSCRIPT:

Voice-over: Welcome to Spotlight on Migraine, hosted by the Association of Migraine Disorders. Join us for fresh perspectives by medical experts and advocates as we explore the spectrum of migraine and dig deeper into this complex disease.

This episode is brought to you by our generous sponsors Amgen and Teva. 

Spinal CSF leak may be misdiagnosed as migraine, but they are very different conditions. A patient and provider give details on what spinal CSF is, what it is like to live with, and how it can be treated.

Jeremy Cutsforth-Gregory: My name is Jeremy Cutsforth-Gregory. I’m a neurologist at Mayo Clinic in Rochester, Minnesota. I’d like to spend a few minutes talking about spinal CSF leak. We’ll center the discussion around these four questions. First, what is spinal CSF leak and how is it different from cranial CSF leak? Second, what are the causes or pathologies of spinal CSF leak? Third, what are the symptoms? And, fourth, what are the treatment options? 

Spontaneous spinal CSF leak goes by many names. It’s originally known as aliquorrhea, or lack of spinal fluid production, but is more recently called low CSF pressure headache, CSF hypovolemia, CSF volume depletion, or, most commonly, spontaneous intracranial hypotension. Whatever you call it, it’s distinct from the post-dural puncture headache that patients may get after a spinal tap. It’s also distinct from cranial or skull base leak of spinal fluid, which the fluid usually comes out the nose or the ear. 

Let’s talk about those distinctions. Spinal CSF leak occurs when leakage of fluid is at the level of the spine. The fluid usually distributes out into the soft tissues or the bloodstream, and it’s invisible in that way. In contrast, a skull base CSF leak comes out the bottom of the skull and usually appears out the nose or out the ear. 

Spinal CSF leak symptoms are usually worse with upright posture like standing, whereas skull base leaks may be exacerbated simply by tilting the head forward or to the side. Spinal leaks are occasionally associated with connective tissue disorders, whereas in skull base leaks, there’s a prior history of trauma or surgery or perhaps idiopathic intracranial hypertension, where the primary problem is actually high pressure rather than low. 

And, finally — and probably most importantly for patients — treatment is different. Spinal CSF leaks are treated by plugging the leak and raising spinal-fluid pressure. Skull base leaks are treated almost always by surgical plugging the leak and then reducing pressure if high pressure was the primary problem. 

We classify spinal CSF leaks into one of these three categories. In the upper left panel is a fast leak near the front of the spine because of a small bone spur. A leak along a nerve root sleeve that occurs close in to the spine can also cause a fluid collection in the spine and be a fast leak. That’s the upper right panel. In the lower left panel, we have a nerve root sleeve tear, in which the fluid escapes out of the spine off into the soft tissues. We usually call these meningeal nerve root sleeve diverticulums or cysts, but it’s not always the biggest one that’s caused the leak. 

And, finally, the most recently discovered type of leak is called a CSF venous fistula, where the spinal fluid sac, or dural sac, gets directly connected to a vein. And spinal fluid doesn’t wash off into nowhere, but instead directly into the venous system and washes off with the bloodstream. 

There are a few identified risk factors for spinal CSF leak, including joint hypermobility, sometimes with a diagnosed connective-tissue disorder like Marfan syndrome or Ehlers-Danlos syndromes; the bone-spur-type leak that I already mentioned; or the intracranial hypertension, which I also mentioned.

There’s an association with bariatric surgery. And, finally, for those CSF venous fistulas, there may be an underlying venous or venolymphatic malformation. 

I like to measure just how flexible patients are with a very simple scale called the Beighton hypermobility score. You simply ask the patient to touch their thumb down to the forearm or bend the pinky backwards or the elbows backwards, and the final points come from bending the knees backwards or touching the palms flat to the floor. A score of five points or more out of the nine possible is consistent with joint hypermobility syndrome, and it just makes it a bit more likely that that patient could develop a leak, although, certainly, there are flexible patients without leaks and patients with leaks who are not flexible. 

There are common symptoms and uncommon or rare symptoms of spinal CSF leak. By far the most common is orthostatic headache. This is a headache that comes on when patients are upright and gets better when they lie flat. Over time, that pattern might be lost, so it’s really important to think back to how the headaches started and recognize if there was an orthostatic pattern back then. Nausea and vomiting are common, usually also orthostatic. Back pain is quite common, but it’s important to note the site of the pain is often not the site of the leak.

A number of problems can develop when the nerves that come out of the base of the brain are stretched or compressed, so people can get double vision or other visual complaints, or dizziness, vertigo, altered hearing, plugged-ear feeling, all those cochleovestibular symptoms. Those would all be common symptoms of spinal CSF leak. Less common are other headache patterns, say, a patient whose headache only comes on with coughing or actually gets worse when they lie flat. We’ve seen almost any type of headache, so it’s important to be open-minded. 

There’s a type of dementia that can come from degenerative disease of the brain but be mimicked by a severe brain sag and a spinal CSF leak. It’s called frontotemporal dementia or frontotemporal brain sagging syndrome, when leak is the cause. A few symptoms might come out of the spine itself by compressing the spinal cord or stretching the nerve roots, but, again, most of these symptoms result because of the changes in the brain related to the leak in the spine. 

If patients have symptoms suggestive of a spinal fluid leak, then we should be looking for a leak, but I think it’s important to address this systematically. Question number one: Is there an active CSF leak? Question number two: Can we classify that leak as fast, slow, or maybe a fistula? And, finally, we get to question number three, which is “Where is the leak?”

We all want to get to question three — find the leak and fix it — but we have to know first that there actually is a leak, because the symptoms I mentioned can be caused by a variety of things, not just spinal CSF leak. Here at Mayo Clinic, we follow this algorithm. Generally, if the patient has a story suggestive of a leak, we start with an MRI of the brain, and that’s with contrast. About 80 percent of the time, there will be abnormalities on that MRI that suggests the patient indeed has a spinal CSF leak. The brain might sag down or the dura — again, the lining around the brain that’s supposed to hold all the spinal fluid in — can be thickened and take up the contrast dye.

Twenty percent of patients with leaks will have a normal MRI brain scan, however. In that case, we’ll do a test called a radioisotope cisternogram. It’s not going to find the leak — it’s low-resolution imaging — but it is going to show changes if a leak is present. It’s done by doing a spinal tap and injecting a mildly radioactive tracer that then diffuses up through the spinal fluid up and over the top of the brain. Within 24 hours, there should be tracer over the top of the brain. If a patient has a spinal leak, however, the tracer will never quite make it over the top of the brain and we’ll be able to tell that the patient has a leak and be able to move on to the next step of finding it.

To separate fast leaks from slow leaks, we do an MRI of the spine. If there’s spinal fluid outside the dural sac but inside the spine, we know there’s a leak and it’s a fast one. We just don’t know where it is yet. But, in that circumstance — that’s the left side of the algorithm here — we’ll do a prone hyperdynamic CT myelogram, getting pictures in real time while the patient has the dye injected in the CT scanner. 

In contrast, and actually more commonly these days, the MRI of the spine does not show pockets of spinal fluid in the spine and we presume then that the leak is either slow or perhaps a fistula directly off into a vein. For either of those leaks, they tend to come off the side of the dural sac, and so we’ll do a myelogram with the patient on their side, what we call lateral decubitus digital subtraction myelogram. 

Once you find the leak, you can move on to treatment. Now, conservative treatments are just that, quite easy: hydration, caffeine, bed rest. And, for some patients, those are effective. If patients’ symptoms go beyond one month, in my experience, they tend not to improve on their own further.

So if they get to me and they’ve already tried the conservative things for a month or more, I’m more than ready to move on to the next step, which is often an epidural blood patch. This is taking the patient’s own blood out of a vein in their arm, usually, and injecting it through something like a spinal tap into the back. The difference between a spinal tap and a blood patch is that the needle goes in a little further for a spinal tap and not quite so far for the epidural blood patch. 

And we might do a blood patch at one level. We might do it before or after we know exactly where the leak is. Perhaps we spread the blood over multiple levels if we have multiple suspicious sites. And we may or may not include fibrin glue to try to make that blood patch stickier.

If a leak is confirmed in terms of its location and conservative or blood patching strategies haven’t worked, then surgery usually comes into play. And that might be sewing up or suturing the defect in the dura. It might mean cutting off a nerve root and all the associated veins. Or, most recently, we’ve pioneered a procedure we call paraspinal vein embolization. This is only for CSF venous fistulas, but one of our radiologists will use a catheter-based minimally invasive technique to navigate up to the culprit level of the spine and glue the vein shut from inside. The nerve root remains untouched, there’s no incision of the skin, and minimal recovery for an outpatient procedure. It’s been quite effective, but, again, it’s only for CSF venous fistulas.

Aiza Jose: Well, hello, everyone. First of all, I’d like to thank the Association of Migraine Disorders and the Coalition for Headaches and Migraine Patients for the invitation to speak to you all today about my spinal CSF leak journey. And before we get started, I’d like to give you an outline. I’m going to talk about my background, so you know who I am; my journey; and then I’m going to be talking about my diagnosis and what I did to overcome my illness. 

And just to give you a little bit of how active I was and then what happened to me, so you can see I’m a mom of two children, and I am married. I have two dogs, and I am actually an engineer. And I’m open about — do quite a bit of work outside as well as travel and meet with a variety of people in an office setting, as you can see there. I’m also pretty active. I exercise daily, and I do lots of outdoors activity, as you can see there. 

All this changed back in 2014. I started — all of a sudden, without any preexistence of headache problems — I started with some tachycardia and headache that wouldn’t stop. I went to the doctor, and the first diagnosis was just migraines. Lots of studies, and they determined for over five years that I just had migraines.

And that eventually was obvious that was not the only problem I was having, as eventually I went into full disability, and I was totally bedridden for about five months back in 2017. Thank goodness, in July 2017, I was diagnosed with spontaneous intracranial hypotension due to a CSF spinal leak. In October 2017, I received three multilevel blood patches, which was my cure at the time. I did have a complication in 2017, which is pretty common but easily managed with medication, which is called rebound high pressure. 

I was pretty good. I did physical therapy. During the time that I was bedridden, I lost my job. But, 2018, I did a lot of physical therapy. I was determined. I went back to work and doing the same work I was doing before. I was good for several years. 

In 2020, during the pandemic, I actually had another leak or I reopened the old leak, and again I was three months bedridden again. And, again, it was pretty disabling because I couldn’t really walk without falling. In August 2020, again, I had several patches, multilevel patches, and this time I was totally fixed. Again, a lot of physical therapy, and today I have no rebound headache pressure. And I do have some PTSD after all that I went through, but I’m working on it, and we’ll talk about that in a little bit.

It was not only the headache; it was, as I said, the tachycardia. But my headaches were non-positional, so it was a very hard diagnosis. I had a lot of brain fog, mental haze, photophobia, sound sensitivity, a lot of visual disturbances. The lines were fuzzy; the colors were not bright. I had a lot of nausea. I lost a lot of weight. And, finally, the worst symptom of all — because that was really what caused my disability — was the lack of balance. 

A lot of treatments — I probably tried everything that was available for migraines and other neurological symptoms. Nothing seemed to work. I even had infusion therapy. None of it worked or just worked marginally or just briefly. But nothing was helpful for me until I found the right doctor, the right diagnosis, and the treatment. 

My doctor, Dr. Deborah Friedman, at UT Southwestern, diagnosed me pretty quickly. I had blood patches under MRIs. I also had myelograms to check where possibly the leak was located. I also had, as I said, some extra post-leak challenges, including the rebound intracranial hypertension, or the high pressure that I talked about during my chronological events. I also had this last time restless leg syndrome, or fasciculations, which is the twitching of your legs, which I have resolved with physical therapy and running. 

As I said, I have some PTSD anxiety, especially when I was living through my second leak and through COVID. I was extremely worried about catching COVID and reopening the leak, but I’m working on it, reassuring myself that I won’t get sick again. And even if I get sick, I could get treatment. 

For prevention and management, I do have some residual headaches. Most of the time — I would say 95 percent of the time — I’m fine. I have my life back. I work, I do activities, I exercise, but I do have prevention and management. Here are some of the medications that I currently take. And I have these ear plugs that I wear when there’s changes in barometric pressure, which seems to be some of my triggers. 

Getting my life back, I did a lot of physical therapy. Here are some of therapists. I jog, which has been very helpful for me. I used to be a jogger all my life, and so jogging has been my rescue too. And this is a photo of me back at work. You can see that I’m just doing the same work I was doing before. I’m feeling pretty powerful about it, apparently. 

The support groups have been my lifesavers. There were times that were extremely difficult for me, especially when I was bedridden. And these different groups — the Spinal CSF Leak, both in the US and Canada, and the CSF Leak Association in the UK — have been extremely helpful, providing information for me, as well as the Facebook groups — just different experiences from people that suffer the illness. There’s the two — the CSF LEAKS, as well as the Rebound High Pressure groups — that have provided friendships that up to this date have been the best that I’ve done in my life. 

This picture here was made by one of my friends that was doing some awareness. And this is the picture of my back after this last round of multilevel patches, and she made some artistics out of it for an awareness campaign that she was doing. 

So with that, I’d like to thank you all for your attention. I hope that some of the information that I share with you is helpful.

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Voice-over: Thank you for tuning in to Spotlight on Migraine. For more information on migraine disease, please visit MigraineDisorders.org.

*The contents of this podcast are intended for general informational purposes only and do not constitute professional medical advice, diagnosis, or treatment. Always seek the advice of a physician or other qualified health provider with any questions you may have regarding a medical condition. The speaker does not recommend or endorse any specific course of treatment, products, procedures, opinions, or other information that may be mentioned. Reliance on any information provided by this content is solely at your own risk.