Migraine or Spinal Fluid Leak?
“Do you feel better when you lie down?” My migraine specialist asked this question at a recent appointment. He wanted me to consider a new treatment approach. After a lifetime of migraine that turned chronic 10 years ago, I am among many who are desperate for relief. There is research gaining momentum focusing on spinal cerebrospinal fluid (CSF), CSF leaks, and how they relate to migraine.
What is a spinal CSF leak
The Cedars Sinai Medical Center explains that "Spinal CSF leaks can be caused by surgeries, spinal taps, and also as a result of nerve root cysts. Spontaneous leaks may also arise from bone spurs along the spine. A spinal CSF leak is when a hole or tear in the dura allows this fluid to leak out. When the volume of CSF becomes low, the brain will sag inside the skull, causing migraine-like headaches that worsen when the patient is in an upright position (sitting or standing up) and improve when lying down. It must be noted that not all headaches that improve when lying down mean there is a spinal CSF leak present."1
Pain from a spinal CSF leak or migraine?
It is due to the similarity in severity of pain between a headache caused by a spinal CSF leak and a migraine that patients are sometimes misdiagnosed as having migraine when they actually are suffering from low spinal CSF levels.
Personally, I am eager to lie down when an attack is at its worst. However, I am often unable to lie flat right away. In fact, doing so often increases the pain. I will, therefore, spend hours icepack on my neck, easing my way down into a horizontal position. Even so, given other known symptoms related to CSF, including joint hypermobility, my doctor felt I’d be a good candidate for evaluation.
Researchers leading the way
I was referred to Dr. Linda Gray-Leithe, MD, a Duke neuroradiologist, who is among those leading the way in spinal CSF leak research. Bahram Mokri, MD of Mayo Clinic in Rochester, now deceased, advanced understanding of this issue in recent decades and work at the Mayo Clinic continues. Wouter Schievink, MD and his team at Cedars-Sinai in Los Angeles, has published extensively on this topic and continues to be a leader in the field. They offer a full range of diagnostic imaging, spinal injection procedures, and surgery. The team of four neuroradiologists at Duke has been active in this area, led by Gray-Leithe, MD. They do a range of diagnostic imaging and spine injection procedures, as well as refer for surgery when indicated.
There are various diagnostic and treatment options available depending on the needs of the specific patient. For me, it was a three procedure-process which occurred over the course of two separate days, spaced several weeks apart.
CT-guided lumbar puncture
The first procedure was a CT-guided Lumbar Puncture. This involved the use of a CT scan for careful placement of a needle to perform a spinal tap to get a read of the CSF levels in my spine. I went into this procedure with a migraine on the pain scale of 7. The team inserted fluid into my spine while monitoring my pain. The idea was that if I had low levels of spinal CSF caused by a leak, the temporary addition of fluid should restore those levels to normal, resulting in pain relief.
As I lay on the table during the procedure, I felt no initial improvement, but then, after an additional increase in fluids, I did notice a lift from and clearing of pain. This procedure is quite subjective and I suspect has a high likelihood for a placebo effect for those of us desperate for improvement. Surrounded by doctors asking if you are improving every few minutes might lead us to want to respond accordingly. That said, I do remember feeling a clarity from pain that is unusual for me. It was determined that my spinal CSF levels were indeed low and that I would therefore be a good candidate to proceed with the next diagnostic evaluation. It should be noted that the removal and addition of fluid done at Duke is not performed at other centers.
Myelogram showing cysts on my spine
The second and third procedures were conducted on a separate day. To prepare for the second diagnostic procedure, a Myelogram, I once again underwent a CT-guided Lumbar Puncture. Contrast was inserted and images were taken of the spine, spinal cord, and surrounding areas searching for leak sites. This procedure does not rely on the subjective opinion of the patient. As with much in medicine, the images to be read by the radiologist do not always point to an obvious solution. In my case, at least, it was not entirely clear whether or not to proceed. There were questionable-looking cysts on my spine (the presence of these cysts are quite common) which could potentially be causing CSF leaks, but there was no obvious leak in my case.
The neuroradiologist who oversaw my case admitted that if it weren’t for my chief complaint of frequent migraine, he may not have proceeded with patching the leak sites. Based on my history of migraines and report of improvement during the first diagnostic test, he decided to go ahead.
Plugging the leak with an epidural blood patch
The third procedure, an epidural blood patch, was conducted later the same day. This involved inserting my own blood back into my spine in the locations where potential leak sites had been identified. The theory is that my blood, inserted in precisely the right location, would clot and close the leaks. Some patients report immediate resolution of headache after this procedure. For others, improvement may take a couple of days, as the procedure itself can cause a high-pressure rebound headache (not the same as MOH).
This reaction, doctors theorize, is based on continued overproduction of spinal fluid by a body attempting to address a leak. Once plugged, it may take days to weeks for the system to realize it no longer needs to overproduce the fluid and for the high-pressure rebound headache to resolve. For those who experience positive results, the period of relief varies from months to years.
All three procedures went very smoothly and were uneventful. Once home and recovering, I experienced intense but expected soreness in my back for the first two days. I believe I did have a bit of a high pressure rebound headache for several days to a week. It felt quite similar to my regular migraine attacks. I was prescribed a specific medication to address it which was not terribly effective.
I certainly did not experience the dramatic lift in pain that some report. However, I have noticed some improvement in my overall pain pattern. It should be noted that it is possible to have both migraine and low spinal fluid resulting from a spinal CSF leak. In this scenario, plugging the leak sites may resolve the headaches related to the low spinal fluids only. However, for those of us with chronic migraine, we welcome and embrace any relief from pain. And perhaps in that scenario, the resolution of spinal CSF-leak-related headache could turn a chronic migraineur into someone who is episodic. In my case, however, it appears that my daily pain is still quite present, yet my episodic migraines (which appear atop that pain several times a week) have somewhat decreased. I consulted with the neuroradiologist at Duke who performed my procedure about this outcome and he did not seem to feel confident that their procedure would have had this impact.
Regardless of my outcome, I was very impressed with the idea behind this approach, the momentum building around the research, and its success thus far. I am surprised by how little information exists on the topic for the general public. Awareness is on the rise with the goal being to identify those who are undiagnosed and misdiagnosed as it is potentially curable. Although these diagnostic procedures are indicated for only a small subset of patients with headache, some patients with a diagnosis of migraine might actually have a spinal CSF leak causing their headache rather than migraine. Given this possibility, if you have intractable and intense migraine-like headaches that improve when lying down, it is certainly worth learning more about and discussing this approach with your migraine specialist.
There are some great resources on the topic to explore:
- The Spinal CSF Leak Foundation provides online education, support, and a wealth of information.
- The Cedars-Sinai CSF Leak Program has a similarly helpful online resource with educational information for potential patients.
- National Organization for Rare Disorders provides an easy to understand overview of the condition.
How much has your migraine disease changed or evolved over time?