Jeff suffered from textbook symptoms of intracranial hypotension with zero results on imaging. But after extensive testing, the reason for his symptoms was finally discovered.
Jeff was 44 when his headache first began. It came on in the early evening, a dull ache in the front of his head that became increasingly intense as the night went on. When he woke up the next morning, the headache was gone, and he was relieved—but by the afternoon, it was back, the sensation of pressure in his forehead, even his eyebrows sensitive to the slightest touch. This headache pain was the strangest thing he’d ever felt.
As the daily afternoon headaches continued, he noticed they were starting to become centered on the back of his head and seemed to improve when he was lying down. Jeff made an appointment with his general practitioner, who diagnosed him with “tension headaches.” This didn’t sit right with Jeff. Yes, he was a busy actor; yes, he was running a new production company; yes, he was stressed out; but these headaches seemed more urgent than that.
Jeff saw a second doctor, who ordered a brain MRI. It came back normal, with no signs of what might be causing his headaches. Jeff then went to a neurologist, who prescribed him strong migraine meds, to no avail. After six months of living with these strange headaches, Jeff was spending most of his time flat, to cope with the pain. He noticed that during the brief times when he was upright, bending over provoked his head pain to the point of almost vomiting, and it was this symptom that prompted him to search online. He discovered an article about a doctor who had experienced symptoms similar to Jeff’s. In fact, this doctor’s symptoms read to Jeff as though he were reading a list of his own symptoms. The article ultimately explained that the doctor’s headaches were caused by intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak. Jeff was so excited, he couldn’t sleep. This had to be the answer to the question of what was causing his headaches.
He went back to his neurologist and explained what he’d discovered, but his hopes for a quick diagnosis were dashed: the neurologist told Jeff it was highly unlikely that he was suffering from any kind of CSF leak. The doctor suggested Jeff have a lumbar puncture (spinal tap) to test the pressure of his cerebrospinal fluid. This test, too, came back normal.
Jeff was disappointed, but he knew he was on to something. He contacted a spinal CSF leak specialist he’d found online, asking the doctor what he would recommend for someone in his situation. The specialist responded almost immediately, suggesting that Jeff have a full MRI of his brain and spine. Jeff had the test done. Again, heartbreakingly, it came back normal. Jeff continued to manage his headache with caffeine, iced coffee, over-the-counter pain medication, and staying flat. The specialist scheduled him for another test, a type of spinal imaging called a digital subtraction myelogram (DSM). As with all the others, the test came back normal. No signs of a leak.
At this point, Jeff was so discouraged he began to doubt, thinking maybe he didn’t have a leak after all. He had had a medical issue years before that had been difficult to figure out, and the experience of searching for this leak and not finding it was giving him flashbacks to that other time in his life when he had been a question mark to all the doctors he saw. He wanted to give up, and he worried his doctor might give up; but in addition to all the disappointment, discouragement, and fear, he had a new pressing issue: he and his girlfriend Susan had just learned that Susan was pregnant. He needed to find an answer.
Finally, he shared his fears with the specialist, who assured Jeff that he would not give up on him, and recommended that Jeff have an epidural blood patch. Blood patches are often the suggested treatment for patients with intracranial hypotension, and some patients experience relief from their symptoms. For others, the relief is short-lived. For Jeff, the blood patch offered only minimal relief, but the specialist took this small improvement as supportive evidence that Jeff did have a leak. He recommended Jeff have one more test, another DSM, this one performed while Jeff was lying on his side.
This time, they found the leak.
In fact, what they discovered was that Jeff’s leak was a CSF-venous fistula. A CSF-venous fistula is an abnormal channel between the space where cerebrospinal fluid is and a vein outside the dura mater. What this meant was that instead of cerebrospinal fluid being circulated within the dura mater, Jeff’s cerebrospinal fluid was being siphoned off through a vein. CSF-venous fistulae can be hard to visualize on spinal imaging, which is why it was so important for Jeff’s doctor to have pursued additional testing via digital subtraction myelogram.
Jeff was overjoyed: finally, he had proof of what was wrong, and, more importantly, he was told, it could be fixed. Jeff was scheduled for surgery right away, and the operation was a success. Afterward, Jeff experienced minor rebound intracranial hypertension, a different kind of headache experienced by many patients after epidural blood patching or surgery, but by six weeks he felt almost fully recovered. In fact, by the time his son was born in May 2019, Jeff was well enough to fly to Australia for a two-week “babymoon.”
Now, over a year after his surgery, Jeff characterizes the 18 months he spent living with his spinal CSF leak as a kind of dream. He considers himself an evangelist for education about the disorder, and marvels daily that he is able to be fully present and engaged as a partner and a father, just the way he had hoped. He wants people to know that it’s important to not give up hope, and above all to be persistent.
Our team at Spinal CSF Leak Foundation wishes to extend our appreciation of all those physicians working so hard to help patients, as well as those who assisted with this feature story.