Melissa's Story

After years of symptoms, I finally had surgery for the internal jugular compression that led to intracranial hypertension that led to spinal CSF leaks that led to POTS. It seems to have fixed basically everything that was ever wrong with me. Here's my story.

I was healthy and active my whole life, with only vague signs of an intracranial pressure issue--catching mystery bugs after air travel, sleeping hard after crying, not being able to stand in place for hours or sit still on bleachers, that one time my vision blacked out running high school track. They were easily dismissible as one-off incidents (and doctors always did dismiss them).

Everyone has intracranial pressure, just like blood pressure.

But mine was a little high. I was not especially bendy but did have a mild abnormal curve to my spine (a lot of people do) and some family members were a little hypermobile (a lot of people are). These would both be contributing factors. 

In 2018, I slipped sock-footed on stairs and went down with my dog, letters for the mail carrier, and a mug of hot tea. I landed on the edge of the bottom step with my lower back. (The dog was fine. The emergency room doctor told me I was, too.)

It's likely what started a steep decline.

The following year a lot of events stacked up on me. Both my parents were diagnosed with cancer. I moved house to become their caregiver (so much lifting). I replaced the flooring. Had abdominal surgery. An infection that required antibiotics. My job was high stress.

These events, which happen to all of us, can each raise intracranial pressure. I did not know at the time that my left side internal jugular—the vein responsible for outflow in the brain—was underdeveloped (again, not unusual) or that the curvature in spine had caused my C1 to slide forward to choke off the other vein, or that any of the other anatomical issues were causing a problem.

Any doctor will tell you they usually don’t.

But my situation wasn’t usual. I put too much stress on the system. So when I jumped down from the bed my truck after bending and loading heavy items a year later (again, a pressure trigger), that system gave out. 

I felt a strange jolt through my spine.

The next afternoon someone asked me where I worked and … I just couldn’t reach the answer. Like it was forgetting where I laid my keys.

I did not feel especially horrible the instant it happened, likely because a leak would have relieved the too-high pressure on my system.

I did not get the classic leak head pain. In fact, I might never have known. Leaks can happen with any pressure event, even something as minor as a sneeze. Head pressure also raises during illness, which could be important in conditions with illness-onset (many chronic illnesses begin with a virus or inflammation).

I had brain fog. Fatigue. Vague feelings something was off. I went to the doctor; he shrugged. Things worsened over the following days, months, years. New symptoms all the time. I learned the word comorbidity. Got told I was probably just depressed.

Covid happened. Everyone was sick. Medical care got harder.

I ended up bedbound, the picture of very severe ME/CFS. My chief symptoms initially had been fatigue and cognitive issues, and strange ever-changing symptoms and neurological weirdness that felt very hard to pin down. I did not fit the profile, but 17 doctors and 3.5 years in, I found a possible answer online:

Spinal CSF Leaks without the classic orthostatic headache. 

My MRI was normal; my opening pressure was normal. Most doctors would stop there. But we had dragged my body to a leak specialist who understood the condition.

A CT Myelogram found several large lumbar area leaks with more higher up. Leaking for a long period had visibly worsened my connective tissue and pushing through with exercise, physical therapy, and the wrong medical treatments had made everything worse. One leak had become many. 

My leak symptoms looked like this:

• Cognitive issues (memory, attention, complicated thinking) 
• Exercise intolerance/post exertional malaise
• Orthostatic intolerance (worsening symptoms when upright)
• Severe weakness and fatigue in limbs  
• Pain (teeth, face, head, neck, shoulders, coat hanger, back, limbs, ribs, feet) 
• Tinnitus (ears ringing), pulsatile tinnitus (whooshing in ears)
• Light, sound, scent, temperature, and movement intolerance
• Vision issues
• Pallor, skin changes
• Nausea, low appetite, food sensitivities, gag reflex, gut motility issues
• Sleep (initially sleeping up to 20 hours per day, later only 4 hours per day)
• Pressure behind eyes and in head and neck
• POTS
• Plus an ever-changing variety of neurological weirdness, gait changes, random puffiness, nerve sensations, chills, low fevers, mood changes, watery nose, irritated throat, auditory processing issues, problems reading blocks of text or focusing, tingling in face and limbs, problems communicating, and more.

Leak testing is specialized. It is critical to find a doctor knowledgeable in spinal CSF leaks. 

Mine were patched. My symptoms gone. I could walk again. Life was great.

But I was on a medication to lower my intracranial pressure, which is sometimes necessary post-patch. Restrictions after the procedure include taking it easy, with no bending, lifting, or twisting--the exact triggers for high pressure events.

I hadn't realized the leaks had worsened the underlying high pressure condition (intracranial hypertension/IH). 

Once my system was back under stress, it became clear. 

It cannot be emphasized enough how both leaks and high pressure look similar to one another and identical to other chronic conditions. 

I only knew because of * the ordeal. * Because none of the treatments offered me a cure. Maybe I was in denial. But had I accepted the many incorrect diagnoses of many otherwise adequate doctors, I would still be in bed.

What's more, high intracranial pressure is a symptom driver in many other chronic conditions. Most people are never told how to recognize or manage those symptoms, or offered treatment of any kind.

My life had changed for the better. When my pressure found equilibrium, I had no symptoms at all. But life does not allow one to live stress free, physically or otherwise.

I still needed help.

My leak testing had revealed vascular compressions, yet every neurologist refused to look further into the possible cause for the IH they were treating. Even the more commonly acknowledged transverse sinus stenosis was dismissed. 

I had to find new specialists. It took 3.5 more years. 

My surgery was a success, but many others with these conditions have a long list of comorbidities. EDS, pelvic congestion, spine instability, and more.

Here’s what the symptoms of increased intracranial pressure looked like for me:

• Fatigue
• Sensitivity to light, sound, and smells
• POTS (heartrate, heat intolerance, sob, nausea, etc.)
• Vision changes
• Motion intolerance
• Face, tooth, jaw, neck, shoulder, and back pain, and head and neck pressure.
• Tinnitus, pulsatile tinnitus, sensation of thumping and fluttering in neck.
• Brain fog, word finding, memory, concentration, alertness, reaction speed, ability to communicate or have longer conversations. 
• Exercise intolerance, occasional dizziness, eye pressure, nausea, and more.

There were others, including the poking sensation at my throat (Eagle Syndrome) and constant coat hanger pain. 

Importantly, decompression surgery relieved symptoms that are not commonly associated with vascular compressions.

I had hiccups for more than 5 years.

They are now gone. There are many structures in that area of the neck, including lymph nodes (which can swell during illness), and it is threaded with vasculature and nerves. It takes a surgeon who understands the condition--what symptoms each of those structures can cause--and knows how to not make things worse.

Not everyone who has compressions or IH needs surgery. Most can manage with lifestyle changes or medication. But knowing what’s happening makes all the difference in treatment and symptom management. Misdiagnosis can make the situation so much worse.

The science is changing quickly, revealing many of the common assumptions and testing methods are wrong. 

To clear up a few of the myths and misconceptions:

• Spinal CSF leaks do not always present with a headache. Neither does intracranial hypertension. (Mine did not.)
• It's possible to have both conditions at the same time: low volume (spinal CSF leaks) and high pressure (IH). (I did.)
• Opening pressures are not reliable. (Mine was not.)
• POTS can be caused by spinal CSF leaks. (Mine was.)
• A normal MRI does not rule out a spinal CSF leak. (Mine was read as normal.)
• The orthostatic aspect of leak symptoms can change or disappear over time. (Mine did.)
• Lack of papilledema does not rule out IH. (I did not have optic nerve involvement.)
• Weight is not the only cause of IH--losing weight does not always improve symptoms. (I have always been thin.)
• You do not have to have a connective tissue disorder to have leaks or IH. (I only had hypermobility in my hips and neck.)
• Leaks are not always self-limiting (they don't always resolve on their own). This is critical, given that many spinal CSF leaks are caused by lumbar puncture, epidural, or surgeries. (Non-cutting needles like Whitacre can reduce postdural puncture headache by up to 80%.)
• Intracranial hypertension is on the increase and IH can contribute to leaks.

It is honestly stressful how much outdated info is out there. Spinal CSF leaks are misdiagnosed at rates over 90% and many related illnesses, some made by diagnosis of exclusion, dismiss leaks as a possibility without proper evaluation.   

Migraine, headache disorders, sinus conditions, POTS, Chiari, dementia, movement disorders, post-concussion or whiplash conditions, depression, anxiety, the list of misdiagnoses is long. Intracranial pressure can also be a factor in ME/CFS, fibromyalgia, long Covid, sleep apnea, PCOS, inflammatory or autoimmune diseases, and more.

If you or a loved one experience any of these conditions, I hope you'll look into leaks and IH. And please remember these conditions are not well known. Most doctors are inadequate or dismissive when it comes leak testing and treatment and while many neurologists do treat IH, care is frequently lacking or biased.

No one should have to go through this to get care. My aim is to raise awareness in the hopes of preventing the same thing happening to others.

Links, research, and resources can be found here.