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7 People with Chiari and CCI Share Their Stories

To complete our series on Chiari malformation and Craniocervical Instability, I wanted to share more of the interviews that I didn’t have the opportunity to use during my exploration of the Issues of Diagnosis and Treatment. These interviews are great supplemental material to that discussion and help to see the problem from the perspective of those who have the condition. We wrap it up with my own information, as representation of what I suspect are hundreds of thousands of people suffering from undiagnosed forms of craniocervical junction disorders and why I believe I have one. While this group is by no means insignificant, ultimately none of us will know what affects us until finally put through proper testing, as described in this post.

7 people share their journey with CCI, or Chiari Malformation, from diagnosis and treatment, to symptoms and barriers to care. A special Zebra Pit series in honor of Chiari Awareness Month

This is the third post in our Chiari Awareness Month Series: The first two are available here:

Out of respect for the privacy of participants, I am only publishing their first names and general location. Some people preferred to use a pseudonym, which is denoted with an asterisk (*) next to the participant’s name.

Name: Jackie

Location: Sacramento, CA

Time from onset of symptoms to diagnosis: 25-30 years

Jackie underwent posterior fossa decompression with craniotomy, C-1 laminectomy and duraplasty in November, 2017. Prior to surgery, Jackie suffered severe head pain, persistent weakness in her right arm, and by 2015, extreme dizziness, lightheadness and near syncope that was caused by orthostatic hypotension that had no other cause. It was believed that Jackie’s Chiari malformation which was classified as Chiari 1.5, was congenital in nature, due to the small size of her posterior fossa, which caused everything to get pushed forward. When asked if she considered the surgery a success, she noted “For me the procedure was extremely successful. It eliminated my neck pain and chiari headaches completely. It also completely cured my chronic hiccups and motion sickness (which I wasn’t expecting). The only thing that remains is the orthostatic hypotension which is less severe. I manage that with increased salt intake only.”

Despite the successful outcome of Jackie’s surgery, achieving it was a bit of a feat. “My primary care doctor sent me to a neurologist who ordered the MRI that revealed the Chiari. The neurologist completely blew it off, insisting it couldn’t possibly be causing my symptoms. He thought my problem was “vestibular migraine.” Even though my headaches never completely fit the migraine profile, that’s what doctors assumed I had. I had to keep trying to find the cause of my dizziness, and it took until September 2016 to pinpoint orthostatic hypotension. It was tough navigating the different workups. Once I was able to get two neurosurgery opinions, things got much easier. I was pretty lucky in that I had good insurance (a plus in the United States) and I had access to a brilliant neurosurgeon at Stanford University.”

Jackie’s case demonstrates how even when Chiari is clear cut and easy to diagnose; the conclusions of doctors who aren’t really qualified to make a determination about intervention, but who do anyway can be a huge obstacle to intervention and treatment of Chiari. Lucky for Jackie, she didn’t let his ill-advised treatment plan (to do nothing and assume her problem was something else) stop her from seeking treatment elsewhere. I also feel it’s important to note that Jackie’s doctors were smart enough to evaluate her for the presence of hypermobility before making a final determination on how to treat her.

Name: Christina

Location: South East England

Time of Symptom Onset to Diagnosis: Unsure.

Christina has EDS related Chiari malformation. It took Christina 30 years to get a diagnosis of EDS. She had an upright MRI because of awful pressure headaches. A horizontal MRI missed the presence of a malformation, but she persisted and was given an Upright MRI at the Medserena in London, which finally confirmed the diagnosis. To date, Christina hasn’t had any procedures. For her Chiari, if symptoms continue to progress, she will need surgery: However, Christina’s chiari malformation and symptoms are complicated by Craniocervical Instability. The procedure which she needs, a spinal fusion, isn’t available through UK’s National Health Service, forcing Christina into the position of raising funds to so she can have both surgeries with a doctor in Spain.

Name: Maura

Location: Newark, DE

Time from onset of symptoms to diagnosis: unknown

 Maura was diagnosed with Chiari I in 2007 at age 6 via a traditional horizontal MRI. “I had a decompression shortly after diagnosis and had significant relief for 8 months. After that symptoms returned but slightly differently, so I had a full decompression. Turns out it was craniocervical instability that caused the symptoms to return after the first decompression so the second decompression was ineffective.” Despite this setback, today Maura’s only residual symptoms of CCI and Chiari is that she struggles with fine motor coordination. In additiona to chiari I and CCI, Maura lives with hypermobile Ehlers-Danlos Syndrome, Gastroparesis and POTS.

In Maura’s case, had she been evaluated for the presence of hypermobility like Jackie was, it’s conceivable she would have been able to avoid two separate surgeries. It is unlikely the condition developed in such a short span as eight months.

Name: Kaitlyn

Location: Worthington, KY

Time of Symptom Onset to Diagnosis: Unsure. Kaitlyn had symptoms from early childhood that were ignored due to similar familial symptoms until an injury in high school revealed her condition.

Kaitlyn was diagnosed in July of 2006 with Arnold Chiari Malformation type 1 via traditional horizontal MRI following her cheerleading accident. Treatment for Kaitlyn came quickly, being performed in a matter of months after diagnosis. She had posterior fossa decompression surgery for a 2.2cm herniation. During the surgery they performed a laminectomy of her C1 to create more space in the spinal canal, opened the dura, cauterized some of her cerebellar tonsils and inserted a stent for cerebrospinal fluid drainage.

Kaitlyn considers her surgery successful and has no plans for further intervention: However a number of her pre-operative symptoms remain. “I have debilitating [hemiplegic] migraines that replicate stroke symptoms, neck stiffness, neck pain, trouble swallowing and my head shakes.”

Kaitlyn notes location and lack of knowledge to be the biggest obstacle in her ongoing care. “I live in a small town in Kentucky and the doctors around here have little to no knowledge about Chiari Malformation. There have been doctors [who] Google ‘Chiari malformation’ in front of me and then act like experts. Recently I have traveled to Cleveland Clinic and Johns Hopkins to meet with doctors who were knowledgeable about Chiari Malformation. Being able to meet with a doctor who knows about Chiari Malformation has been the hardest part of this journey.”

Name: Martha

Location: Kent, England, UK

Time of Symptom Onset to Diagnosis: None

Martha’s Chiari I malformation diagnosis for Chiari was incidental. At the time, she wasn’t experiencing any symptoms. That was in 2012, but lately she’s found things have changed. “I very recently have developed tingling in my lower jaw/around my mouth, chattering of the teeth, TMJ type pain and severe headache mostly on the right side.” Martha is currently waiting for another neurology consultation to discuss her new symptoms and next steps. During another MRI for her symptoms in 2017, they diagnosed her with possible facet joint compression due to posture, which has been helped by regular physiotherapy. In addition to Chiari, Martha has Ehlers-Danlos Syndrome, Fibromyalgia, hypothyroidism and lipedema.

Name: Jennifer

Location: Fairhope, AL

Time of Symptom Onset to Diagnosis: 15 months

Jennifer has Chiari I malformation which was discovered through MRI, but she couldn’t recall which kind. In addition to Chiari, she has pseudotumor cerebri. Jennifer had posterior fossa decompression, which failed. Multiple procedures, from nerve blocks to a spinal stimulator were attempted in an effort to reduce her pain, but none were sufficient. Her final surgery in January ’18 entailed a fusion of C1-3 at Johns Hopkins.

 Today, Jennifer regrets all these attempts. “If I had to go back, [I] would not have any of the surgeries or procedures. The 8 years that I was sick was nothing but obstacles. It was a nightmare. I almost took my life last Halloween, because that final surgery just about broke me. We have struggled financially. It’s been hell for my children, and it destroyed my marriage. I’m now a single mom and rebuilding my life from nothing.”

7 people share their journey with CCI, or Chiari Malformation, from diagnosis and treatment, to symptoms and barriers to care. A special Zebra Pit series in honor of Chiari Awareness Month

Since Jennifer has given up on surgical intervention, she lives a different sort of life now.

“When I walked away from doctors and pharma last year, I really, really did. Makes me feel icky to even look at medical stuff. None of the surgeries or procedures helped me. They just made me worse. I got off of pharma and trained myself to process pain in healthy ways. Found the app, Curable, super helpful, and I started CBD oil, which began healing the damage to my cerebral cortex.

“My pain level varies with the weather, and today it’s kicking my ass. In another month or two, I may have a totally different story. I’m currently in spasm and probably functioning at 6-7… have to slow down to a snail’s pace during the summer… and that’s very hard on me mentally, because I often feel like I missed out on 8 years and don’t want to waste time.

“I’m in as much if not more pain, now, compared to 2013, but I don’t process pain the same. I’ve dealt with the emotions that came along with pain… guilt, fear, disappointment, shame, etc. So, now pain is just pain. All humans function in some type of pain, whether physical or emotional. It’s up to us to find a healthy way to walk through it.”

Name: Rose*

Location: Chicago, IL

Time of Symptom Onset to Diagnosis: Unsure.

Rose has a somewhat unusual story in that she was first diagnosed with Chiari at birth, but while the surgeon discussed her hydrocephalus and the intervening shunt they would place, he failed to report and explain that she also had a Chiari malformation.  When she was diagnosed in 2004 with Chiari, it came as a total surprise to her. It wasn’t until after that Rose discovered the paperwork in her parents’ records, which briefly mentioned the diagnosis.

The onset of Rose’s symptoms are hard to determine due to a cascade  of health problems that probably coincided with onset. “I started having problems that I thought were allergies about 9 months before. Then in September I had a shunt failure. I was officially diagnosed June of 04 through the use of traditional head/ abdominal CT, MRI and x-rays. There was still concern that the shunt was failing again. Decompression was done June of 2004, after 3 shunt revisions had been performed.

“[I] haven’t needed any other surgeries for that. My symptoms after surgery were stable, despite a six month recovery.” In addition to Chiari, Rose was diagnosed with Ehlers-Danlos Syndrome in 2010 and developed POTS a few years after her Chiari treatment, so while she’s had favorable outcomes to her chiari, she still struggles with many other symptoms.

Name: Michelle

Location: Cincinnati, Ohio

Time of Symptom Onset to Diagnosis: 10 Years and Counting

I believe my own story belongs here like that of many others. While I do not have a diagnosis of Chiari or CCI, I have symptoms consistent with CCI. My earliest started in my early 20’s, when I started passing out every time I tried to extend my neck backwards to look up. My symptoms have compounded over time and I struggled the most over the last ten years with the following symptoms, which have fluctuated over time; severe head and neck pain, tremors, feeling like my head was simply going to drop off, occasional speech impairment, significant impairment of cognition and memory, weakness in my arms, dizziness, difficulty with my fine motor skills, tinnitus, insomnia and depression. I have gone through jags of vomiting for days to entire weeks due to my head pain and go through periods where I get hiccups several times in a single day.

As I explained in this post, like many of the men and women who are absent diagnosis despite such strong symptomology, I do not have a diagnosis because my malformation doesn’t become apparent until I am in the upright position or my neck is extended and flexed and the only testing available to me is a traditional MRI. Despite my request for an upright dynamic MRI or flexion and extension MRI, I have been told by multiple neurologists that “upright MRIs don’t work” or “I have no idea how to order that test,” or “let’s see what we can do about this in other ways,” which usually entails failed drugs with side effects too great to bear, attempts at nerve blocks, and other unsavory options like botox.

7 people share their journey with CCI, or Chiari Malformation, from diagnosis and treatment, to symptoms and barriers to care. A special Zebra Pit series in honor of Chiari Awareness Month

Another EDSer in my hometown had to go to New Jersey to be treated by a surgeon who first ordered and read her MRIs through email for a fee. Without doing so she would still be living with her symptoms and yet we both live and go to the best teaching hospital in the area, where one would hope the doctors would be aware of the most cutting edge techniques.

Recently, I had a lumbar puncture to relieve the pressure my CSF was causing. At this time, the source of my increasing pressure is considered “idiopathic” or of unknown origin, but I feel strongly that there is a cause that could be easily determined with the proper test. Removing some CSF fluid helped initially with my tinnitus, light sensitivity and positional headaches, but they have returned in just a matter of a few weeks and I feel it is likely the next steps will be a shunt.

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My symptoms have fluctuated over the years and I feel I’ve made good headway in shoring up my neck through physiotherapy and excessive rest. Still, my neck occasionally slides out of joint and that familiar, unbearable pain returns. One of my uppermost vertebrae now has so much play in it, I can move it freely with only a gentle push, but for whatever reason, it hurts me much less and I have greater range of motion than I used to. Still, my head pain persists and I have to take daily abortive medications for migraine and limit the amount of time I spend in upright positioning. Even sitting in my recliner can cause a nasty headache that’s impossible to relieve without lying flat. 

While I want to know if Chiari and/or CCI are the cause of my misery, I am not at all sure I would agree to surgery, so I haven’t contacted an expert outside of my area for consultation. I may yet, depending on the evolution of my symptoms.

During the course of my interviews, I had no less than a dozen people come forward with similar stories and I see others on my friend’s lists, among my followers and in my support groups who suffer with these symptoms and also suspect strongly the cause to be the same. Like me, these people have neither the means nor the access to do the proper testing. My belief is that Chiari and CCI contribute heavily to the missing millions of ME/CFS and is largely occupied by undiagnosed people affected by EDS. I feel certain one of these conditions is indeed responsible for the years I spend in bed, barely able to function and fear regression. Though I treat my symptoms well through the use of diet, exercise and supplementation, my life is greatly affected by this condition, which I’ve come to privately term “the condition that shall not be named.”

Have your own story to tell? Please add it in the comments. This story is far from over and the more patient voices who get heard the better.

As always, thanks for reading. I hope the stories of people with Chiari and CCI have helped you to understand firsthand what it’s like to live with these rare conditions. If you’d like to know more about these conditions, please take a look at the other posts in this series meant to bring awareness to the issues of Chiari, CCI and craniocervical junction disorders overall.

For More Patient Perspectives on Chiari and CCI, check out these posts:

Resources:

All interviews were conducted through internet correspondence from September 5-13, 2019.

An eighth story is also included, one of a person who can't achieve diagnosis for clear cut symptoms of a craniocervical junction disorder and EDS, myself. Learn what factors prevent diagnosis and how people with Chiari and CCI live before and after surgical intervention of their condition.

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Lumbar Puncture

I recently underwent an exploratory lumbar puncture, and I wanted to share my experiences and talk about why my neurologist and I opted for this somewhat risky procedure. I’ll talk about some of the reasons to undergo exploratory lumbar punctures and how they are performed. I include the guidelines and procedures both you and your doctor will likely be following before during and after the procedure. This way you’ll be prepared and cool as a cucumber the day you or your loved one undergoes this procedure, also known as a spinal tap.

As a sufferer of intractable migraine for over a decade, this is a procedure I have long wanted performed, but been equally afraid to undergo, so I never pushed for it with my old neurologists, who didn’t seem to be taking it at all seriously. It took me a long time to decide the procedure was for me. After finally getting my migraines under control just this past year and realizing it did nothing for the terrible pressure in my head, it had me wondering if more needed to be done than just finding the right migraine medication(s). This ever growing pressure, which became almost unbearable this summer, was accompanied by sharp pain in my head that was positional, intermittent vision and hearing loss, non-stop tinnitus, and a strong pulse in my ears that had the power to turn my tinnitus into the never ending *beeeeep beeeeep beeeeep* of an alarm clock; and the on/off switch seemed to be connected to standing or straining.

Then I had the worst dystonic attack I’ve ever had and things only got worse from there. That’s when my head pain specialist and I knew it was finally time. If I’m being honest, it was probably way past time. You can read more about it here: Why Vacations Don’t Exist for People with MCAS.

Enough about me. Let’s learn more about CSF, lumbar puncture and find out what to expect if you need to undergo the procedure.

The Role of Cerebrospinal Fluid

When my doctor started talking about performing a lumbar puncture for signs of high pressure known as intracranial hypertension, I did what I always do, research everything I could find on the procedure, from what to expect during and after the procedure, what type of conditions can be diagnosed using LP and what exactly cerebrospinal fluid is, along with how its analysis can be interpreted. Join me in learning more about this procedure now.

Cerebrospinal fluid is defined as a “clear watery fluid which fills the space between the arachnoid membrane and the pia mater.” Essentially, it’s the liquid that fills your spinal canal and surrounds your brain and spine. CSF serves several important functions in the central nervous system. CSF cushions the brain and spinal cord against shock. It provides a barrier. It helps to maintain pressure at a constant level inside the skull. Finally, CSF delivers nutrients to the brain and carries waste products away into the bloodstream. CSF is protected and separated from our blood by the blood-brain barrier. There is about 100-150ml of CSF in the normal adult human body. It is primarily made up of water and is alkaline. CSF is continually produced, predominately by the brain’s ventricles, and is entirely replaced every 6 to 8 hours.

An increase in the volume of blood or brain tissue results in a corresponding decrease in the fluid. Conversely, if there is a decrease in the volume of matter within the cranium, as occurs in atrophy of the brain, the CSF compensates with an increase in volume.

Cerebrospinal Fluid-Britannica Science

What is a Lumbar Puncture?

A lumbar puncture (LP) is often performed to check for the presence of bacterial, viral and fungal infections, excessive intracranial pressure, a cerebrospinal fluid (CSF) leak, cancers of the brain or spine, subarachnoid hemorrhage (bleeding on the brain), or to aid in the diagnosis of other neurological conditions such as Guillain-Barre syndrome and multiple sclerosis or a mitochondrial disease. LPs can also be performed for injection of chemotherapy or other medications into the spinal canal, as directed by your physician. They can be performed on an emergency basis, such as in the case of injury, or be prearranged, which is often the case with non-emergent exploratory procedures.

A physician usually performs the procedure, however even a well trained nurse practitioner can administer a spinal tap. My own procedure was performed by a nurse practitioner in training who was supervised by a neurologist; he did a fantastic job.

To perform a lumbar puncture, a thin, hollow needle is inserted into the spinal canal. If pressure is a concern, then your neurologist will request a reading of opening and closing pressures. Mine was 14.5 to begin, a middle of the range number that could be too high for some and just right for others. My closing pressures were a 9.5, after they had removed two small vials of CSF to be sent to the lab for testing.

Is Lumbar Puncture Safe?

Spinal taps have a reputation for being a high risk procedure, likely due to early failures during the development of the procedure back in late 19th century. This was in part due to the lack of imaging at the time. Today the procedure is much safer, with the advent of imaging technology, prospective and retrospective study have informed the development of clinical features that are associated with low risks and complications.

In trained hands, Lumbar Puncture is a straightforward procedure with few complications. According to The Health and Social Care Information Centre for England, there were 55,427 episodes of hospital care that included a diagnostic Lumbar Puncture in 2011-12, 0.53% of all hospital consultant episodes, which in Northern Ireland’s health system, with 600,000 admissions annually, would equate to about 8 diagnostic Lumbar Punctures per day.

“Diagnostic Lumbar Procedure,” NBCI

Preparing for a Lumbar Puncture

Before administering a lumbar puncture for your symptoms, your doctor may perform additional testing. These can include MRI, CT and blood tests which check for your ability to clot, as well as your kidney function.

Prior to having a LP, your medications will be reviewed, along with any recent illness, medical conditions or allergies. If you think you may be pregnant, you also need to alert your team right away so the necessary precautions can be taken. You may be asked to discontinue the use of any drugs which may thin the blood, from NSAIDs (advil, aleve, toradol, etc.) to heart medications such as Warfarin (Coumadin®), Pradaxa®, Heparin®, Lovenox®, or clopidogrel (Plavix®). Generally speaking, you can expect to be off of these medications anywhere from 3-7 days prior to your procedure to ensure good post-operative clotting of the needle puncture.

In addition to reviewing any and all medications taken, including supplements, any allergies also need to be discussed and you should provide a complete list to your surgical team so they can help to ensure your safety. This was a long list for me and my surgical team went so far and to cover every substance I would come into contact with the day of my surgery and again, just before their use to ensure no mistakes were made. I found this incredibly reassuring and would love to see more surgeons employ these tactics.

On the day of the procedure, you will be asked to refrain from eating and drinking for a period of time prior to your procedure. If only a local is used, as was the case for me, you can eat up to two hours prior to your procedure. If twilight sleep or sedation is employed, you may need to discontinue foods by midnight on the night before your procedure.

Finally, you will need to be accompanied by an adult who can drive you home and stay with you for at least 24 hours after your procedure. You may be given a gown for your procedure or your clothing may be draped in order to protect it and create a sterile field.

If the procedure is to be performed on a child, parents may be allowed to accompany them during the procedure.

How a Spinal Tap is Performed

Image by David Mark from Pixabay

Most scheduled LPs are out patient procedures and you can usually go home within 30-60 minutes of completion. A LP is performed with the use of fluoroscopy. In fluoroscopy, an x-ray is taken and then converted into a digital image your physician can see on screen. This helps them to be very precise when placing the needle in the spinal canal, reducing the danger of injury to the spinal cord.

At the beginning of the procedure, an IV may be placed in the arm or hand to administer the sedative, if one is given. You will likely be asked to lay on your stomach and may be asked to position your arms above your head with your face to the doctor so you can easily communicate. If you have difficulty with this position due to spine issues or other disability, ask the team to help you get into a position you’re comfortable in. It is very important to be able to lay still during the procedure, so you must be comfortable.

Once in position, the patient will be draped with sterile cloth and the surgical field will be sterilized with a preparatory solution, such as Betadine. A local anesthetic will be administered, which may feel like a pinch or sting.

The x-ray is taken and appears on the fluoroscopy monitor to guide your doctor. Once she or he has located the correct spot, they will begin the insertion of the needle and may occasionally take other x-rays to determine the depth of the needle is correct. There are several layers to get through, and while you shouldn’t feel pain, you may feel the needle access each layer of tissue and feel a pop as it goes through the final layer. It’s best to keep this in mind, so you remain calm and still despite these strange sensations. According to Doherty and Forbes:

The Lumbar Puncture needle pierces in order: skin, subcutaneous tissue, supraspinous ligament, interspinous ligament, ligamentum flavum, epidural space containing the internal vertebral venous plexus, dura, arachnoid, and finally the subarachnoid space.

Consent should include the risk of Post-Lumbar Puncture Headache (PLPH), which has a published incidence of 32%. Other risks to discuss include failure to obtain CSF, localised bruising, bleeding and local discomfort at the injection site. Iatrogenic meningitis and nerve root injury are exceptionally rare.

“Diagnostic Lumbar Procedure,” NBCI

This is important, not only because you can feel the needle break through some of these layers, but also because the puncture of ligaments can cause some soreness and back pain in the days following the procedure and people with collagen disorders should probably consider the need for extra healing time due to our deficiency and slow healing times.

Once the needle is inserted, you may be helped to reposition yourself to your side, taking the fetal position. The fetal position is the proper position for which spinal taps are usually performed when entering from the lumbar region of the spine.

If opening pressures are requested, as in the event of suspected CSF leak or IIH, the doctor will call out these numbers to the nurse for documentation and may or may not be commented upon. Then the appropriate amount of CSF fluid will be removed for testing.

During the procedure, you will be asked to monitor how you feel and whether or not your symptoms are changing as your fluid is collected. I didn’t feel any real difference during the procedure, however by the time I left the facility 30 minutes later, my headache was completely resolved, I was no longer light sensitive and my vision cleared as if the ward I was on had gone from dismal and dreary to full sun; a sun I could now look toward without intense pain. This indicated to the team that my pressures were indeed too high for my particular physiology. Once an adequate amount is removed for testing, the needle is removed, a band-aid is applied and you are asked to lay on your back to allow gravity to help with the work of closing the wound.

Post Procedure Expectations

Your team will likely check on you a couple of times while you recover in post-op. They will ask you questions about your symptoms and ensure everything is fine before sending you home. While it is important that you lay flat in your bed for a period after your procedure (usually 24 hours), you can simply tip the seat back in your car for the ride home.

Be sure to follow the recommendations of your care team to facilitate healing and avoid infection, which could be very serious. For this reason, it’s also very important to keep your team informed about any new symptoms or changes you experience after your procedure, both to ensure your safety should complications arise and in the event that further testing or procedures are indicated to take care of any problems which may arise.

It is important that the patient lay flat both to facilitate healing and to help avoid a spinal headache, which can happen if too much CSF is removed or continues to leak. Each day beyond the 24 hour period, you should attempt to be up and about. If no headache occurs, you’re generally free to go on about your life, with the exception of swimming and bathing (showering is okay). However if a spinal headache begins, the only solution is to lay in bed for another day. This process is repeated over the next two days. If on the 4th day you rise and experience a headache, a blood patch may be necessary.

A blood patch requires a second lumbar puncture in which your own blood is used to bring the pressure back up and to help facilitate clotting.

Barring any initial complications which might indicate the need for a blood patch, some patients may experience a spike in intracranial pressure within a few weeks after the procedure which would indicate an overproduction of CSF. In this case, another lumbar puncture will be performed to bring the pressure back down and a stent may be inserted to maintain drainage.

CSF Testing and Analysis

There are many tests that can be performed on CSF. Which tests your doctor orders depend largely on your symptoms and the outcomes of other diagnostic testing. Tests range include the observation of its physical characteristics (pressure, color, viscosity, turbidity), chemical tests to analyze the composition and content of fluid (immunoglobulins, proteins, LD, CRP and others), microscopic examination of red blood cells, white blood cells and cytology, and infectious and parasitic tests. For a complete list with detail, go here.

My CSF Analysis:

Because my doctor and I were most concerned about intracranial hypertension due to my specific symptoms, most of my tests were directed at this problem, with a few extras to rule out other possibilities. These are the tests he ran:

Rather than showing you the negative ones, I’ll include images of the surgeon’s notes, along with those which were abnormal.

This test indicates my blood-brain barrier is slightly impaired. This could explain why my “brain fog” has continued to grow, as everything that’s come up in my search on the subject points to either Alzheimer’s Disease or multi-infarct dementia (MID). I don’t find this overly surprising as my grandmother had Alzheimer’s and my mother was showing definite signs as early as 50. It seems I must beat them to every diagnosis. I will likely write more on this subject as it’s entirely too vast and somewhat off-topic, but obviously I need to explore this further.

The high protein levels in my CSF may indicate there’s brain injury (possibly due to a mild chiari malformation that hasn’t been caught on a supine MRI, but could also be a sign of a tumor, bleeding, or nerve inflammation. This needs to be explored further.

Next Steps

As you can see, more exploration is necessary based on my test results. It will likely be a while before I have an official diagnosis in light of these tests. My doctor started by putting me on a diuretic, which will likely interfere with my POTS and have to be withdrawn. If my pressure climb again, I may need a repeat procedure and a stent placed. Until I meet with him in mid- September, I will not know for sure if he is diagnosing me with intracranial hypertension, but it seems highly likely. At this point, he would have no choice but to label it as idiopathic since no source has yet been discovered.

For many people with EDS, the cause is often related to craniocervical instability (CCI) and/or Chiari malformation (CM), though sometimes a tear in our delicate soft tissues is indicated as the cause of a CSF Leak. Interestingly, CSF leak is often what causes Intracranial hypertension. While I have struggled with symptoms of these problems for years, I need a qualified neurosurgeon who understands and can order proper flexion and extension MRI’s to either confirm or rule out CCI or CM. As much as I love and appreciate my head pain specialist, it’s beyond his realm of expertise.

CCI, CM, CSF leaks and IIH are all topics we have yet to explore on the Zebra Pit. They are topics I plan to cover in the coming months. Of these conditions, which do you think we should cover first? Are you affected by any of these conditions? We’d love to hear your experiences with lumbar puncture and diagnosis! Let us know about it in the comments.

As always, thanks so much for joining us! I hope you found this post educational and interesting. I hope you’ll share it with others who might also find it useful.

Resources and Further Reading


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When my doctor started talking about performing a lumbar puncture for signs of high pressure known as intracranial hypertension, I did what I always do, research everything I could find on the procedure, from what to expect during and after the procedure, what type of conditions can be diagnosed using LP and what exactly cerebrospinal fluid is, along with how its analysis can be interpreted. Join me in learning more about this procedure now.