Exquisite Goods

Tag: Acquired Chiari

  • The Michelle Cole Story – A Chiari Warrior’s Journey [UPDATED]

    The Michelle Cole Story – A Chiari Warrior’s Journey [UPDATED]

    As I sit down to update my journey, I am crushed that we’re still figuring things out (and nothing really was as I was initially told it would be), yet at the same time, I’m so thankful that we’re continuing to figure things out. Nobody should have to fight a fight like this (every symptom, every diagnosis), but all of this just increases my resolve to change it before anyone else in my family (or yours) is having to fight it! What we fail to change in our generation, our children and their children will face in theirs!

    Looking back, I have always had symptoms of Ehlers-Danlos Syndrome (EDS). As a child, I was in the school nurse’s office for stomach problems at least once a week. I was “double-jointed” and my friends always asked me to do maneuvers that I thought everyone should really be able to do if they tried. I was athletic early on, a tom-boy. I particularly loved playing softball (or baseball with tennis balls was the absolute best), but my ankles rolled when I started to run. Despite the fact that I was the only player that twisted their ankle multiple times in every game, I didn’t think there was really anything abnormal about me. Later, as an adult, I had repeated miscarriages and complications in all of my pregnancies, but the doctors conveniently came up with different explanations for each “rare occurrence.” It couldn’t possibly be all those rare mishaps, but EDS explained it all.

    My mother passed away from a brain aneurysm the day after my 18th birthday. She was just 37 years old when she died. As a child she had a lazy eye and scoliosis of the spine, so an eye patch and back brace were a normal part of her childhood attire. She suffered from migraines throughout her adulthood, but nothing was more tale-telling than reading her journal after she passed, with multiple entries about repeated headaches and neck pain. Decades after her death, my maternal grandmother (my mother’s mother) developed multiple brain aneurysms over the course of a decade. Each time one appeared, she had it filled with titanium coils. I always admired her fight for life.

    Me and my mom (1971).

    The first headaches that I remember started immediately after giving birth to my first son in 1992. It was a cesarean section at an Army hospital in Fort Ord, California. Instead of an epidural, they gave me three spinal injections to numb me from the chest down. At my postpartum check-up, I complained of daily headaches when upright. My primary care doctor ordered a CT scan, but because it was just a few years after my mom had died they looked only for brain aneurysms and found none. I was still having those orthostatic headaches six months later.

    Me and my eldest son, Johnathan (1992).

    The Accident that Shook Everything

    In 2000, I was a Bible College student and stay-at-home mom of three happy and active children (ages 8, 5, and 2). One September night, I was in a car accident that changed all of our lives. My neck was never the same again. My initial symptoms were head/neck pain, but all radiology reports indicated that everything was “unremarkable.” I tried everything they offered to me: rest, acupuncture, acupressure, steroid injections, osteopathic and chiropractic care, nerve stimulation units, physical therapy, pain meds, etc. Nothing worked long-term. Then in 2005, my neurological problems started intensifying. I began having bouts of partial paralysis in my legs and hands. I would just wake up one morning and out of the blue, I would have no fine motor skills. I would wake up feeling as though I had no thigh muscles to support me when I walked or tried to step up a step, and I had difficulty coordinating my footsteps. My primary care doctor at the time did blood tests and concluded that my “potassium level was on the low side of normal, so it must have been from potassium shock,” and he thought that no other tests were warranted. I started having vertigo whenever I was at any elevated height, even just a step or two up, like my brain couldn’t figure out how to balance with visual changes in height (I’d take a step up or down like the step was much higher or lower than it actually was). I also started having noticeable memory issues and intermittent trouble processing information. They tested to see if I was having small seizures in my sleep. When that was ruled out, they referred me to the memory clinic for further cognitive testing. They had no cognitive baseline to compare my results to, but said that I “tested higher than 89% of the population, so I should be happy,” and that I should just try reducing stress in case it was stress-related. They didn’t understand that it didn’t matter to me “how I compared to others.” I was only 34 years old and something was very wrong with me; I wanted answers that had nothing to do with the general population. In 2006, my eyes started twitching all day, every day, until the muscles just wore out and I could no longer hold them open completely. Oddly, one of my college professors inquired about my eyes and recommended that I have it investigated because it “could be neurological in origin.” When I did talk to my doctor about it, he saw the recommendations of the Memory Clinic and attributed it to stress as well, without any testing.

    My Chiari Diagnosis

    Finally, in 2010, ten years after the car accident, another MRI was done at my insistence to check for aneurysms once again (because I still was having excruciating head/neck pain and trouble holding my head up). I received an email from my primary care doctor that they found a cause of all of my symptoms. It was a condition called Chiari Malformation and the neurosurgery department would be contacting me to make an appointment. The neurosurgeon (who became my neurosurgeon) checked through my MRIs and said that the Chiari Malformation was evident on my first MRI after the accident ten years earlier. I was told that it was congenital and that it is commonly believed to be a result of prenatal drug use or lack of proper prenatal care (which was devastating to hear, but not all that unlikely as I was born in 1971. It also ended up being very wrong “textbook information” that they tell us all). Desperate for a measure of relief, I underwent a full decompression surgery a few weeks later. Missing the fact that part of my brain was in my spinal canal was 100% the hospital’s fault, but in hindsight, I really wish that I had done more research before surgery. I had comorbid conditions (many of which my doctors hadn’t even heard of, didn’t fully understand, and more importantly, they didn’t know the connection between these comorbids and my herniated tonsils). Initially, I felt quite a bit better. The release of pressure in my head helped my headaches. It was short lived though. Those undiagnosed comorbids caused my decompression to ultimately fail, although it all unfolded over several years.

    My preoperative MRI (2010).

    Post-op Complication: Pseudomeningocele

    When I was released from the hospital following decompression surgery, I was instructed not to lift, push, or pull anything for two weeks so that my dura patch would have a chance to adhere. The problem was, I could feel fluid squeezing out of the patch far beyond that two-week limit. I developed a pseudomeningocele (blue box above), which can be normal immediately after surgery before the dura adheres, but as long as there is no active leak, the body should absorb the fluid and the pseudomeningocele should quickly resolve. My neurosurgeon tapped some of the fluid out with a syringe twice and we waited patiently to see if it would subside on its own. It did not subside and in December 2012 (just over two years post-decompression), I developed acute vertigo. Everything was spinning and rocking, non-stop. It didn’t matter if my eyes were open or closed. I was waking up vomiting in my sleep from the dizziness. I couldn’t walk at all without falling hard to my right. I had no sense of balance at all and it didn’t just come and go, it was constant. Another MRI was done and it showed that my cerebellum was absorbing the fluid from the pseudomeningocele (so the cerebrospinal fluid was inside my brain, not just surrounding it; see light blue circle in image above). The decision was made to put in a subgaleo-peritoneal shunt (SP shunt), which runs from the pseudomeningocele to my peritoneum. They expected that it might take up to six months to fully drain from my cerebellum, but I woke up from the anesthesia with no signs of vertigo. I believe this surgery saved my life, but as with all shunts (especially amongst those with EDS, which I had not yet been diagnosed with), the shunt was destined to cause problems all by itself.

    Postoperative MRI (2012)

    My Many Shunt Revisions

    In April 2013, an unrelated CT Scan revealed that my shunt was no longer in my peritoneum. My NS scheduled for a general surgeon to “tie in” my shunt so it would not happen again (surgery #3). We went several months without complication until that November. The tied in shunt pulled out of my peritoneum again (it was excruciating). Hoping gravity would help in the matter, my NS did an incision just under my right rib cage and dropped it down into my peritoneum (surgery #4). Shortly thereafter, radiologist reports started showing a concern for the location of my brain and I was diagnosed with “Sagging Brain Syndrome.” So my six-week post-op appointment (which my NS did faithfully after every surgery) became my pre-op appointment for my 5th related surgery. This time a non-adjustable valve was attached to the shunt (at my chest) in hopes that by slowing down the amount of CSF being drained by the shunt, my head could retain more fluid and my brain could once again lift and become buoyant. Five months later I developed a hernia and upon closer examination (during surgery), it was found that my peritoneum was literally falling apart from all the trauma of the shunts; so my hernia removal surgery became a reconstruction surgery where my abdominal wall was pulled together with mesh, while carefully ensuring that the shunt didn’t come out (surgery #6). The shunt never moved again. As my brain continued to sag, the choice was made to replace the valve with an adjustable valve and in November of that same year, I was having surgery #7. The valve was adjusted to its slowest possible setting in hopes of finding a balance where it drained enough to keep the hydrocephalus at bay, yet retain enough CSF to lift my brain and keep it lifted and out of my spinal canal (so we could establish flow to the spinal canal and avoid the possibility of a syrinx).

    Diagnosis: Ehlers-Danlos Syndrome

    Despite my concerns that I might have a connective tissue issue and being told over-and-over again that I “didn’t look like someone with Ehlers-Danlos Syndrome,” I was finally diagnosed with it in May 2015. After finally finding a neurologist who understood the role that our connective tissues can have in a Chiari Malformation, I was given a referral to a geneticist. It still wasn’t as easy as it should be though. The geneticist did not know much about Chiari or Ehlers-Danlos related conditions (although he didn’t initially admit to that), so I had no idea at that point what was and was not related, and neither did my doctors. I received a call from the geneticist’s assistant and I agreed to send her pictures of my hypermobile maneuvers from the Beighton Scale. I could do all but bend over and put my hands flat on the floor with my knees straight, but I was able to do that when I was younger (and thinner). I was given a 9/9 on the Beighton Scale and was told that he would just mark my chart as diagnosed “hypermobile” and that he didn’t need to see me. I honestly didn’t know any better at this point, but I was about to learn something very important. I sat there thinking about what this “hypermobile” diagnosis would mean for me and decided to look more into EDS for myself. I read about the high risk of aneurysms, organ tearing, miscarriages, etc. and I was back on the phone with that assistant within twenty minutes. She asked if she could call me back, and within the hour the geneticist had decided that he needed to see me. He set up an appointment with me within twenty-four hours and asked if it was okay if he had a few others (doctors and medical students) there as well, since they’re a training hospital and they “don’t really come across patients with Ehlers-Danlos” (he should have told me that from the beginning). I agreed. Despite his lack of knowledge on EDS related comorbidities, he did know exactly where on my body to look for characteristics of EDS (all of which I thought I didn’t have). For instance, my skin isn’t unusually elastic, except in my upper arms and upper thighs. My skin isn’t translucent (I’m olive complected), except for on my breasts, back, and inner forearms. My skin isn’t unusually soft, except on my back. Now concerned that I might have Vascular Type EDS (vEDS), he decided to have me tested for that. The test was easy on my part but expensive on theirs. They drew blood and had it refrigerated and shipped to a lab in Washington state. It took thirty days for them to make sure that there was no mutation in my COL3A1 (collagen 3; alpha 1) gene, which has a median mortality age of 48. Initially, I felt devastated, since I was already 44. I decided that I hadn’t fought through all that I had, to only live a few more years. Thirty days later, the test came back indicating that I didn’t have vEDS and by default, I was diagnosed with Hypermobility Type EDS (hEDS). I was relieved, but the geneticist assured me that I still needed to be cautious. Since EDS symptoms are known to cross the type boundaries, and we already knew that vascular complications ran in the family (with the aneurysms) and with me personally (my peritoneum tearing), it technically made me “hEDS with vEDS crossover symptoms” and I’d probably have to explain that to my doctors for the rest of my life, so they remain aware of my potential to have additional vascular problems.

    My Poor Mess of a Neck

    The electric shock feeling in my spine (Lhermitte’s Sign) that I’d had intermittently for years, became an all-day, everyday thing, and much stronger in intensity. The MRI revealed that the herniated disc I had between my C3/4 was getting worse. The disc was removed with cadaver put in its place and the discs were fused together. My 8th surgery (ACDF = Anterior Cervical Discectomy and Fusion) wasn’t related to Chiari, but it was related to the EDS. We knew that my cervical spine was really bad from the beginning, but it got worse. I am now actually diagnosed with Degenerative Disc Disease in all three levels of my spine, but my neck has by far taken the brunt of it all. The ACDF, while 100% necessary, compromised the discs adjacent to it, and every disc from C4-7 is either bulging or herniated (Subaxial (cervical) Instability), so additional surgeries are likely to be needed.

    Learning to Advocate for Myself

    Over the past several years I have become an enthusiast of Chiari related research and MRIs (out of medical necessity more than anything). It became apparent to me that I absolutely needed to know everything that was going on in my body in case my doctors didn’t. When I first started, I’d print out studies and lay in bed with multiple high-lighters. I had such brain fog that I’d lay there crying at the fact that I was reading and rereading the same paragraphs over again, but I knew that I had to learn it despite how impossible it seemed. I prayed a lot for God to help me with my understanding and He did. I also started looking at the medications I was taking, the supplements I was taking, and what the ideal doses were for me (especially those that would help with inflammation and cognition), and other natural remedies. The first thing that I removed was all of the nerve meds that they had me on for peripheral neuropathy. I was maxed out on Nortriptyline (a tricyclic antidepressant) and almost maxed on Gabapentin (both of which had caused me to gain an incredible amount of weight over the years). When I informed my primary care doctor that I wanted to go off of them all, he thought it was a bad idea because of the severity of my neuropathy. I insisted though and asked him to help me to wean myself off of both of them in healthy intervals, and let’s “just see.” With the first down-dose, I physically felt a reduction in inflammation. It took me many months to wean off and get them out of my system, but in hindsight, I think this was the single best decision that I could have made. The longer I was on supplements instead of the nerve meds, the more my brain-fog improved, and I now believe that I have regained all that I’ve lost cognitively and then some.

    Syringobulbia

    In 2016, I was reviewing some of my old MRIs and I saw a large CSF filled hole in my lower medulla oblongata (lower brainstem). It was obvious in all MRI series since 2015, yet I was told that all was stable. After researching it, I asked my neurologist to take a look and see if it could be Syringobulbia. She referred my question to my neurosurgeon and he confirmed that I had an 11mm cyst in my brain stem. This type of cyst happens when there is a blockage of cerebrospinal fluid and is most frequent when the brain stem is also herniated below the foramen magnum (Chiari 1.5). It explained a lot of the problems that I was having, that we had thought to be unrelated. For instance, and I had a decreased sensitivity to temperature for years, never feeling hot or cold; and never having the automatic reactions that I should have had in response to temperature, like sweating and shivering. I could comfortably be outside in heat above 100° without breaking a sweat, or be outside in shorts and a tank-top when it was a chilly 30° morning without ever shivering. I also developed tachycardia and I am now medicated to keep my heart rate down to a safe level. My neurosurgeon ordered a new MRI in April 2017. The size of the syrinx had decreased to 9mm but was draining down my spinal cord forming an additional syrinx (Syringomyelia).

    Syringobulbia. Left – Syrinx in 2015 measuring 11mm in diameter. Right – Syrinx in 2017 measuring 9mm in diameter.

    Consulting a Specialist

    After all that I had been through in my fight, in April 2017, I decided to pay out of the pocket and have an online consultation with a Chiari Specialist in New York, who specializes in Chiari with EDS (the best $300 that I’ve spent in my fight). I sent him my pertinent medical records and copies of my MRIs in advance, wanting to find out what my doctor did right, and what he did wrong; and what course of action should be taken at that point. My expectation was that he would give me reasons why I should go to New York to see him, but that’s not at all what he told me. He told me what my doctor did right and that he didn’t disagree with the course of action that my neurosurgeon wanted to take. He said that my brain had sagged as low as it really could, but that since my high/low pressures had balanced out, and I was feeling better than I had in years, my syringes really should dictate our next course of action.

    In March 2018, following an exceptional year (at least where my head and neck are concerned) new imaging was done. My neurosurgeon asked me to come in to review it. It gave me a chance to tell him about the specialist’s opinions. My MRI showed that the Syringobulbia had decreased another 2mm. I asked him what that meant for the cervical syrinx, and that had almost completely disappeared. I asked him to go back to my images and correct me if I was wrong, but “the only reason that a syrinx (in either location) would dissipate like that was if I was finally getting CSF flow down my canal (despite my severe brain sag).” He agreed and I think he was a little surprised to see me think on my feet and figure that out in front of him (where I wasn’t having to ask anyone or look it up). He also confirmed that I had an Acquired Chiari, secondary to Intracranial Hypertension. He applauded me for learning all that I had and said that he wished that he had checked my pressures before decompressing me, as it may have changed the course of action that we had taken. And we agreed to wait a year and see where the syringes (syrinxes) are. As I left his office that day, I felt such a sense of relief, that we were finally getting CSF flow like the decompression in 2010 was meant to do.

    My Extensive Epidural CSF Collection

    In 2022, my neurosurgeon contacted me telling me that he was retiring and he’d like to have one last MRI of my entire spine (he added the brain to the request at my request). Unbeknownst to me, he ordered a CSF Leak Protocol, which consists of less slices, but they’re specifically looking for leaks. The images showed an “extensive extradural CSF collection from C7-L4, consistent with a CSF Leak and probable dural tear or CSF Venous Fistula.” They followed up with a Dynamic CT Myelogram. A Dynamic is different than a regular CT Myelogram, as they do it over 2-3 days, and they insert the contrast little by little into my spinal canal, and watch carefully for it to leave the spinal canal. CSF leaks and dural tears aren’t uncommon amongst Ehlers-Danlos patients, and usually happen in the front or back of the canal. CSF Venous Fistulas on the other hand are a much newer phenomenon, and they usually happen on the sides of the canal (more often on the right side). After two days of grueling tests, they found no active leaks or evidence of fistulas and surmised that what they saw on the MRIs to be “residual artifacts” from a leak that I had in the past… a leak that could have pulled my brain down into my spinal canal in the first place.

    Sagittal and axial views of my thoracic and lumbar images showing the residual artifacts of an extensive extradural CSF collection.

    It’s been a long road, hard road. I still battle inflammation and I’m definitely not done with surgeries. Eventually, I will need a ventriculoatrial (VA) shunt to hopefully resolve my high-pressure issues and enable us to remove my over-draining SP shunt that is making my brain sag. But for right now, I’m just enjoying feeling so much better! I praise God every step of the way, as I know that He’s there making a way out of no way. I have no idea why He took so long or why others haven’t seen the same results (because He loves them as much as He loves me), but I don’t have to have all the answers. I’ll just praise Him through the course of my journey, as He’s never let me go through it alone!

    *I dedicate this story to my family: John (husband), Ron (dad), Johnathan (son), MyKaella (daughter), Jojo (son) and my daughters-in-law, Violet and Sarah. Thank you all for all your help and for standing and kneeling beside me throughout my entire ordeal. You’ve been there for me and loved me through this long haul and I praise God for each and every one of you. 

    Originally written in 2018. Updated April 2022.

  • Brain Under Pressure – A Guide to Understanding Intracranial Hypertension [Updated]

    Brain Under Pressure – A Guide to Understanding Intracranial Hypertension [Updated]

    INTRACRANIAL HYPERTENSION (IH) MEANS HIGH PRESSURE INSIDE THE SKULL.

    Intracranial Pressure (ICP) is measured in millimeters of mercury (mmHg). Most scholars agree that on average, “normal pressure” should be between 5-15 mmHg, mild to moderate intracranial hypertension between 20-30 mmHg (which “requires treatment in most circumstances”), and an ICP of > 40 mmHg indicates “severe and possibly life-threatening intracranial hypertension.” [1] When high intracranial pressure is left untreated, it creates a “pushing effect” towards the only natural escape at the base of the skull (the foramen magnum), and the cerebellar tonsils in the pathway are pushed through the foramen magnum. [2]

    Understanding the Monro-Kellie Doctrine (pressure-volume relationship)
    The association between IH/IIH and Chiari Malformation appears to be a malicious intricate pathological circle. The cranium (skull) consists of brain matter, cerebrospinal fluid, and both venous and arterial blood. A hypothesis, referred to as the Monro-Kellie Hypothesis (now better known as the Monro-Kellie Doctrine), states, “The sum of volumes of the brain, CSF, and intracranial blood is constant. An increase in one should cause a decrease in one or both of the remaining two.” Therefore, if an abundance of cerebrospinal fluid (IIH or hydrocephalus), both cranial blood volume and brain matter should be forced to deplete. This depletion is usually directed in the path of least resistance – through the foramen magnum and into the spinal canal. When the brain matter closest to the bottom of the skull (cerebellar tonsils) is pushed through the foramen magnum and into the spinal canal (an Acquired Chiari Malformation), the tonsils act like a cork and blocks the flow of cerebrospinal fluid (regardless of the size of the tonsillar descent), which in turn, continues to raise intracranial pressure.[3]

    More Symptoms of Intracranial Hypertension

    Venous Hypertension
    When an etiological cofactor exists (such as a space-occupying mass), it is considered Secondary Intracranial Hypertension (SIH); when no other cause was identified, it is known as Idiopathic Intracranial Hypertension (IIH) formerly known as Pseudotumor Cerebri. However, recent studies on the connection between Intracranial Hypertension and Venous Hypertension might put an end to the “idiopathic” theory.

    Oxygen-rich blood travels from the heart to the rest of the body through the arterial system, then the oxygen-depleted blood returns to the heart through the venous system. We have a host of small veins in our head and they dump into a series of large veins, called sinuses. Dural Venous Sinus Stenosis occurs when there is a narrowing of one or more of the venous sinuses (most commonly seen in the transverse sinuses or transverse/sigmoid sinus junction), which in turn compromises cerebral venous outflow through the jugular vein (stenosis/compression of the jugular vein can also result in elevated intracranial pressure [4]). Transverse Sinus Stenosis (TSS) is most common in Idiopathic Intracranial Hypertension (IIH). Depending on the study that you are reading, it is proving to be present in 90-100% of IIH patients [5]. While its connection might sound obscure if you look at it from a Monro-Kellie perspective – The blood going into the head, cannot get out at the same speed (because of the narrowed sinus). When this inflow of blood remains constant and the outflow is hindered, the transverse sinus on that side (we have two transverse sinuses, one on each side) enlarges, forcing the CSF and brain matter to reduce to maintain the volume equilibrium. This reciprocation can happen when any of the sinuses or jugular narrow (stenosis). While scholars continue to debate whether TSS is a cause or consequence of IIH, surgeons continue to decompress us without checking our pressures or decompress (the most invasive treatment) in hopes that it will lower our pressures, and patients are left with untreated high pressure still causing a “pushing down effect” and an enlarged foramen magnum for our brains to be pushed down. [2] The sagging brain once again obstructs the flow of cerebrospinal fluid by plugging the foramen magnum, and that in turn raises the intracranial pressure even more. Or, the untreated high pressure blows through the duraplasty and causes a post-operative leak, known as a pseudomeningocele.

    Reducing the Risks of Post-Op IH/IIH Complications
    Brain MRIs often show indicators of Intracranial Hypertension (IH/IIH), therefore, we recommend that all Chiari patients have full brain MRIs and not just cervical MRIs.

    • When the pressure builds inside of the dura mater the pressure pushes the dura and fluid inside of the crevice that holds the pituitary gland (the sella turcica or pituitary fossa). When the amount of fluid is equal to or greater than 50% and the pituitary gland size is 2mm, the condition is known as Empty Sella Syndrome. (Doctors now recognize that < 50% (where the pituitary gland size is 3-7mm) can also cause symptoms and they now refer to that as a partially empty sella.) [8]

    • Slit like or flattened lateral ventricles from the increased pressure, however, when the Foramen of Monro (the aqueduct that connects the lateral ventricle to the third ventricle) is stenosed, the fluid will back-up and the lateral ventricle will not appear flattened. [7]

    • Enlarged/swollen optical nerves (papilledema). [8]

    • Low lying or herniated tonsils (often diagnosed as a Chiari Malformation). [2]

    What We Recommend BEFORE DECOMPRESSION is considered:
    If you have symptoms of IH/IIH accompanied by any of the MRI indicators mentioned above, it is both reasonable and prudent to ask your neurosurgeon to investigate further BEFORE DECOMPRESSION.

    • See a neuro-ophthalmologist to check for signs of papilledema, including Optical Coherence Tomography and Ultrasonographic B-scanning. [8]
    • Magnetic Resonance Venography (MRV, preferably with the ATECO technique) to check for venous stenosis of any of the cranial sinuses and/or jugular vein. Stenosis is not exclusive to the transverse sinus and it can happen in multiple sinuses simultaneously.
    • If overweight, consider trying to lose weight. Studies show that a weight loss of 5-10% of one’s overall body weight, when accompanied by a low-salt diet, can offer some to IH/IIH symptoms.[9]
    • Consider trying Diamox (Acetazolamide) and/or Topamax (Topiramate) to see if that improves the pressure headaches.
    • Request a lumbar puncture (spinal tap) to test your opening pressures. We recommend that it’s guided with fluoroscopy with a small gauge needle (and not the standard 22 gauge) that they allow to drip (as opposed to syringe pull) and ensure that someone is available to perform an epidural blood patch if necessary. Time should be allotted afterward to lay flat for several hours immediately following the procedure and for several days once returning home. The potential for CSF leaks is high for the EDS/Chiari patient. A doctor that marginalizes the risks ahead of time, will generally marginalize your symptoms when you are actively leaking.
    • ICP Bolt Monitoring can record the differences experienced in pressure over time, and how different positions affect ICP.

    Note: When the intracranial pressure gets high enough, it can cause a cranial leak. This is especially true for the Ehlers-Danlos patient where the dura mater is thin and fragile. When a cranial leak decreases the intracranial pressure, the papilledema, empty sella, stenosis, and high-pressure headaches can sometimes start to revert to normal or near-normal, and the leak will affect any attempts to check intracranial pressure (reducing the pressure from what it was before the leak occurred), however, the tonsillar herniation will usually remain if the pressure gets too low. [10]

    TREATMENT OPTIONS:
    If Venous Stenosis exists, stenting should be considered as leaving the sinus/jugular stenosed can post other health risks, and stenting is proving to have much better success with fewer complications requiring revisions. When medication fails to decrease ICP, and a stent is not an option, a Ventriculoperitoneal Shunt (VP Shunt) or Ventriculoatrial Shunt (VA Shunt) can be surgically placed to drain cerebrospinal fluid straight from the ventricle. Shunts are known for failing and often need a multitude of revisions, but even with all the revisions, it is less invasive than a decompression. Shunts under the foramen magnum should never be used as a means of controlling ICP.

    For the IH/IIH patient, herniated tonsils should be assumed an Acquired Chiari Malformation (even if a small posterior fossa is evident), and by correcting the high pressure before decompression, the decompression will be less likely to fail.

    Helpful Tips:
    If you have IH/IIH, it is best to avoid caffeine, avoid progestin based birth control, and all EDS patients should try to avoid the use of fluoroquinolones such as ciprofloxacin (Cipro), levofloxacin (Levaquin/Quixin), gatifloxacin (Tequin), moxifloxacin (Avelox), ofloxacin (Ocuflox/Floxin/Floxacin), norfloxacin (Noroxin), due to the increased risk of aneurysm.

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    References: 

    1 Rangel-Castillo, Leonardo, et al. “Management of Intracranial Hypertension.” Rangel-Castilla, Leonardo et al. “Management of intracranial hypertension.” Neurologic clinics vol. 26,2 (2008): 521-41, x. doi:10.1016/j.ncl. Feb. 2008, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2452989/>.

    2 Aiken, A.H., et al. “Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation.” American Journal of Neuroradiology; Nov. 2012, <http://www.ajnr.org/content/33/10/1901>.

    3 Mokri, B. “The Monro-Kellie Hypothesis: Applications in CSF Volume Depletion.” Neurology., U.S. National Library of Medicine, 26 June 2001, <https://www.ncbi.nlm.nih.gov/pubmed/11425944>.

    4 Zhou, D., et al. “Intracranial hypertension induced by internal jugular vein stenosis can be resolved by stenting.” European Journal of Neurology, November 2017 <https://onlinelibrary.wiley.com/doi/abs/10.1111/ene.13512>.

    5 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

    6 Pietrangelo, Ann. “Empty Sella Syndrome.” Healthline, Oct. 2017, <https://www.healthline.com/health/empty-sella-syndrome>.

    7 Hingwala, Divyata R., et al. “Imaging signs in idiopathic intracranial hypertension: Are these signs seen in secondary intracranial hypertension too?.” Annals of Indian Academy of Neurology vol. 16,2: 229-33. doi:10.4103/0972-2327.112476, June 2013, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3724081/>.

    8 Mollan, Susan P., et al. “A practical approach to, diagnosis, assessment and management of idiopathic intracranial hypertension.” Practical neurology vol. 14,6: 380-90. doi:10.1136/practneurol-2014-000821. May 2014, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4251443/>.

    9 Thurtell, Matthew J., and Michael Wall. “Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Recognition, Treatment, and Ongoing Management.” Current Treatment Options in Neurology, U.S. National Library of Medicine, Feb. 2013, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3554852/>.

    10 Pérez, Mario A., et al. “Primary spontaneous cerebrospinal fluid leaks and idiopathic intracranial hypertension.” Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society vol. 33,4: 330-7. doi:10.1097/WNO.0b013e318299c292, Dec. 2014, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4040082/>.

  • Spine Pulled Tight – A Guide to Understanding Tethered Cord Syndrome

    Spine Pulled Tight – A Guide to Understanding Tethered Cord Syndrome

    TETHERED CORD SYNDROME (TCS) INVOLVES A STRETCHING OF THE SPINAL CORD, AND OFTEN YOUR MEDULLA OBLONGATA
    AS WELL, WHICH LEADS TO A HOST OF NEUROLOGICAL PROBLEMS.

     

    Before we talk about Tethered Cord Syndrome, let’s first talk about the anatomy associated with the spinal column (in layman’s terms).

    •  The role of the vertebral column is to hold the spine strong (so it can be upright) and protect the spinal cord from injury. In a normal vertebral column, there are thirty-three vertebrae on each side (seven cervical vertebrae, twelve thoracics, five lumbar, five fused vertebrae in the sacrum and another four fused vertebrae in the coccyx).

    •  Each vertebra in the upper twenty-four vertebrae is separated by intravertebral discs largely composed of the fibrous protein, collagen. The main role of these discs is to allow the vertebral column to move and flex.

    •  The role of the spinal canal is to hold cerebrospinal fluid around the spinal cord, which not only cushions the cord against injury, but it also lubricates the cord, cleanses the cord, and brings essential nutrients that the spinal cord needs. The spinal canal is made up of several layers that form the meninges. These layers are also composed of high concentrations of collagen. The outermost layer of the meninges is the dura mater. The dura mater should be dense and strong, so cerebrospinal fluid cannot leak from it.

    •  The spinal cord relays messages from the brain to the nerves that allow the body to function. When part of the spinal cord is compromised, it can inhibit signals from getting to the nerves from that point downward. The brainstem (midbrain, pons, and medulla oblongata) is attached to the spinal cord at the top (the medulla and spinal cord meet at what is called the cervicomedullary junction) and the spinal cord continues down to the mid/lower back. From there it becomes a delicate elastic band of fibrous collagen-based tissue called the filum terminale that extends from the conus medullaris (the lowest point of the spinal cord before it becomes the filum terminale) to the dural sac at the S2 level.1

    Chiari Malformation has many conditions that can be associated with it (comorbid conditions) and sometimes those comorbid conditions can be at the root cause (etiological cofactor) or one of the causes along the way (pathological cofactor) to the tonsils being as low as they are (making the Chiari “secondary” to one or more “other” conditions). Tethered Cord Syndrome (TCS) is one of those pathological conditions.2 Like Chiari, it is a neurological disorder; however, it is one of the spinal cord.3

    Tethered Cord happens when the sticky fibrous tissue of the filum adheres to fatty/scar tissue or the dura lining of the spinal canal.1 While this tethering can happen anywhere in the spinal canal, it is most common at the lumbosacral level.4 When the tethered filum pulls the spinal cord tightly enough that it causes neurological problems, it becomes known as Tethered Cord Syndrome (TCS). Tethered Cord is most common in patients with Spina Bifida (myelomeningocele, meningocele), Spina Bifida Occulta (lipomeningomyelocele, lipomyelocele) and patients with Ehlers-Danlos Syndromes (EDS), a Hereditary Disorder of Connective Tissue (HDCT) where one or more of the types of collagen (the most abundant protein in the human body) is mutated at a cellular level. Tethered cord can be congenital or acquired. It can be obvious in childhood or symptoms may not present themselves until adulthood. Some children may develop minor signs that are overlooked by untrained medical professionals and can progress slowly or rapidly over time.

    More Symptoms of Tethered Cord Syndrome

    A Tethered Cord Syndrome diagnosis can be somewhat of a challenge. The signs and symptoms of the condition are not always present and when they are, they are often not recognized, so it is important to know all indicators. People with Tethered Cord (TC) can have sacral dimples, discoloration, and hairy patches on their lower back that can lead a doctor to investigate further, however, some have no external signs at all. Some have kyphosis (rounded back) and scoliosis (curved spine). Sometimes radiological criteria are not met or are ambiguous, yet an Occult Tethered Cord (characterized by the presence of symptoms with normal conus position and inconclusive findings of the filum) can still exist.5 Symptoms can be elusive as well and can happen all at once or gradually over the course of many years. Many symptoms worsen due to activity; climbing stairs has been reported as causing pain that varies from uncomfortable to excruciating.

    One of the reasons that Tethered Cord is often overlooked is that many neurosurgeons are not aware of the connection it can have with a Chiari Malformation and the medical tests used for determining if a tethered cord problem exists are not always accurate or accurately read.

     

    Magnetic Resonance Imaging (MRI)

    • A lumbar MRI is usually the first step. This gives a visualization of the spinal cord in relation to the surrounding vertebrae. The actual tethering is not always obvious on MRI, sometimes the only proof of the tethering is the pulling it creates on the spinal cord. Doctors will look for the position of the conus medullaris when looking for signs that the spinal cord is being pulled. The consensus amongst most surgeons is that a normal conus should be located from the T12 to the lower L2. There is much debate on the importance of establishing evidence of a low-lying conus.5 When conus reaches the lower L2 or below, doctors should be investigating why it’s low and consider if the cord might be tethered. When looking for the location of the conus, your position can make all the difference. MRIs are generally performed supine (lying down) and the cord is not pulled as tightly as it is when upright. For this reason, upright MRIs are becoming the method of imaging preferred by most neurosurgeons looking to confirm or deny if tethering exists in a patient showing symptoms. Other signs of tethering that might be visible in a lumbar MRI include an enlarged foramen magnum, thick or fatty filum, presence of fatty tissue inside the canal, or the filum might be pulling to one side of the canal.5

    • A prone MRI of the lumbar region can be an invaluable tool for those where other MRIs indicate that the filum might be pulling to one side (usually the back side) of the canal. With prone MRIs, imaging is done while the patient is lying face down (as opposed to facing up, like most supine MRIs). If the anteroposterior conus movement of >10% of the canal width was evident from the supine to the prone, then the likelihood of it pulling to one side do to tethering is less likely and more conservative management might be better appropriate than a surgical release.6

    • A cervical MRI can also show signs that a tethered cord might exist. The cervical spinal cord can sometimes appear narrow from it being pulled tight. The medulla oblongata can become elongated. This happens because the brainstem is attached to the top of the spinal cord and that cord is being pulled tight, essentially pulling everything down and tight. This elongation of the medulla from the tethering can cause secondary symptoms by itself, known as Dysautonomia.

    · Low/herniated cerebellar tonsils consistent with what is seen in a Chiari Malformation. When the brainstem is herniated (where part/most of the medulla is below the foramen magnum) along with the cerebellar tonsils, it is considered a Chiari 1.5 (which should be a good indicator that you might be dealing with an Acquired Chiari Malformation, where the herniated tonsils are secondary to another condition). One study quoted that out of 2,987 patients with a tonsillar herniation of 5mm or greater, 14% met the diagnostic criteria (based on “generally accepted clinical and radiographic criteria”) and 63% of the 289 patients with tonsillar herniations of < 5mm.5

    · A syrinx is common with Tethered Cord, as it causes a blockage of fluid at the foramen magnum. A syrinx can develop anywhere in the spine, usually in the lower cord, but with Tethered Cord Syndrome it can develop in the lower medulla (Syringobulbia) as well because of the low brainstem is at the point of the blockage of fluid from the Chiari Malformation.

    Even with an upright MRI and every symptom listed, patients are often told they do not have Tethered Cord. This is simply due to a lack of education on the subject and medical bias between doctors. It is important to make sure that you have the images viewed by a neurosurgeon that is familiar not only with Tethered Cord but Chiari and Comorbids as well. (Nearly half of the large study quoted above were referred following a failed Chiari Decompression.5) The combination of the images and the patient’s symptoms should tell the neurosurgeon if surgical intervention is required. Patients often require several consultations before they can find a knowledgeable enough physician.

     

    What We Recommend BEFORE DECOMPRESSION is considered:
    If you have symptoms of TCS, especially if accompanied by any of the MRI indicators mentioned above, it is both reasonable and prudent to ask your neurosurgeon to investigate further before decompression is considered. A Tethered Cord Release Surgery prior to decompression may relieve the tension that is pulling the brainstem and cerebral tonsils downwards reducing the risk of a failed decompression. There is a chance with small tonsillar herniations that the Tethered Cord Release might allow the cerebellar tonsils to rise enough to the point that cerebrospinal fluid flow is reestablished to where decompression is no longer needed. However, failure to release a tethered cord prior to decompression surgery increases the likelihood of a failed decompression. (In fact, in the study quoted above, out of the 3,276 patients with herniated tonsils, 46% of them were referred for evaluation after a failed decompression surgery.5) An MRI of all three levels of the spine should be done to rule out other possible causes for leg/back symptoms along with urodynamic testing, an Electromyogram (EMG) Test and Nerve Conduction Study (NCS) of the lower limbs is also suggested.

     

    TREATMENT OPTIONS:
    For some, physical therapy can help with symptoms for a while. However, ultimately it will likely need to be surgically treated with a Tethered Cord Release.

    Tethered Cord Release (TCR) Surgery involves the untethering of the spinal cord. An incision is made in the lumbar area, the filum terminale is separated and the factors that are tethering the spinal cord to the vertebrae are severed. Surgical treatment is not without risk and does not guarantee relief of symptoms. However, in a large study, up to 83% of adult patients report relief, 16% unchanged, and 1% report feeling worse.5 In children, the numbers are even better with 93% obtaining improved symptoms and 7% unchanged.5 Most patients describe the surgery as extremely painful for the first two weeks and “better than they ever remember feeling” (often because they have been tethered for much of their lives) after two weeks. The most common complication involves retethering (often from the scar tissue from the release) and multiple surgeries may be required over a lifetime. Finding a neurosurgeon experienced with TCRs and the surgical treatment of Ehlers-Danlos patients can sometimes help reduce the risks associated with scar tissue formation, but scar tissue can happen with even the best of neurosurgeons.

    For the TCS patient, herniated tonsils really should be assumed an Acquired Chiari Malformation (even if a small posterior fossa is evident), and by correcting the tethered cord before decompression the decompression will be less likely to fail.

     

    Special Note: There are other conditions that can present with similar symptoms. Diastasis Recti is a type of abdominal hernia common to pregnant women, those with obesity, and EDS patients. This separation in the abdominal muscles is known to sometimes cause lower back pain and many of the same pelvic floor problems seen with Tethered Cord Syndrome (TCS). Unlike TCS however, it does not usually require surgical treatment. If you suspect Diastasis Recti, we recommend that you talk to your Primary Care Physician about referring you to physical therapy to bring your abdominal muscles back together before considering Tethered Cord Release (TCR).7

     

     

    [wpedon id=”4396″ align=”center”]

     

     

    References: 

    1 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

    2 “Section of the Filum Terminale Surgery for Tethered Spinal Cord Syndrome in Patients with Chiari Malformation and Syringomyelia.” North Shore-Long Island Jewish Health System The Chiari Institute, Chiari Connection International, 02 Oct. 2006, <http://www.chiariconnectioninternational.com/docs/TCS_SFT_Explained.pdf>.

    3 Quake. “Overview: Chiari Comorbidities & Etiological/Pathological Cofactors [Revised].” Chiari Bridges, 16 Nov. 2019, <http://chiaribridges.org/overview-chiari-comorbidities-etiological-pathological-cofactors/>.

    4 Arslanoglu, A., et al. “Multidisciplinary Combined Approach for Tethered Spinal Cord Syndrome: Radiology, Surgery and Physical Therapy.” Balkan Military Medical Review, 2006, <https://pdfs.semanticscholar.org/8c30/18bf5bfbd6f3e7e5e9c3559bbbfdcac82e04.pdf>.

    5 Milhorat, Thomas H., et al. “Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale.” Surgical Neurology, Elsevier, July 2009. <https://www.sciencedirect.com/science/article/abs/pii/S0090301909002572>.

    6 Aoun, Salah G., et al. “The Use of Prone Magnetic Resonance Imaging to Rule Out Tethered Cord in Patients With Structural Spine Anomalies: A Diagnostic Technical Note for Surgical Decision-making.” Cureus vol. 11,3 e4221. 11 Mar. 2019, doi:10.7759/cureus.4221. <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6510567/>.

    7 “A Complete Guide to Diastasis Recti: Truths on Abdominal Muscle Separation.” Braceability, 05 June, 2018. <https://www.braceability.com/blogs/info/diastasis-recti>.

  • A Mother’s Story

    A Mother’s Story

    Today is 11th April, 2019. Spring is in the air, yet I struggle to appreciate its presence. My daughters are at school, my son is at home in bed yet again. Like so many other days he is unable to get up. My son is 19 years old and looks just like any other 19 year old. You would never guess that this 19 year old is fighting a tremendously unfair battle every single day and has done so for several years.

    Let me rewind.

    My son was around 9 years old when he first complained of a lack of feeling on his right side and regular headaches. Doctors in Ireland, where we were living at the time, told him to drink more fluids after his daily soccer practice and put the numbness down to a trapped nerve. When he was 14 years old and living in Canada, he was told exactly the same by doctors there. However, when I finally insisted on him being referred to a neurologist, this very neurologist laughed at my son for wasting his time. He was told that it was all in his head. I vividly remember telling him off myself in the carpark on our way home.

    I also remember being disappointed about his worsening school reports, blaming the onset of teenage years for his inability to concentrate and retain information. Blurred vision was also dismissed when his eye test came back just fine. Doctors didn’t grow concerned until he was 16 years old and living in France when a routine soccer medical check-up showed a sudden scoliosis deterioration from 8 degrees to 40 degrees. Subsequent MRIs showed Chiari Malformation (CM) with extensive Syringomyelia.

    Neurosurgeons were quick to reassure him that all should be fine after a decompression surgery. Nevertheless, I spent hours researching these unknown rare conditions and found two experienced neurosurgeons, one in England and one in Belgium, for second opinions. Whereas surgeons in France took a more traditional approach and talked about inserting a shunt, both these surgeons warned strongly against this and so we made the decision to go to Belgium for the surgery. We felt well informed and were full of hope when my son embarked on his healing process 3 years ago.

    Let me tell you where we are now.

    Doctors in Europe tell us that my son is one of the unlucky few as his health has drastically deteriorated. Scar tissue has attached itself to his brain tonsils but that only explains part of his deteriorated health. So I embarked on a mission to get to the bottom of these problems. Surely there was hope to be found in the health system in France, one of the best in the world! After countless appointments with multiple health professionals, we were dumbfounded by the complete lack of understanding, knowledge and pure arrogance in relation to CM and its associated conditions, which resulted in my son’s mental health being questioned yet again.

    I started carrying out my own research, which clarified the distinct link between brain disorders and compromised immune/digestive systems. Whereas his doctors are reluctant to make that link, the evidence is clear. 18 months after surgery, my son got struck down by glandular fever. Again, we were hopeful that this would only be a temporary setback. Today however, my son suffers from chronic fatigue syndrome as well as dysautonomia.

    At our wits end last summer, we turned to a hospital in the United States that specialized in Chiari Malformation. Our first consultation with its Managing Director turned out to be an eye opener. This neurosurgeon could literally finish our sentences. My son was finally understood. It turns out that doctors in Europe had failed to diagnose another condition, Ehlers-Danlos Syndrome (EDS), which caused craniocervical instability. This in itself can be a debilitating condition but the combination of craniocervical instability with brain decompression surgery can be a death sentence. He further explained that such patients are deemed to benefit from Occipitocervical Fixation (OC) Fusion surgery. However, this surgery has not yet received the green light for these conditions from Health Services in Europe.

    Armed with a diagnosis of Complex Chiari, we faithfully returned to my son’s French doctors, only to be met, yet again, by a lack of understanding. My request for an upright flexion/extension MRI was seen as outlandish and peculiar. Turns out, an upright MRI is not yet available in France. Instead they still rely on flexion/extension X-ray images which fail to adequately detect craniocervical instability.

    In recent discussions, our son’s Belgian neurosurgeon cautiously recognizes the link between CM and EDS. However, as these studies are in their infancy in Europe, doctors still carry out decompression surgeries without checking for EDS. He also questions the durability of an OC Fusion but agrees that much more extensive research needs to be carried out in Europe and that my son is extremely unfortunate this hasn’t happened yet.

    So where does this leave my son? In the land of limbo. Knowing that Europe is trailing some 10 years behind the States in this field. France, with its inherent reluctance to change, probably closer to 15 years. School is no longer an option for my son as his brain fog and memory loss have become more and more of a problem, his fatigue too debilitating and his headaches too frequent.

    We are tired of fighting the system, tired from having to spell out the name of his conditions to health professionals, tired of being misunderstood. There are days I avoid going out as I don’t want to answer people’s well-meant questions. There are days I am ashamed of the anger that wells up inside me when friends air their worries about their children’s school results. There are days I feel like I am being punched in the stomach when I see his friends play a soccer match. People tell me I am strong. I don’t agree. I wish I had been strong all those years ago and believed my son over his doctors.

    My son is my hero. My son is a fighter. My son has generally done what health professionals told him to do, taken every medication health professionals told him to take, followed the advice health professionals told him to take, yet the system continues to let him down. When I look into my son’s eyes, I still see this steadfast determination but I now also see pain and disillusionment. My son believed me when I told him we would overcome this together. My son believed me when I told him the worst would be over soon. My son doesn’t believe me anymore. I feel that I have failed him.

    Complex Chiari diagnosed so late is a life sentence. It has brought pain, sadness and isolation, not only to my son but to my entire family. My husband and I are learning to compartmentalize, enjoy moments. We live in hope that our son’s better periods will begin to lengthen and pick ourselves up every time these come to an abrupt end. We have to. We owe it, not only to our son but to our daughters too.

    We continue our journey through the unknown, thankful for the sources of information coming from the United States, usually met with skepticism and resistance by French health professionals. However, one thing I have learned from our journey so far is that we cannot fight these conditions and health services alone. Surely there must be more people out there in Europe. Surely, as a group we can start making a difference. Let’s unite! Let’s educate! Let’s raise awareness! Our children deserve so much better! Our children deserve to be heard, supported, and at the very least, understood!

  • Brain Under Pressure – Understanding Intracranial Hypertension [Archived]

    Brain Under Pressure – Understanding Intracranial Hypertension [Archived]

    INTRACRANIAL HYPERTENSION (IH) AND IDIOPATHIC INTRACRANIAL HYPERTENSION (IIH) ARE CONNECTED, BUT ARE NOT THE SAME THING AND THEREFORE SHOULD NOT BE USED INTERCHANGEABLY.

    Intracranial Hypertension (IH) means high pressure inside the skull. Intracranial Pressure (ICP) is measured in millimeters of mercury (mmHg). Most scholars agree that on average, “normal pressure” should be between 5-15 mmHg and that 20-25 mmHg is when the ICP crosses the line into being IH. Pressure can be brought on by several different means: space-occupying masses such as hydrocephalus and cranial cysts/tumors; cranial edema (Encephalitis); trauma; stroke; aneurysm; certain infections/diseases (Meningitis), liver failure[1], kidney failure[2]; or as a side-effect of certain medications (such as: Tetracycline[3][5], Sulfasalazine[4], Lithium[5], excess amounts of Vitamin A, steroid use[6], growth hormone treatments[6], and the hormonal Intrauterine Device (IUD), “Mirena”[7]); however, sometimes the cause of the pressure is completely unknown. When an etiological cofactor exists, it is considered Secondary Intracranial Hypertension (SIH); when no other cause is identified, it is known as Idiopathic Intracranial Hypertension (IIH) or Primary Intracranial Hypertension (PIH).

    “Idiopathic Intracranial Hypertension (IIH) was first noticed in 1893, by the German physician Heinrich Quincke, who named it Serous Meningitis. As its absence of space occupying masses/lesions began to draw more thought, it was renamed Pseudotumor Cerebri (PTC) by Max Nonne in 1904. Sometime later, the term “Benign Intracranial Hypertension” began being used interchangeably with Pseudotumor Cerebri, to describe the fact that while it is sharing some of the same characteristics that a cranial tumor would cause, it is benign (not harmful), but arguments were made against it in that blindness is not indicative of being benign.”[6] The name finally settled as “Idiopathic Intracranial Hypertension,” which means IH of an unknown cause. No matter what you choose to call it, the pain and damage remains the same for those who have it.

     

    UNDERSTANDING IDIOPATHIC INTRACRANIAL HYPERTENSION
    IIH is a neurological disorder where the cerebrospinal fluid within the skull is elevated, without the presence of a space-occupying mass, edema (brought on by things such as trauma, infection, or disease), or any adverse reactions to certain medications. Studies show that IIH is more common amongst women between the ages of 20 and 50,[8] and there is a slight increase amongst those that are overweight. Some studies also suggest a connection between obstructive sleep apnea and transverse cerebral venous sinus stenosis.[9] Amongst the general population, IIH is believed to exist in 1/100,000 (0.00001). Amongst those that are 10% above their ideal body weight, the numbers increase to 13/100,000 (0.00013), and rising to 19/100,000 (0.00019) in those 20% above their ideal body weight.[10] Although doctors often tend to pass this off as merely a side effect of weight gain, the increase is slim and seems to decrease as the percentage of weight gain above ideal weight continues to rise above the 10% margin. Additionally, the weight factor excludes men and children under the age of 10, which may simply be because women are more likely than men to have comorbid conditions that would lead to Intracranial Hypertension. Studies show that the women to men ratio for Chiari Malformation is believed to be 3:1 and those with both Chiari Malformation and Ehlers-Danlos Syndromes 9:1[11]). However weight is not irrelevant with IIH, the overweight/obese patient population report finding improvement of some symptoms when weight loss of 5-10% of one’s overall body weight, when accompanied by a low-salt diet[12]. 

     

    UNDERSTANDING THE IH/IIH CONNECTION: THE MONRO-KELLIE DOCTRINE
    The association between IH/IIH and Chiari Malformation, appears to be a malicious intricate pathological circle. The cranium (skull) consists of brain matter, cerebrospinal fluid, and both venous and arterial blood. A hypothesis, referred to as the Monro-Kellie Hypothesis (or Monro-Kellie Doctrine), states, “The sum of volumes of brain, CSF, and intracranial blood is constant. An increase in one should cause a decrease in one or both of the remaining two.”[13] Therefore, if there is an abundance of cerebrospinal fluid (IIH or hydrocephalus), both cranial blood volume and brain matter should be forced to deplete. This depletion is usually directed in the path of least resistance – through the foramen magnum and into the spinal canal. When the cranial brain matter closest to the bottom of the skull (cerebellar tonsils) goes through the foramen magnum and into the spinal canal (an Acquired Chiari Malformation), it blocks the flow of cerebrospinal fluid, which in turn, continues to raise intracranial pressure.

     

    SYMPTOMS OF INTRACRANIAL HYPERTENSION
    Intracranial Hypertension (IH) can be either acute or chronic and comes with a variety of symptoms, many of which can help distinguish IH pain from typical pain associated with Chiari Malformation. A typical Chiari headache originates at the back of the skull (at the occiput), but IH headaches are usually described as pressure at the top of the head, that radiates downward. Headaches tend to be worse when laying down (which is opposite of low pressure headaches that are often relieved by laying down). Those that suffer from IH, often report waking up from sleep with a bad headache, and often a slight incline can help alleviate the headache pain. Pulsatile Tinnitus occurs when you hear a ringing in your ears that coincides with your heart beat. The tale-tell symptom of IH involves the damage done to the optical nerves.  Papilledema is when the optic discs swell in response to the increased cranial pressure.[14] Symptoms of Papilledema include: headaches behind the eyes, blurred vision, fleeting vision, dimmed vision, double vision, visual obscurations, decreased peripheral vision, and photopsia. Another source of IH damage is seen in the pituitary gland and is known as Empty Sella Syndrome (ESS). As the high intracranial pressure (ICP) tries to take over, cerebrospinal fluid finds its way to the sella turcica and starts filling it with spinal fluid (partially or completely)[15]. The intruding CSF attempts to envelope this depression in the sphenoid bone, and squeezes the pituitary gland, flattening it until it appears “empty.” While some initially suffer no symptoms of the damage done to the pituitary gland, most eventually develop a variety of hormonal issues, known as hypopituitarism.

     

    DIAGNOSIS CRITERIA
    Diagnosis of Intracranial Hypertension usually begins with investigating either the headaches or the vision problems. The least invasive test is having a neuro-ophthalmologist check behind your eyes for Papilledema. It is not considered conclusive in testing for IH, but it is essential in determining the extent of the damage to the optical nerves. Magnetic Resonance Imaging (MRI) of the brain can be useful in showing signs of Intracranial Hypertension. In cases where one or more space-occupying masses exists, further imaging and often biopsy may be required. The type of mass, its exact location, and the amount of damage that it is believed to be doing, will be used to determine the best treatment. If imaging gives an indication that the intracranial pressure is high, but no space-occupying mass exists, additional testing is usually necessary to confirm, some of which can be potentially be dangerous for those with Heritable Disorders of Connective Tissue (HDCT), such as Ehlers-Danlos Syndromes (EDS). Lumbar punctures (LP), also known as a spinal tap, are often used to test the opening CSF pressure, but by puncturing the dura (which is thinner than normal with Connective Tissue Disorders), the risk of a CSF leak is high. When an LP causes a CSF leak, the first indication is usually a post-dural-puncture headache (PLPH) and eventually, the intracranial hypertension will decrease, as the leak causes intracranial hypotension.[16] CSF leaks can escalate very quickly and can be difficult to identify and treat; therefore, we recommend that LPs be done only when absolutely necessary, and that they be done only under fluoroscopy, by qualified surgeons that fully understand the likelihood of Connective Tissue Disorders, the symptoms of leaks, and have a plan of action should those symptoms occur. Sometimes, ICP can fluctuate and have high spikes that cause problems, rendering LPs useless unless they are done at the precise time. When these spikes are suspected ICP monitoring bolts might be the better option, but still poses a risk of leaks.[17] 

     

    TRANSVERSE SINUS STENOSIS (TSS)

    Transverse sinus stenosis (TSS) occurs when there is a narrowing of the transverse sinus (dural venous sinus), which in turn can compromise cerebral venous outflow. TSS is common in idiopathic intracranial hypertension (IIH). Depending on the study that you are reading, it is proving to be present in 65-100% of those diagnosed specifically with IIH. Its direct connection seems relatively obscure, and there is no indication of its prevalence in intracranial hypertension (IH), but it is worth looking for and treating if found. While scholars remain undecided as to whether TSS is a cause or consequence of IH, if it does prove to be a cause of high pressure, IIH will likely no longer have an idiopathic element to it and it will become another etiology of Intracranial Hypertension. TSS can often be undetectable with standard Magnetic Resonance Imaging (MRI). The correct procedure would be Magnetic Resonance Venography (MRV, with the ATECO technique [18]), specifically looking for signs of stenosis, to include looking for fistula(s) and aneurysm(s). The lack of a fistula or aneurysms however, does not exclude the possibility of a TSS existing (remember it’s being found in 65-100% of those with IIH). Even with MRV, TSS can often be misinterpreted as “flow-related artifacts.” [18] Because the prevalence of TSS in IIH patients is high (some studies call it “universal”) [19], we recommend that all IIH patients have a MRV with the ATECO technique done before surgical treatment and that venous stenting be considered as a viable surgical treatment.

     

    TREATMENT OPTIONS
    Treatments for Idiopathic Intracranial Hypertension usually starts with weight loss and/or medicinal options; Diamox (Acetazolamide) and Topamax (Topiramate) are most frequently prescribed. Those with IH/IIH should avoid consuming caffeine, as it can increase pressure and therefore is counter-productive to treatment measures. Diamox is a carbonic anhydrase inhibitor and Topamax can also inhibit carbonic anhydrase, but is an anticonvulsant, often prescribed for the treatment of neuropathy and seizure disorders. Both are believed to successfully lower the production of cerebrospinal fluid. Topamax can also help suppress the appetite, which can help with weight loss, but it also comes with many side-effects like all nerve meds do. When medication fails to decrease ICP, a Ventriculoperitoneal Shunt (VP Shunt) or Ventriculoatrial Shunt (VA Shunt) are surgically placed to drain cerebrospinal fluid straight from the ventricle. Shunts are known for failing and often need a multitude of revisions. Venous stenting is not a new procedure, yet it is not readily offered. While there are studies indicating that the successful reduction of intracranial pressure can help with TSS. Stenting is not only a surgical treatment for the stenosis (which could significantly reduce the possibility of a life-threatening aneurysm in patients with a connective tissue disorder), but it is also a surgical treatment for intracranial hypertension as it “improves CSF resorption in the venous system.” [18] Therefore, it seems illogical to shunt (just dealing with the pressure) and leave such a potentially life-threatening condition untreated. [20] Studies are indicating as high as a 94% of patients being cured of all IIH symptoms as a direct result of venous stenting. [18] While all surgeries pose a risk of complications, and the statistics for stenting are likely inflated and skewed (like that of decompression surgeries), these statistics on stenting are definitely encouraging!

    Intracranial Hypertension is a complex issue that should be explored whenever a Chiari Malformation exists, before a decompression surgery is performed. When both Intracranial Hypertension and Chiari Malformation are found to co-exist, the treatment should be in consideration of the correlation of the two, as they both are pathological co-factors of one another. Failure to recognize and treat Intracranial Hypertension before or soon after decompression surgery, will increase the likelihood of a failed decompression. While a decompression surgery can lower Intracranial Hypertension, as cerebrospinal fluid is once again allowed to flow, if space-occupying masses or a case of Idiopathic Intracranial Hypertension (where too much cerebrospinal fluid is being created) are left untreated, those problems will still exist after decompression surgery and the high pressure is likely to cause the cerebellar tonsils to fall once again.

    *Revised October 2018

     

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    References:

    1 Jalan, R. “Intracranial Hypertension in Acute Liver Failure: Pathophysiological Basis of Rational Management.” Seminars in Liver Disease., U.S. National Library of Medicine, Aug. 2003, <www.ncbi.nlm.nih.gov/pubmed/14523680>.

    2 Chang, D, et al. “Benign Intracranial Hypertension and Chronic Renal Failure.” Cleveland Clinic Journal of Medicine., U.S. National Library of Medicine, <www.ncbi.nlm.nih.gov/pubmed/1525975>.

    3 Holst, Anders Vedel, et al. “A Severe Case of Tetracycline-Induced Intracranial Hypertension.”Dermatology Reports, PAGEPress Publications, 31 Jan. 2011, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4211491/>.

    4 Sevgi, E, et al. “Drug Induced Intracranial Hypertension Associated with Sulphasalazine Treatment.” Headache., U.S. National Library of Medicine, Feb. 2008, <www.ncbi.nlm.nih.gov/pubmed/18070060>.

    5 Kelly, S J, et al. “Pseudotumor Cerebri Associated with Lithium Use in an 11-Year-Old Boy.”Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus., U.S. National Library of Medicine, Apr. 2009, <www.ncbi.nlm.nih.gov/pubmed/19393521>.

    6 Aylward, Shawn C. “Intracranial Hypertension: Is It Primary, Secondary, or Idiopathic?”Journal of Neurosciences in Rural Practice, Medknow Publications & Media Pvt Ltd, 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4173226/>.

    7 Etminan, Mahyar, et al. “Risk of Intracranial Hypertension with Intrauterine Levonorgestrel.”Therapeutic Advances in Drug Safety, SAGE Publications, June 2015, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4519742/>.

    8  “Pseudotumor Cerebri Information Page.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, <www.ninds.nih.gov/Disorders/All-Disorders/Pseudotumor-Cerebri-Information-Page>.

    9 Thurtell, Matthew J., et al. “An Update on Idiopathic Intracranial Hypertension.” Reviews in Neurological Diseases, U.S. National Library of Medicine, 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC3674489/>.

    10 Wani, Irfan Yousuf, et al. “Complete Ophthalmoplegia: A Rare Presentation of Idiopathic Intracranial Hypertension.” Annals of Indian Academy of Neurology, Medknow Publications & Media Pvt Ltd, 2015, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4683894/>.

    11 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

    12 Thurtell, Matthew J., and Michael Wall. “Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Recognition, Treatment, and Ongoing Management.” Current Treatment Options in Neurology, U.S. National Library of Medicine, Feb. 2013,<www.ncbi.nlm.nih.gov/pmc/articles/PMC3554852/>.

    13 Mokri, B. “The Monro-Kellie Hypothesis: Applications in CSF Volume Depletion.” Neurology., U.S. National Library of Medicine, 26 June 2001, <www.ncbi.nlm.nih.gov/pubmed/11425944>.

    14 Schirmer, Clemens M, and Thomas R Hedges. “Mechanisms of Visual Loss in Papilledema.”Journal of Neurosurgery, <www.thejns.org/doi/full/10.3171/FOC-07/11/E5>.

    15 “Empty Sella Syndrome Information Page.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, <www.ninds.nih.gov/Disorders/All-Disorders/Empty-Sella-Syndrome-Information-Page>.

    16 Panikkath, Ragesh, et al. “Intracranial Hypertension and Intracranial Hypotension Causing Headache in the Same Patient.” Proceedings (Baylor University. Medical Center), Baylor Health Care System, July 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4059569/>.

    17 Abraham, Mary, and Vasudha Singhal. “Intracranial Pressure Monitoring.” Journal of Neuroanaesthesiology,  <www.jnaccjournal.org/article.asp?issn=2348-0548;year=2015;volume=2;issue=3;spage=193;epage=203;aulast=Abraham>.

    18 Ahmed, Wilkinson, et al. “Transverse Sinus Stenting for Idiopathic Intracranial Hypertension: A Review of 52 Patients and of Model Prediction.” American Society of Neuroradiology, July 2011. <www.ajnr.org/content/32/8/1408.long>.

    19 Riggeal, Bruce, et al. “Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis.” American Academy of Neurology, 2012. <www.ncbi.nlm.nih.gov/pmc/articles/PMC3589184/>.

    20 Patel, et al. “Evaluating and treating venous outflow stenoses is necessary for the successful open surgical treatment of arteriovenous fistula aneurysms.” Society for Clinical Vascular Surgery, Volume 61, Issue 2. February 2015. <www.sciencedirect.com/science/article/pii/S0741521414014116>.