In her last blog, Melissa reported that her symptoms were greatly improved by a spinal patch. She returns with a new blog on high intracranial pressure, and ME/CFS/FM and long COVID.
This is a post on intracranial pressure and its effect on many illnesses and their symptoms. If you’re experiencing any pain or fatiguing illness, please read on. One big but simple question could save years of suffering.
Too high or too low cerebrospinal fluid pressure can cause a multitude of problems.
Each time we enter a doctor’s office, our arms are cuffed, and blood pressure checked. It’s common knowledge that high or low blood pressure can cause a variety of symptoms, yet a large portion of the medical community (and I’d wager even more of the general public) pays no heed to cerebrospinal fluid pressure.
Whether you have a severe chronic illness, are mostly functional, have an injury, or no illness at all, please take a moment to learn about cerebrospinal fluid or intracranial pressure and how it may be affecting you, along with millions of others, often without our awareness.
The skull contains a closed system of brain, blood, and cerebrospinal fluid. To maintain intracranial pressure (ICP), a delicate balance must be preserved.
Cerebrospinal fluid (CSF) surrounds the brain and spinal cord, providing protection, nourishment, and waste removal. When something goes wrong—with, for instance, the mechanism that absorbs or drains CSF, the “supply lines” into the skull, illness, or injury—pressure can increase inside the skull, the bone is unable to accommodate expansion and the brain is in danger of getting squished. This is high pressure—intracranial hypertension.
One of the body’s solutions in that case is to leak some of the fluid out; i.e. create a cerebral spinal fluid leak. In some cases, then, a spontaneous cerebral spinal fluid leak functions as a kind of relief valve to let off the pressure and prevent the brain from being too damaged; i.e. it’s actually saving us.
The anatomy of fluid flows in the brain.
Even in the presence of a leak, though, the brain squish that results from high intracranial pressure can cause neurological and autonomic symptoms (anxiety, depression, memory, processing speed, attention, gut issues, tachycardia, breathing, nerve pain, weakness, etc.). That’s apparently why many people with ME/CFS feel better after a lumbar puncture or spinal tap has relieved some of the pressure.
High intracranial pressure can injure your brain or spinal cord, limit blood flows to your brain, and cause a myriad of symptoms. Low intracranial pressure can cause an even wider variety.
(Recently, I’ve been healing from a cerebrospinal fluid leak that produces low intracranial pressure. CSF is removed from the brain through the venous sinus, but I have something called “venous sinus stenosis”, meaning the flow between my brain and neck is decreased. This narrowing causes a build-up of pressure in my veins which keeps the CSF from being properly removed from my skull, creating increased intracranial pressure (IIH).
That increased pressure makes a cerebral spinal fluid leak (which I’ve had) more likely. When my leak is present, my brain doesn’t get enough fluid, particularly when upright, so it responds to that reduction by creating more CSF. Leakers do better lying down because they get better flows to the brain (no fighting against gravity) – and lying down puts less pressure on the spot that leaks. This is not an intracranial hypotension post, but this little detour shows how the pressure can fluctuate at times. The intracranial hypotension/spinal patch post, which includes the many, many symptoms of low pressure, can be found below.
Intracranial hypertension in adults is usually defined as an intracranial pressure of 250mmH2O or above as measured during a spinal tap or lumbar puncture. An opening pressure on a lumbar puncture is not, however, necessarily a useful guide, as recent findings reveal that the pressure can fluctuate. Positional changes, medications, or the durations between spinal taps can also affect results. It’s actually common to have a normal opening pressure even if you have intracranial pressure issues. Note that your symptoms may fluctuate depending on your intracranial fluid pressure.
Acute intracranial hypertension (IH) can have a rapid, identifiable onset as the result of head injury/brain swelling or intracranial bleeding (aneurysm or a stroke) into the sub-arachnoid space surrounding the brain. In these cases, parts of the skull may be removed to accommodate the additional pressure.
Chronic intracranial hypertension (CH), on the other hand, is a neurological disorder in which CSF pressure remains elevated over a sustained period of time. Causes can include injury, illness, medication, stenosis blood vessel narrowing), and much, much more. If the cause is unknown, it may be referenced as idiopathic intracranial hypertension (IIH) or if secondary to another cause, secondary intracranial hypertension (SIH). (It was previously known as pseudotumor cerebri because it mimicked the symptoms of brain tumors, but this term is out of use.) Intracranial hypertension can be a lifelong condition and may be mild, intermittent, or disabling.
(Please don’t leave, I’m getting to the Big Question soon and it’s important.)
Like blood pressure, cerebral spinal fluid (CSF) pulses and circulates through the system via the arterial pulse wave. CSF is secreted by the choroid plexus and leaves the subarachnoid space via villi and arachnoid granulations—a filtration system and pathway into the venous system—along the superior sagittal venous sinus, intracranial venous sinuses, and the roots of spinal nerves.
Key Symptoms
Symptoms will vary. Not every person will have headaches, vision issues, or any of the less common side effects of high pressure. Assumption otherwise is the reason many cases are misdiagnosed.
- Headache, head pressure, or head pain, as with low pressure, is a key symptom of IH. Intracranial pressure headaches may not be relieved by pain medication.
- IH headache may share migraine characteristics, such as light and scent sensitivity, and worsening with exertion, cough, or straining, but migraine should not be accompanied by pulse synchronous tinnitus (see below), or the visual issues excluding the characteristic aura (temporary flashing or flickering objects, usually lasting about 20 minutes prior to migraine onset, and disappearing after head pain begins). IH headache also may not occur in one specific area as migraine does.
- The pain may be located behind the eyes, forehead, one side of the head, back of the head, base of the skull, top of the head, and maybe burning or pressure-type pain, and may be made worse by eye movement. Not everyone with IH has all the head pain symptoms. Any head pain changes related to pressure or positional changes count.
- Vision—IH can cause rapid or progressive vision changes. Vision symptoms may include gray spots, dots, floaters, or dim-outs in one or both eyes, blurred vision, or double vision. Papilledema (swelling of the optic nerve) caused by CSF pressure on the optic nerve and blood supply can lead to vision loss. Not everyone with IH has all the visual symptoms. Any vision changes related to pressure or positional changes count.
- Pulse-synchronous tinnitus (whooshing, whistling, humming, or marching noises in one or both ears that correlates with your heartbeat).
Other Symptoms
- Fatigue or sleepiness
- Lack of alertness, brain fog, memory or other cognitive issues
- Mood issues including depression, anxiety, irritability, and more, or behavioral changes
- Weakness, issues with movement or speech
- Nerve pain in the neck, shoulders, arms, upper or lower back, hips, legs, or feet
- Neck or shoulder stiffness
- Dizziness, lightheadedness, balance problems
- Numbness or tingling in hands, feet, or face
- Nausea or vomiting
- Clear fluid leaking from the nose (many assume this is only allergies)
- Endocrine issues (due to empty sella syndrome and the flattening of the pituitary gland). Note that empty sella syndrome has been proposed for both ME/CFS and fibromyalgia.
- Malaise
- Exercise intolerance.
Look familiar? I’m sure these symptoms occur in a variety of patient groups, yet rarely are they attributed to intracranial hypertension. One of my first acquaintances with these symptoms since learning about pressure issues was here, in an MS group.
It begs the question: why is this not being considered as a contributing factor in illnesses in which doctors know intracranial pressure plays a role? As far as I can tell, it comes down to this: diagnoses considered rare are disregarded because they are rare—they stay unreported and therefore continue to be considered rare—and though I have no experience in the area, it seems that changing the medical code (especially without pharmaceutical dollars behind a push) isn’t easy. This does not mean the science isn’t there. It only means the response will be slow and likely suffer painful missteps. But we can act on it now.
What does this have to do with ME/CFS or Fibromyalgia?
Once you’ve taken some time to consider the Big Question (it’s coming, I promise), it’s hard to ignore the parallels between ICP and other illnesses. In my post on CSF leaks, I explained how low intracranial pressure mirrored the symptom set of ME/CFS right down to PEM, and that some intracranial symptoms are relieved by pacing and rest because getting flat allows the CSF to resume its place in the brain).
With high intracranial pressure, the increased pressure can force cerebrospinal fluid into nerve root sheaths, causing severe nerve pain that can be relieved somewhat by gentle upright activity, as in fibromyalgia. If nothing else, these similarities could be causing misdiagnosis, and intracranial pressure should be considered as part of standard testing. IIH has been found in COVID-19, and no one can deny the similarities between it and ME/CFS and FM. Indeed, several studies and reports suggest it is present.
The Chronic Fatigue Syndrome (ME/CFS) and Fibromyalgia High Cerebrospinal Fluid Pressure Connection
In 2013, a Cambridge doctor named John Higgins found that 20% of 20 people with ME/CFS had high cerebrospinal fluid pressures. He suggested that an obstruction to venous outflows was inhibiting normal cerebrospinal fluid (CSF) flows and causing high CSF pressure.
In 2017, Higgins produced a Medical Hypothesis paper, “Chronic Fatigue Syndrome and Idiopathic Intracranial Hypertension: Different Manifestations of the Same Disorder of Intracranial Pressure?“, proposing that many people with ME/CFS actually have idiopathic intracranial hypertension. While 20% of his ME/CFS group had high lumber pressures, it was the patients without high lumbar pressures that were the most interesting.
Despite the fact that 80% of his study participants did not, according to the medical profession, have IIH, 85% felt significantly better after getting a lumbar puncture. Their headaches were diminished, they felt more alert and their energy was improved. The heightened sense of well-being and health they experienced lasted for weeks for some.
Next in 2018, Hulens produced three hypothesis papers suggesting that many symptoms in ME/CFS and fibromyalgia and other widespread pain disorders could be explained by IIH. Hulens noted that standing or sitting up causes the sleeves of the nerve roots in the spine to fill up with cerebral spinal fluid, putting pressure on those nerves and potentially causing everything from facial pain to gut and leg pain. Hulens also believes that a leaky blood/brain barrier, in conjunction with high CSF pressures, may be causing issues in the brain, including brain fog, in these diseases as well.
In 2020, a large Swedish spinal study found, depending on the test used, that between 55 and 84% of ME/CFS patients met the criteria for intracranial hypertension. IH was by far the most common spinal diagnosis found.
Just this year, Higgins reported that clearing the jugular vein of obstructions dramatically improved the symptoms of a woman with ME/CFS and asserted that “it is probable that many cases of spontaneous CSF leaks, manifesting as the syndrome of spontaneous intracranial hypotension, have IIH (idiopathic intracranial hypertension) as their underlying condition”; i.e. he believes that IH is causing the cerebral spinal fluid leaks found in some people with ME/CFS.
Higgins proposed that many people with ME/CFS simply have an uncharacteristic form of IIH which lacks papilloedema (swelling of the optic nerve) – thought to be a signature sign of IIH – causing it to be missed. He noted that several groups have found that many people with ME/CFS who do not have papilloedema nevertheless experience relief when their cerebral spinal fluid pressure is lowered during a lumbar puncture. Higgins did not mince his words regarding the failure of the medical profession to appreciate the role IH plays in diseases like ME/CFS and FM.
“Specifically, that the criteria put in place to define IIH have led to a failure to appreciate the existence, clinical significance or numerical importance of patients with lower-level disturbances of intracranial pressure. We argue that this has led to a grossly implausible distortion of the epidemiology of IIH.” Higgins
In 2014, over 60% of about 3,000 people participating in a poll on Health Rising reported that their doctor told them that they had high intracranial pressure during a lumbar puncture. Seventy-two percent reported they felt much better or somewhat better after the procedure had reduced their pressure.
Recently, Hulens proposed that high cerebrospinal fluid pressure is also causing empty sella syndrome in ME/CFS and fibromyalgia.
Other Causes
Other additional causes of increased intracranial pressure include:
- Underlying illnesses, such as meningitis, Lyme disease, Coxsackie B viral encephalitis, Guillain-Barre syndrome, infectious mononucleosis, Lupus, Sarcoidosis, Hypoparathyroidism, Addison’s disease, Behcet’s disease. Notice the potential overlaps – three post-infectious diseases and several autoimmune diseases (which could have been triggered by an infection).
- Medications (this is an extensive list, but some major actors are the cyclines—doxycyline, tetracycline, minocycline—or Accutane, Retin A and topical products and face creams, prednisone (which may cause temporarily improved and then severely worsening symptoms), estrogen, and more—Google the medicine with “cranial pressure” to check).
- Vitamin A.
- Cerebral blood clots (cerebral venous thrombosis).
- Infection.
- High blood pressure.
- Tumor.
- Stroke.
CSF pressure issues may also be aggravated by mechanical problems such as spinal stenosis (spinal cord narrowing vessel) – which has been found in fibromyalgia – and drainage issues with the arachnoid granulations, that interrupt fluid flows and which are made worse by lax dura mater related to connective tissue disorders such as Ehlers Danlos Syndrome (EDS).
Jeff Woods, an ME/CFS patient who recovered after surgery to fix his craniocervical instability, proposed that a “chronic, ongoing disturbance of blood flow and cerebral spinal fluid flow” caused by mechanical issues, which is exacerbated by exertion, causes ME/CFS. He could be right.
It is known that the longer a leak (low ICP) goes untreated, the less pronounced the orthostatic changes can become. Less clear is whether this also happens with high-pressure cases, but it’s worth noting the signs may be clear at the onset (but more difficult to untangle over time).
The Big Question!
Okay, here it is. It’s time for the Big Question. (Trust me, I’m ready for it too.)
The question of position.
If you read my post on CSF leaks, you’re going to remember the mantra of positional symptoms. Surprise! It’s the same question in reverse. (Don’t leave yet—you have to try it!)
Pressure issues may not be part of standard testing, but this simple at-home test could give major insight.
- Stand straight up or lie completely flat (spine straight, no pillow).
Focus on your symptoms. Write them down if you need to—especially given cognitive issues can be a big symptom. (If you’re bedbound, read my post on leaks first.) Think about the entire package: scan the pain in each area of your body. Don’t leave any parts out. Feet, neck, back, and stomach, but especially pay attention to what’s happening in the area of your neck and skull. Think about your mood and the general feeling of wrong or off you may find.
If you’re flat, do you start to suddenly have anxiety? Do your legs become restless or move of their own accord? Do you feel tingling or burning at the top of your skull or ears? Does your vision change? Can you read a spot of text at a distance with the same clarity as before? Are your ears ringing more? Can you feel your pulse in your ears? Is your heart racing?
- Change position, opposite of what you were. If you were standing, get flat. If you were flat, gently walk through the room.
Focus on your symptoms. Do you find relief—even minor—of any of the symptoms you noted before? If you stay in those positions longer, hours or more, does it change your response? If you’re uncertain, watch it for a week. Do you only find yourself weeping uncontrollably after you’ve done a physically demanding project? Do you feel worse after activities where you’re eating restaurant food or indulging in high-sodium meals? Write it down, track it over time, and notice every change in relation to your position, activity, and food.
And here’s the answer to the Big Question:
- Do you feel better when you lay down and stay down? Look into hypotension and leaks.
- Do you feel worse when you’re flat? Look into intracranial hypertension (high pressure).
If you may have low pressure, head to the leak post.
If you may have high pressure, read on.
If you believe you have both, the leak needs to be dealt with first.
THE GIST
- Melissa Wright returns after her spinal patch blog with a new blog on high intracranial hypertension; i.e. high cerebral spinal fluid pressure and ME/CFS/FM. (GIST done by Cort).
- Virtually every time we step into a doctor’s office, we get our blood pressure checked, yet doctors rarely consider checking the pressure of the cerebral spinal fluid that encircles our brains and courses down our spinal cord. Low or high cerebral spinal or intracranial fluid pressure can produce many, if not all, symptoms of chronic fatigue syndrome, fibromyalgia, and long COVID.
- High intracranial fluid pressure (Intracranial Hypertension) can cause neurological and autonomic symptoms (anxiety, depression, memory, processing speed, attention, gut issues, tachycardia, breathing, nerve pain, weakness, etc.). Because the bones of the brain cannot expand to accommodate the pressure, a cerebral fluid leak in the spinal cord will open up to save the brain and reduce the pressure. It will cause problems of its own.
- Intracranial hypertension in adults is usually defined as an intracranial pressure of 250mmH2O or above, as measured during a spinal tap or lumbar puncture. Pressures measured during a spinal tap, though, are not always accurate as they can vary widely within the same person.
- Idiopathic intracranial hypertension (IIH) is a neurological disorder in which CSF pressure is elevated. Many things including injury, illness, medication, stenosis (blood vessel narrowing), and much, much more can cause it.
- The symptoms produced are similar to those found in ME/CFS, FM, and long COVID. Headache, head pressure, or head pain is a key symptom. Note that this type of headache may not be relieved by pain medication.
- As with migraine, light and scent sensitivity, which worsens with exertion, cough, or straining may occur. The characteristic migraine aura is not produced, however.
- The pain may be located behind the eyes, forehead, one side of the head, back of the head, base of the skull, and top of the head, and may include burning or pressure-type pain, that is made worse by eye movement. Changes in head pain that are associated with changing the position of the head are often present.
- Vision symptoms may include gray spots, dots, floaters, or dim-outs in one or both eyes, blurred vision, or double vision. Again, vision symptoms provoked by changes in position could suggest intracranial hypertension.
- Pulse-synchronous tinnitus (whooshing, whistling, humming, or marching noises in one or both ears that correlate with your heartbeat) may also be found.
- Other symptoms include many that are found in ME/CFS/FM, including exercise intolerance, fatigue, brain fog, lack of alertness, anxiety, depression, irritability, weakness, nerve pain, dizziness, numbness or tingling, endocrine issues, nausea, vomiting.
- Why, Melissa asks, given this huge overlap, is this condition not paid more attention in these diseases? She believes that diagnoses considered rare are disregarded because they are rare—they stay unreported and therefore continue to be considered rare.
- Studies suggest, though, that high intracranial pressure and the conditions it can cause such as cerebral spinal fluid leaks, Chiari malformation, and empty sella syndrome may not be as rare as one might think in ME/CFS and FM.
- Two researchers (Higgens and Hulens) have separately been producing reports over the past ten years which suggest that intracranial hypertension may be greatly underdiagnosed in these diseases. While Higgens, for instance, found high opening CSF pressures in 20% of his patients in a small study, 80% of those without high opening pressures nevertheless had their symptoms relieved when their pressures were reduced by a lumbar puncture. Higgins believes these people also have IIH.
- Over 60% of people in a 2014 Health Rising poll of approximately 3,000 people reported being told by their doctor that they had high intracranial fluid pressures. Over 70% reported their symptoms were at least somewhat improved following a spinal tap.
- A large 2020 Swedish study found evidence of IIH in between 55 and 84% (depending on the test used) of patients. It also found evidence of increased rates of Chiari malformation – which can be caused by IH.
- Many things can cause IH (see blog) including several associated with post-infectious illness states such as infectious mononucleosis, Lyme disease, Coxsackie B viral encephalitis, and autoimmune diseases. Mechanical obstructions in the spine such as spinal stenosis – which has been found in FM – can aggravate it.
- The Big Question that this post asks involves position – specifically if your symptoms are altered by changes in your position from lying down to standing.
- A simple at-home test can help. Stand straight up or lie completely flat (spine straight, no pillow) and focus on your symptoms (see the blog for details). Then change your position.
- If you feel better when you lay down and stay down – then look into intracranial hypotension (see Melissa’s first blog on the spinal patch that helped her so much. If you feel worse when you’re flat, look into intracranial hypertension (high pressure) – and read on. If you believe you have a leak, note that the leak needs to be dealt with first.
- A diagnosis of IIH is usually established with a neurological exam, followed by imaging (likely MRI and CT). (Note that lumbar punctures may do more harm than good – and intracranial pressure can be gauged during a CT myelogram.)
- MRI findings may include flattening of the pituitary gland, which gives the appearance of an empty sella turcica. In addition, the sclera (white outer layer of the eye) at the back of the eye can appear flattened.
- Feeling better after a spinal tap or lumbar puncture is a sign of high intracranial pressure. Feeling worse for an extended period after a spinal tap could indicate you have a cerebral spinal fluid leak.
- As with leaks, it is essential to find a specialist experienced in pressure issues and armed with the most current information.
- High intracranial pressure can be treated in a relatively easy manner. Carbonic anhydrase inhibitors can reduce spinal fluid, including Diamox (acetazolamide) and the less potent Lasix (furosemide), Topamax (topiramate), and Neptazane (methazolamide).
- Outside of drugs things like reducing sodium intake, avoiding straining, heavy lifting, bending, sleeping on an incline, and others (see the bottom of the blog for more) may be able to help.
- Some more things to check for: If you feel worse—either immediately or within days –after strenuous activity such as lifting, high-stress events, after sneezing/coughing, or bending or twisting you may have a leak or high intracranial pressure, or you may cycle between the two.
- Having an unusual (good or bad) reaction to doxycycline, prednisone, or any medicine that affects cranial pressure is a sign, as is whether caffeine, salt/processed foods or B1 affect your symptoms. (Google the medicine or food with “cranial pressure” to find connections)
- Do you feel worse in the evenings or have vision or head or neck pain changes as the day progresses? Do you feel worse at different elevations or with barometric pressure swings?
- Do you find your mood shifts with your symptoms after these activities? Are your cognitive or neurological symptoms brought on by bending, lifting, twisting, jumping, straining, or Valsalva maneuver? Is some part of your pain positional?
- Do you have unusual scarring, stretchy skin, or joint hypermobility in your family? You may have a connective tissue disorder, making leaks and pressure issues more likely.
- Do you ever feel anxious or restless when lying down? This may be high pressure urging you to get upright (to ease pressure in the skull). Do you ever find relief by lying flat? This may be low pressure returning needed fluid to your brain.
- Useful links – Intracranial Hypertension Research Foundation (information on IH) / Spinal CSF Leak Foundation Physician Directory (doctors experienced in intracranial pressure issues).
Diagnosis
To diagnose IH, your doctor may start with a general exam or neurological exam, followed by imaging (likely MRI and CT).
It is important to note, again and with emphasis, that lumbar punctures may do more harm than good. Please look into the risk and understand that many doctors are not aware of the most current information regarding ICP. Opening pressure may be gauged during other procedures, like a CT Myelogram, with the proper type and gauge of the needle. A non-invasive helmet system is also apparently being developed. Though lumbar punctures may not be an accurate assessment for all pressure issues, there is an option of monitoring systems (but ideally it can be diagnosed with the least invasive tests).
MRI findings may include flattening of the pituitary gland, which gives the appearance of an empty sella turcica. In addition, the sclera (white outer layer of the eye) at the back of the eye can appear flattened. High pressure may be hereditary, depending on the underlying cause. Imaging may only be one of many tools your doctors use to determine your underlying cause.
Did you feel relief after a lumbar puncture? This may indicate high intracranial pressure. If you eventually resumed your baseline afterward, that’s expected.
Did you feel worse after lumbar puncture? If you felt substantially worse over the following weeks or months, you may not have healed and have gone into low pressure with a leak, or you may already have been leaking. (Get flat and read the leak post now!)
Chiari and Brain Sag—Congenital, Leak, or Downward Pressure?
According to studies, over time, IH may also cause an acquired Chiari malformation. Acquired Chiari (or brain sag) may also be the result of low fluid, such as CSF leaks, LP shunts, or multiple spinal taps. A 2020 Swedish study found that 17% of ME/CFS patients may have a Chiari malformation.
Per the IHR Foundation, research suggests that an acquired Chiari malformation is eight times more common in chronic IH patients who have not had shunting procedures. They also note “additionally, for unknown reasons, certain people with congenital Chiari malformations develop chronic intracranial hypertension, even after neurosurgery (surgical decompression) to remedy any obstruction to CSF circulation.”
- Find out more, including links between IH and hydrocephalus at the Intracranial Hypertension Research Foundation.
As with leaks, it is essential to find a specialist experienced in pressure issues and armed with the most current information. Don’t let the incorrect notion that pressure issues are rare prevent you from finding treatment. If you fit the profile and your physician dismisses the possibility, find a new physician.
- The official ICP diagnostic criteria can be found here.
More connections—high intracranial pressure can lead to leaks. A system under pressure is more likely to create a relief valve, blow a leak, or have issues sealing on its own. Can you see how this is all starting to tie together? An initial illness or injury that creates high pressure can lead to leaking and severe/bedbound patients. Think about your illness progression and consider any signs you may have missed.
How is IH treated?
To treat IH, intracranial pressure must be lowered. Like leaks, this can be done in a relatively easy manner. Carbonic anhydrase inhibitors can reduce spinal fluid, including Diamox (acetazolamide) and the less potent Lasix (furosemide), Topamax (topiramate), and Neptazane (methazolamide).
It is important to understand that while lumbar punctures, or spinal taps, may temporarily ease IH symptoms, spinal fluid regenerates at a rate of .3cc per minute. The body produces, absorbs, and replenishes the total CSF volume several times each day. And, as many well know, lumbar punctures carry the risk of creating a CSF leak that may worsen a person’s condition considerably. The risk is far higher than the reward.
Neurosurgical shunts are an option in certain cases but also carry more risk.
Note: it’s not recommended to take Diamox without a doctor’s supervision. It can deplete potassium or cause other serious side effects if not monitored. B1 or other drugs may also be effective in lowering pressure, but the most important step is to address the underlying cause.
Tips for coping with IH in addition to or outside of medical intervention
- Many people with these diseases increase their sodium intake to help with orthostatic intolerance but sodium also increases intracranial pressure. You might try reducing sodium intake to the minimum daily recommended 500mg and see if it helps. Raise it as you’re able (some may have a much higher tolerance). Be diligent, sodium is in nearly everything. Check your condiments and spices, and read labels and serving sizes on packaged food.
- Avoid straining. No heavy lifting, pulling, etc. Gentle upright activity only. Walking is encouraged.
- Don’t bend over if you can help it (get a stool and lower yourself with a straight spine) and avoid sitting in one spot for long periods (if you’re more functional, you may have noticed this during driving or at events with hard metal benches).
- Sleep on an incline, head elevated and neck not kinked to prevent flow.
- Some people avoid vitamin A foods. Others do well with diuretic foods.
- Watch for barometric changes or hot temps (including hot showers).
- Avoid certain medicines like steroids and cycline antibiotics.
- Avoid Retin A.
- Some people are affected by sugar or caffeine (caffeine affects blood flow in the brain).
- Avoid postures that crimp the neck (like when looking at your phone or tablet).
- Avoid tight clothing.
- Watch for your own triggers, be it food, weather, elevation, or certain positions.
- Know that it may change by factors out of your control.
I’m reposting the questions I shared in the leak post, because they’re important to ask. No matter your condition, pressure may affect you in ways you may not have realized. Even the relatively healthy can be affected by pressure changes (hello, your uncle who could predict the weather with his knee).
Do you feel worse—either immediately or within days –after strenuous activity such as lifting, high-stress events, after sneezing/coughing, or bending or twisting? You may have a leak or high pressure, or you may cycle between the two.
Have you had an unusual (good or bad) reaction to doxycycline, prednisone, or any medicine that affects cranial pressure? Does caffeine, salt/processed foods, or B1 affect the severity of your symptoms? (Google the medicine or food with “cranial pressure” to find connections.)
Do you feel worse in the evenings or have vision or head or neck pain changes as the day progresses? Do you feel worse at different elevations or with barometric pressure swings?
Do you find your mood shifts with your symptoms after these activities? Are your cognitive or neurological symptoms brought on by bending, lifting, twisting, jumping, straining, or Valsalva maneuver? Is some part of your pain positional?
Do you have unusual scarring, stretchy skin, or have joint hypermobility in your family? You may have a connective tissue disorder, making leaks and pressure issues more likely.
Do you ever feel anxious or restless when lying down? This may be high pressure urging you to get upright (to ease pressure in the skull). Do you ever find relief by lying flat? This may be low pressure returning needed fluid to your brain.
Thank you for reading this post. If you find it helpful, and if you’re able, please share your pressure situation in the comments so that the community can better see how much it affects our conditions. And if you’re willing, please share the post to help raise awareness.
Useful links:
- Intracranial Hypertension Research Foundation (information on IH)
- Spinal CSF Leak Foundation Physician Directory (doctors experienced in intracranial pressure issues)
- Melissa’s new website on intracranial pressure and diseases like ME/CFS and FM.
Folks w ED ( Ehlers – Danos ) are often treated for CFS leaks
Yes! And, interestingly, it seems like connective tissue damage worsens considerably when leaking.
Very interesting, thank you! As to ME/CFS and Long Covid we´d urgently need a better description of cranial pressure in terms of its longitudinal course: does it correlate with symptoms? Do the pressures change after exercise? During PEM? That would give us a hunch that this is not just a bystander problem but related to the underlying pathology.
In my observation there seems to be a general fluid leak during PEM, as evidenced by subtle edema in dependent extremities, hand and finger swelling etc. We do not know what causes this – Klaus Wirth suggests vasoactive substances like the bradykinins. Others have postulated fibrin/fibrinogen transudation (which may explain the hyperdensities found in some earlier MRI studies). Bhupesh Prusty has recently found a certain aquaporin in higher concentrations, this could possibly also lead to fluid shifts (just thinking out loud). Some or all of this may also happen in the brain which could explain fluctuating pressures. So, again, we´d need some systematic assessment of this finding (perhaps we´ll hear some from the intramural NIH study)?
I agree with the testing–I would love to see studies on this soon. But as for me and others with high ICP, we can actually start to notice how our pressure changes in relation to activity and sodium. It’s really obvious once you’ve trained yourself to look for it. When I was severe and leaking, I’m not sure I knew anything except that my body was heavy and screaming for me to get flat. But more functional cases can absolutely track it themselves and avoid triggers until the studies catch up.
Dr Jan-Peter Warnke (Zwickau, Germany) has done two very informative webinars on Tarlov cysts (see website Vigdis Thompson Foundation), where he explains how disrupted CSF flow can be caused by arachnoiditis. I recommend checking this out.
For patients there is Dr Forrest Tennant’s protocol to treat the arachnoiditis. To me, in combination with Wahls Protocol (diet against auto-immunity) this has done wonders.
For several years now, I have occasionally been awakened between 2-6AM with really bad dull spine pain; mostly centered at the base of my skull, but with some in the lumbar area and even causing dull pain down in to my thighs. It always seems to happen when there is a lot of humidity (I live in a dry part of CA). Sitting up helps. For the rest of the day I have horrible fatigue and brain fog. I’ve had Chronic Fatigue Syndrome for 30 years but have mostly just dealt with PEM. No doctors have a clue to what is going on and MRIs have shown stenosis. This is an eye opening article for me! I’m not sure where I will go from here, but at least I have more insight now. Thank you for the post!
Lisa! CSF production peaks at around 3:00 am. This is great indicator high pressure could be driving symptoms for you. Many people feel additional symptoms or have trouble sleeping between 1:00 and 4:00 am when they have high pressure. Try sleeping on a wedge pillow (great sturdy and adjustable ones can be found at Amazon for less than $40) and see if that eases your pain, but definitely check out the physicians directory to see if anyone with experience in this is near you. High pressure can worsen after activity as well. Your doctors may dismiss this (the misdiagnosis rate is just unbelievably high) but there are many who are aware of the effects of pressure and it can potentially be a really simple fix. I wish you luck with this!
So that’s why I wake up at 3 am (or between 1 and 4am) frequently! The other theories, blood sugar, cortisol, etc didn’t seem to fit. The info on salt is helpful to because when I had PEM I increased my salt intake and never resuced it when the PEM went away. Also explains why I need large does of B1/B2 to avoid fatigue. The diet info on Google was helpful too, although with MCAS it wants me to eliminate half of my already limited diet. ;-(
Yes! I love how this all falls into place when we just ask the right questions. And it’s so easy to test with lifestyle adjustments like sleeping on an incline or cutting salt for a few days. I’m so hopeful this helps you!
Some of the foods I reacted to when leaking ended up being diuretics, which lower pressure. Vitamin A and sodium are the big ones for raising pressure if that helps. (Watch out for Vit A enriched foods like milk and cereal.)
I had POTS symptoms, but the recommendation of a raised headboard was wrong for leaking. Because the POTS was secondary to a leak, I needed to be flat and have tons of salt and water. Now that I’m high pressure I need to be inclined and have no salt and less water.
Thanks for your help in figuring this out! There are no DRs on your list north of NY. I may be fluctuating between high and low pressure so I have a lot of work left to do, besides trying to figure out diagnostics which is tough when you have MCAS and want to avoid contrast media if possible. Your success gives us all hope. Thanks you!
If you join one of the Facebook groups, they may have a recommendation for a doctor in that area who’s not yet on the list. IH doctors are a little easier to find than leak doctors, but for anyone dealing with long-term symptoms it’s best to have someone who has a bit of experience. I’m not sure how reactive you are to contrast, but some of the leakers have a milder reaction and are still able to get testing with contrast, as long as they work it out with the doctors beforehand.
I hope you find an easy fix! Cycling between high and low could mean a leak with high pressure or could mean there’s some sort of interruption in flow.
Thanks! I’ll give this info to my PCP who is at least receptive to suggestions. Problem is getting an appt at overworked, understaffed, medical facilities. I haven’t been able to even get Scheduled for a Cat scan in over a month, never mind a consult to talk to someone beforehand. Hate to ask but can you suggest a couple of good FB groups. Too many low quality sites out there.
I do not have experience with the other groups for IH but I know some of them are more helpful than others. I like the leak group, and many in there are extraordinarily helpful and have experience with high pressure too. They may be able to recommend a good doctor and/or a high pressure group.
https://www.facebook.com/groups/csfleakers/
I understand completely how difficult finding medical care is. I hope that soon this information will be more widespread and our PCPs will request this testing as easily as they do others. For me, the hardest part of the entire ordeal was finding the right doctor. But once I did she ordered the MRI, CT, and performed the CT myelogram herself and within a few weeks.
Thank you Melissa! I’m so grateful that you’re so willing to share this info. The wedge pillow sounds like a good idea and I’m going to check out the physicians directory. My best to you!
To Lisa in California, possibly seek out the csf clinic of Dr. Wouter Schievink at Cedars-Sinai hospital. He has several videos on YouTube about csf leaks.
Than you Shari! I will do that.
This is an excellent article. I have been previously diagnosed with lupus and intracranial hypertension. I’m currently experiencing the csf leak from the left nostril and will have new mri/mrv scans in a week. I believe that I’m also mildly hypermobile and a previous mrv showed a left transverse sinus cerebral vein to be constricted and the area to be hypoplastic. I’ll be seeing a neurosurgeon this month for stenting evaluation of the transverse sinus vein and most likely having sinus surgery for the leak. The hypermobility characteristic/Ehlers-Danlos seems to be a common link for this syndrome. My symptoms are exactly like you describe in the article and much like a chronic fatigue patient.
Good luck, Shari! Thanks for sharing your experience 🙂
Thank you so much for sharing, Shari! I feel like there are so many of us with these very similar issues and it’s incredibly helpful to see them laid out. I hope you have an excellent care team and that your procedure goes well. I would love to hear you’ve seen great improvement afterward!
Hi Shari, could you share an update since this post on how you’re managing any intracranial hypertension?
Really fascinating, Melissa! And very clearly written, which my non-science brain appreciates. I am going to pay more attention to my upright vs. supine states. Laying down for a rest usually makes my fatigue better within 10-20 minutes, but I’ve noticed that if I linger too long in bed in the morning, I usually feel better after I get up and gently move around. My nose has also been running non-stop for months now and I indeed chalked it up to spring allergies (which it still might be; we’re not out of the woods yet in NY).
I am coincidentally heading for an MRI in two weeks that is a follow up to 2020 and 2021 MRIs that both showed a white hyperintensity (lesion) that I didn’t have prior to COVID in a 2018 scan. Is there anything that a regular MRI would show if I had low or high intracranial pressure?
Also, any tips for finding a neurologist who will consider any of this? I am now under the care of a neuromuscular specialist due to small fiber neuropathy issues, and she’s lovely and open to hearing things, but I’m not sure she’s the right specialist?
Thanks again for this eye-opening piece!
Oh, also – does anyone know what kind of specialist might look into an EDS diagnosis? I’m not hyper mobile per se, but I have a shoulder injury that just won’t heal, and it cracks and moves around a lot. I also have super stretchy/crepey skin that’s gotten worse over the last year. I’ve been chalking it up to perimenopause (I’m 47), but it’s odd how fast it’s come on.
EDS can be diagnosed by a geneticist but there isn’t a marker yet for the hypermobile type. This link might help: https://www.ehlers-danlos.com/healthcare-professionals-directory/
The skin connection is huge. It’s believed leaks damage connective tissue and/or worsen an existing connective tissue disorder. My skin went from seeming normal to unbelievably stretchy and weird. Like you said, crepey, and suddenly I could stretch my neck skin past my ear. After I was patched, it’s gone back to its pre-leak elasticity.
To Amy: I believe Dr. Susan Levine knows a lot about EDS and she’s in NYC. She probably knows doctors in the city who specialize in it and be able to diagnose it. I believe there’s a specialist up at Columbia Presbyterian people are referred to.
She has been my doctor for almost three years! I’ve never had a chance to ask her about this, but I will. There are also a few docs in the EDS provider directory who are local to me.
Yes! This definitely sounds like something you should look into. The tingling/pricking sensations can be from pressure as well. There are imagine findings to look for in both high and low pressure.
Here is a link to possible findings for high ICP: https://ihrfoundation.org/what-is-ih/signs-of-ih
Here is one for low: https://spinalcsfleak.org/what-is-seeps/
It’s important to remember the tech reading your reports may not know to look for these signs and that the absence of them doesn’t rule out pressure issues.
There is a physician’s directory here for leak specialists, all of whom should understand high pressure symptoms as well: https://spinalcsfleak.org/directory/
A lot of us didn’t have luck with neurologists but it really depends on the person not their specialty. This is new information and so they have to be willing to look at the updated guidelines. Many of us see interventional radiologists.
As for the fluid, it could be high pressure forcing non-CSF fluid out or it could be actual CSF fluid draining, depending on what’s happening inside your skull. It could also just be allergies, but if you can maybe pay attention to when it happens that might be helpful. Some will notice it happens more when bending over or with warmer temperatures or some other trigger that doesn’t apply to allergies.
I truly hope this helps you!
Amy, you also need to get an mrv done in addition to the mri to look at the cerebral veins.
Thank you Cort! Another great article.
Please could anyone with CSF leakage tell me if you ever have fluid and not wax drain from your ears? Mine fill up with liquid just before a migraine.
Yes, that can happen with leaks. If you’re having this along with other leak or pressure symptoms, I would recommend seeking out a leak specialist. https://spinalcsfleak.org/directory/
This site can tell you a little about the difference between cranial and spinal leaks, but it’s possible to have both at the same time. https://www.mayoclinic.org/diseases-conditions/csf-leak/symptoms-causes/syc-20522246
Thanks Melissa!! Great resources.
Dr Perrin has found that ME/CFS patients have retrograde lymf flow and dysfunctional glymphatics. Is it possible to see a connection to the higher intracranial pressure? Anyone has a theory around that?
I do know drainage issues can cause IH but I’m not sure anyone has specifics. A lot of these issues have been disregarded until recently. I tried to look but I’m not able to sort through it all right now. Perhaps this will have insight: https://link.springer.com/article/10.1007/s00018-020-03706-5
Thank you, I had a peek at the links also. Conclusion is: nobody really knows. The knowledge on brain drainage is new and incomplete.
Melissa – My IR (interventional radiologist) wants to do a pressure study on my internal jugulars. How do I know if he’s testing for the right thing? My CT shows IJ’s draped over C1’s transverse process’s and the right styloid compressing the IJ between the atlas TP. I’m worried the pressure will be normal and he may not want to look into other potential causes of my symptoms over 25 years. Thoughts or ideas please? Thank you! And so happy for you.
Hi, Kat. I’m sorry you’re going through this. I’m not as experienced with the pressure studies as I am with leak testing. If the pressure is normal or if you believe you fit the profile and this IR dismisses your symptoms, I would recommend seeking a new one from the directory here: https://spinalcsfleak.org/directory/
Meanwhile, you could seek advice in one of the IH communities–perhaps in one of the Facebook groups specific to these conditions. (Is this called Eagle’s Syndrome?)
This research may be helpful: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8106058/
Hi Kat. I just saw this group recommended in the spinal CSF leak group on Facebook and thought it might be helpful for you.
Jugular Venous Outflow Disorders
(Cerebral Venous Congestion)
https://m.facebook.com/groups/3737591956275670/
Melissa – Thank you.
I believe as we most likely have some form of eds, acquired or not, that the normal pressure measurements don’t apply to us
I think this is extremely likely. The changes in my connective tissue when leaking were shocking. And it’s not just our skin. My veins went lax and I developed an aneurysm in the center of my palm that is apparently exceedingly rare and limited to electricians who use screwdrivers daily. But after being patched everything tightened back up. Which means pressures taken while having a pressure condition may not be “normal” by definition.
Thank you, Melissa and Cort! Great article. This hits home for me as I’m sure it does for many others. I always wonder how my mom and both of my sisters—so 4 of us total—all have these exact same symptoms/issues. My sisters have been told they have EDS so maybe that or something else is the underlying hereditary condition.
My one sister just had a brain MRI done after she did something to severely exacerbate her existing constellation of symptoms such as numbness, vision problems, hypertension, headache, etc, etc, accompanied by severe neck pain (more than normal pain levels). No contrast, but they did find a lesion on her brain along with three cervical areas causing pressure on her spinal cord. (We already have stenosis issues, MCAS, EDS, POTS, autoimmune, etc.)
As a result, she’s scheduled for epidural blocks along these cervical areas along with a referral to the MS Clinic (which will likely order a CSF puncture).
So… yes, this article really hits home for us. Any thoughts, comments, suggestions, etc would be much appreciated.
I completed your “question” test and immediately notice all my ocular nerve pain completely resolves when I lay flat. I feel better overall lying flat. After all these years I’ve never taken the time to notice or test this. As you recommend, I’ll have to do a deeper analysis but you’re definitely on to something.
I’m so grateful you’ve persevered in finding your cause and achieving subsequent relief from your symptoms. And I thank you very much for sharing your story and going steps further to help us as well.
If you don’t mind me asking, I get the impression your procedure has left you with a new type of pressure change that you’re now trying to navigate. Am I correct in this assumption? If so, how are you managing? Will you need repeated treatments or is this expected to be a one-and-done procedure?
I’m so happy to hear this may help you and your family find answers! There are truly so many potential causes that the right imaging can locate and once we start looking, it almost always turns out that we’re not the only ones we know who are affected.
My family was unaware of any existing connective tissue or pressure issues until we learned about leaks. Our cases were mild and we’d made adjustments in our daily lives without being aware of it. For instance, at family gatherings we would often stand around the kitchen talking instead of settling into sofas like my in-laws. None of us could tolerate idleness for long. Once we learned about CTD and high pressure, the clues just kept piling up.
Looking back, it’s clear I had an existing high pressure condition that likely contributed to my leak. I can remember events like my vision going black once during a foot race and having issues with motion sickness in cars even as a child. Crying would exhaust me. I often had signs of illness following a physically strenuous event. Separately, those things always got blamed on something else. My neck pain attributed to a car accident in my 20s, my foot pain and mild swelling my calves, everything mild blamed by specialists on some other factor. They don’t have all the information and so can’t connect the dots.
But my condition is mild enough that it can be managed by a low sodium diet and avoiding triggers like straining where possible. Some people need shunts or surgeries to correct a mechanical issue.
My mother has had back and neck pain issues for years and had put off surgery for stenosis (in her lumbar spinal bones, recommended by a neurosurgeon who dismissed the possibility of IH) to help me get through my post-patch restrictions and found that her pain disappears when she follows the salt, bending, and incline tricks. She discovered the surgery they recommended likely wouldn’t have resolved her pain because it wasn’t what was causing the high pressure (and she knows now reducing the pressure resolves the pain). Now that she’s watched it closer, pressure was also affecting her cognition and mood.
After my patch I went into rebound intracranial high pressure, which is a (usually) temporary condition where the body has been used to producing a much higher volume of CSF and when the leak is patched suddenly, it is a little slow to level out to normal production. During that time I experienced extremely high pressure and though it was manageable with Diamox, there was a period where as they adjusted my dose I could see what truly high pressure as like. The symptoms were *incredibly* intense. (Honestly, I find it baffling that the medical community doesn’t give more credit to the potential effects of having your brain smashed.)
So, based on my experience, I would recommend seeking input from someone familiar with pressure issues. There’s a great directory in the links that lists doctors who are confirmed to be using the best practices for leaks and those will be familiar with high pressure. I truly hope this brings your answers and believe it’s affecting all of us to some degree.
So helpful to read your story and the signs that later became obvious. Thank you so much! So I’m understanding your article correctly, if feeling better while lying flat, that means hypo, so low pressure, and not hyper – is that correct?
In general, yes. There are some flow issues that may complicate this and some people can cycle between high and low, but if you can start watching when symptoms occur and when they ease, and in relation to what activities or potentially food or medicines that affect pressure, you should be able to get a good idea after a while.
It can be really difficult when you’re severe or have a lot of symptoms happening at once, because only part of them may change. (This is admittedly easier to discern in more functional people.) But think of low pressure or leaks mechanically: if you hold a nearly empty water bottle upright, no water reaches the cap. If you lay it on its side, the fluid can reach the cap (or your head in the case of CSF fluid).
Someone in high pressure may feel improved by resting, but will generally need their head or upper body raised to avoid that additional fluid pressure in the skull.
Finding relief totally flat is an indication of low pressure or leaks.
Feeling worse totally flat usually means high pressure.
In regard to treatment for pain, I don’t want to overstep my advice, but I have a cousin in a similar situation to your sister, but with low pressure. Her neurologist and neurosurgeon keep insisting she let them treat the pain first (with invasive procedures) but it doesn’t help because they’re just bailing tiny cups of water out of a rapidly sinking boat with no regard for the hole. It has cost her months suffering when in many cases a patch can resolve pain immediately.
There are a lot of pain clinics and doctors who only treat the symptoms without searching for the cause. I don’t know the specifics of her case but I would love to see her with a care team who has knowledge of pressure issues and could potentially help her address that. Dural punctures in anyone with EDS is riskier than their stats reflect.
I want to echo all the gratitude to Cort and Melissa for raising these issues! My daughter suffers from EDS, MCAS and POTS and I can’t even begin to recount all the bizzarre and debilitating symptoms she struggles with. The idea of intracranial hypertension might tie a lot together. But none of the diagnostic or treatment options will be “simple” or even possible for her, given reactions to contrast and electricity. Travel to a Leak Specialist will be another huge obstacle.
I hear multiple warnings against lumbar puncture, but all the imaging will be invasive for her as well. It’s not even clear she can tolerate the patch material. Other than reducing salt and using diuretics, what options are there?
Also, I am curious you don’t ever mention seeing an opthamologist for diagnosis?
So we are feeling both excited and frustrated. Am I missing some options for treatment? Many, many thanks.
Hi Jane. I’m so sorry that you and your daughter are going through this. I know it can seem overwhelming, but many, many spinal leakers also have MCAS either due to the leak or are leaking due to MCAS. Most leak specialists do have a protocol for MCAS patients and depending on the severity of her reactions, they may move forward with contrast using those protections or they may decide to treat without contrast or invasive procedures. It really is very case-by-case. The good news is that understanding what’s happening is a huge piece of the puzzle and can not only lead to better quality of life now but prevent future medical missteps.
Many have patches using only their own blood, no fibrin glue and no additional punctures in the dura (the blood is placed overtop a leak or done blindly and no new hole is made in the dura), should she be leaking. If she has high pressure and does not have a leak, they may be able to do non-invasive tests and/or lower the pressure using medication. It does hinge greatly on getting the right care team, and I hope you’ll check the physicians directory for that.
You’re correct that I should have addressed eye exams to check for papilledema. However, it’s important to note the lack of papilledema or vision issues does not negate a diagnosis intracranial hypertension. New research indicates that many with IH have no sign of papilledema. Many of us do have floaters or vision changes but no other eye issues. Your doctor will decide whether those tests are necessary as they go through symptoms and illness progression. There are so many factors to consider and no case is the same, so finding the right care team with the most current information and knowledge of MCAS and EDS is critical. They are out there.
I understand completely the concern regarding travel. Some providers offer telehealth for visits that involve no testing and some may consult with your local care provider. If you’re able to figure out ways to work with her pressure, that may help. Any movement was horrid for me, but I found that placing a wedge pillow upside down in the front reclined car seat (feet on dash) allowed me to stay flat and cushioned enough to make car rides bearable.
I hope that pursuing care for this will ease her symptoms now and prevent further decline but I understand the logistics of it aren’t ideal. For me, the more time passed, the worse my baseline condition became. My sensitivities and reactions were mild in comparison, but they did mostly resolve (I still skip chemical preservatives but that may be do to with high pressure) and my skin has gone back to normal–so she may see improvement in even her MCAS and EDS by addressing the pressure issue.
I hope this helps. (Also, just to note, a leak specialist will likely only do a lumbar puncture if they’re already making a hole as part of another test. My opening pressure was tested during the CT Myelogram and the CTM was only used because they were confident I had a leak.)
Honestly, Melissa, I have no words for your generosity of time and thought. We are so grateful. I will share this with my daughter and likely respond with more questions. The first is whether this is likely to keep happening over and over given the connective tissue defect/EDS? Thank you again!
I spent so much time on “cures” that never had a chance to work, for no reason other than because a leak is invisible. But it isn’t invisible if we know what we’re looking for. I hope this saves that wasted time and energy and can help your daughter the way it helped me. Please ask as much as you need.
As for the connective tissue issues, it depends. I had mild signs that became extreme during the leak and returned to normal afterward. Some people have severe signs before leaking and find those also get much worse and that they have more comorbid conditions when leaking. Some have POTS with no sign of CTD that resolves after patching. I would love to see more data on this but right now it’s pretty sparse.
It seems to me that almost everyone with CTD gets worse while leaking and that it can take more time and care to heal. Someone with severe EDS may be more likely to reopen a leak or to create new leaks just because their dura is more delicate (this doesn’t mean they shouldn’t treat the leak on the basis that it may happen again, as leaks have severe side effects). Others have one leak and never have trouble again. Most of us make lifestyle adjustments to lower our risk of recurrence but I’ve seen a few in the groups go right back to weightlifting or whatever activity caused the tear in the first place.
It’s impossible to know, but it’s certainly easy to take precautions once you learn what activities are riskiest. I can promise that knowing the signs of high and low pressure will help manage her treatment and improve her quality of life. And with time it will become much easier to recognize those signs and know how to react to them. It will help prevent missteps as well–for instance unnecessary lumbar punctures or medicines that may raise pressure–when an alterative is available.
It’s possible she may never have the issue again once treated. It’s also possible that she may have trouble with it her whole life. But at least you will be armed with the knowledge to make the best choices.
In most cases, though, this is not an ongoing issue when a person has the right care.
To clarify, the tips for dealing with pressure are different for leaks (low pressure) than for intracranial hypertension (high pressure). Low pressure should be flat and more salt and water and high pressure should mean her resting on an incline with less salt and water.
It can be confusing which is which when someone is severe or when they’re cycling from high to low, but if she’s seeing any low pressure positional symptoms then there is likely a leak and low pressure tips are going to be the most important.
I know it can feel impossible to trust in doctors but I hope that when you speak to a leak specialist they’ll be competent and aware of all of the contributing factors and how to best manage them and it will ease your mind. My mom and I cried when we first talked to our specialist because she just got it. And this is a new field so many of them are excited to learn and help.
Hi Melissa,
From your article, I believe you went to the CSF Leak Clinic at Stanford? I have been trying to reach out to Dr. Callen to have a conversation about my daughter’s particular situation (chemical/electrical sensitivities, inability to tolerate contrast agents) just to see if it is remotely possible he can help her under these circumstances and she should endure a 12 hour car ride. They will not do Telehealth for out of state patients. I cannot get through to him, too many gatekeepers, but from the YouTube videos he appears to be seeking patients and to be a compassionate person who could tell us if her symptoms fit and there are other alternatives for imaging. Do you have any idea how I can reach out to him directly? thank you!
sorry, I meant to say Ian Carroll.
Hi Jane,
Dr. Carroll used to have an email address available for patients but as more and more people discover this to be the underlying cause of their illness, leak specialists are becoming overwhelmed and guard their time and access a little more strictly. I do not know a way to contact him directly.
I actually saw a doctor in the Midwest, not Dr. Carroll (he’s referenced so much here because he has leak resources available online). My doctor is currently only able to telehealth with in-state patients but the directory of physicians does list the email addresses of many leak specialists who may be able to help or to consult with a local care provider for ways to look further into this without exacerbating her symptoms.
The directory can be found here:
https://spinalcsfleak.org/directory/
I’m not sure where you are located, but I hope there is someone nearby with knowledge of leaks. When I went in, I was dealing with light, sound, and chemical sensitivities and my team took great care to limit my exposure and be prepared for a reaction (but I did not have a reaction in the end). I hope, truly, that you’re able to reach someone who can help.
Another word of caution, especially to the ME/CFS community: this “test” is laden with confounders (like POTS or decreased cerebral blood flow while upright) so it may not be specific for the assessment of intracranial pressure.
The questions are only meant to bring attention to whether there is a positional element. But it should be noted that POTS can be secondary to high intracranial pressure—as mine was, as well as many others with leaks or IH. The tests of sleeping on wedge and adjusting sodium may make the POTS element more clear for some people. Between these two indicators, this has the potential to determine whether someone needs imaging for IH or leaks. And if they do have a blood flow issue, that could affect intracranial pressure, as the two must remain balanced in the skull. So I hope that instead of discounting any of these issues, people can take a moment to look more closely at them.
Hi Melissa,
Thanks so much for your article via Cort. I just have a question….so does having high intracranial pressure actually cause empty sellar syndrome? I ask because after having had a mini stroke six years ago (and more since then also), I had a CT scan of the brain and the radiologist reported, as a side point, that I had >50% empty sellar. My doctor said that it wasn’t a cause for concern. But I have always wondered about it since then. I have had ME for 30 years. What would you suggest? That I get further testing?
Thank you 🙂
High intracranial pressure can cause empty sella and there are reported cases of it resolving after treatment for IH, so I would definitely look into this. Most doctors are unaware the research on IH and will dismiss it out of hand. It’s clear that IH can and does cause major symptoms so at the very least this may lead to improvements in your quality of life. What’s more, IH could be the cause of the strokes, so finding a care team who takes this seriously could be huge. I hope it’s your answer!
Hi Melissa, We spoke back & forth on your last blog post. I’m going down to Duke this month for a CT Myleogram. I live up in New England. I have EDS, POTS, MCAS, ME/CFS. None of my doctors in Boston believe I have pressure issues. My MRIs have been normal.
Benzodiazepines have helped ease my pain and other symptoms. Have you seen this in the Leak or IIH community?
Also, I was started on Topamax for “migraines” last month. I went downhill with intense “leak” symptoms when we increased the dose from 25mg to 50mg. This blog post made me realize why that might be happening!!! We are tapering me off it now.
It does seem like I swing between high and low pressure. And that’s why it’s been so hard to nail my symptoms down with doctors. They want it one way or the other. And they want it textbook.
A quick internet search found this: “Hypnotics and benzodiazepines decrease ICP due to decreases in cerebral blood volume.” here https://pubmed.ncbi.nlm.nih.gov/8592968
So you may be in high pressure when you’re experiencing pain. Topamax or any of the ICP lowering drugs will absolutely drive you into low pressure—leaking or not—because that is their job.
I’m excited you’re fitting these pieces together! Based on my experience and what I’ve seen in the FB groups, I would tell anyone to set up an appointment with someone who has pressure experience ASAP. It’s so much more efficient with less possibility of missteps than educating one’s current doctors who aren’t quite convinced. So I’m thrilled to hear you’re headed to Duke. Some people say it’s hit or miss there depending on the doctor, so while I hope yours is amazing, don’t give up if these adjustments make a difference for you. There’s definitely something there. And perhaps meanwhile the tips and tricks can show you new elements. Like maybe head or neck position affect symptoms or you’re worse when eating leafy greens. It would be interesting to see if sleeping on an incline changes your symptoms. These clues can maybe help your doctors as well.
In any case, I’m so hopeful for you! Best of luck with your appointment and I would love to know how it works out!
Does anyone know of professionals or doctors in the UK who understand this line of enquiry….
There are a few in the UK on this directory: https://spinalcsfleak.org/directory/
Does anyone know of any Dr’s/specialist also in Australia who understand this line of enquiry please?
Hi Kim. I’m sorry to say there doesn’t seem to be a great deal there. You can try reaching out on the international CSF leaks Facebook group but with a quick search I only found reference to Prince Alexandria Hospital (noted by the person treated there in 2020 that the hospital did not have much experience with this) and that they were trying to get in with a Dr. Parker in Sydney. I wish I could be more help.
Omg ! Thank you Melissa and Cort ! I can definitely relate ! I’ve suggested to two different neurologists that my worsening symptoms ( constant head pressure, throbbing headache, crushing fatigue ) were related to increased ICP….only to be dismissed without discussion. With EdS and documented venous stenosis and venous congestion , I thought it made perfect sense ! Now …searching for new neurologist.
I hope you can find answers! I had been to countless doctors over the years and was continually dismissed and misdiagnosed. The difference between the way those doctors look at our symptoms and the way one who specializes in pressure issues does is just astounding. Highly recommend checking the physicians directory if you’re able to get to one of those!
In the blog, Melissa states: “One of the body’s solutions in that case (high CSF) is to leak some of the fluid out; i.e. create a cerebral spinal fluid leak. In some cases, then, a spontaneous cerebral spinal fluid leak functions as a kind of relief valve to let off the pressure and prevent the brain from being too damaged; i.e. it’s actually saving us.”
I question whether patching the leak is the best solution in the long run. I think it is more important to address the cause of the excess CSF and treat that.
In the beginning of ME/CFS, I had horrible headaches. These lasted for at least 10 years and then mysteriously disappeared one day. If I had read this blog, I would have immediately thought that ICH might be the cause, but this was obviously not true.
Spinal taps, lumbar punctures and MRIs with contrast are not benign procedures. I would recommend caution before embracing changes in intracranial pressure as the cause of ME/CFS or Long Covid.
Longterm leaks can leak to brain sag and extreme issues. I understand that treating the high pressure is important, but I perhaps haven’t illustrated on this post exactly how damaging leaks can be.
Generally, doctors will patch the leak first then address high pressure to prevent future issues. This is why I caution against unnecessary lumbar punctures.
If you had headaches for so long and then suddenly one day did not, it is possible you had high pressure and then opened a leak, relieving the pressure inside your skull (but not resolving the underlying issues).
Do or did you have further symptoms other than the headache afterward? Did they increase with time? As someone with experience in leaks and pressure, the sudden cease of headaches to me seems more obvious it could be IH and leaks, not the contrary.
I hope you’ll consider looking further into the mechanisms behind pressure and how they may apply to your case.
Melissa,
I am so happy that your treatment has helped you, but extrapolating your situation to so many other conditions, is troublesome.
My ME/CFS was first diagnosed by two immunologists in 1984. My original diagnosis was immune deficiency and chronic encephalopathy with immune cell tests that supported this diagnosis. I was so ill that I was originally treated with a drug obtained on orphan drug status from Germany.
When my original doctors retired, I was a patient of Dr. Paul Cheney for over 20 years until his death in 2021. He and his partner were the first to draw attention to the outbreak of ME/CFS in Incline Village.
There is a lot to learn about the origin of ME/CFS and while some readers of this blog may have something else that would be helped by your suggestions, for others, it could be a waste of time and resources.
Please read Osler’s Web by Hiliary Johnson. It is a long and detailed account of the beginning of ME/CFS in the early 1980’s.
You can also learn a lot by joining the Facebook group set up by Dr. Cheney’s former patients: Remembering Dr. Cheney
Thanks, Betty. I’m only sharing this information in the hopes that it’s helpful to others. Perhaps pressure issues will be discovered not to be the underlying cause or even a portion of the underlying cause, but certainly the research indicates pressure is involved and existent.
Both high and low pressure can create a great deal of symptoms and regardless of onset, this aspect can be treated to improve quality of life. I cannot consider that a waste of time, as I was unable to walk or even some days speak due to my own pressure issues.
I do not take your experience lightly and I appreciate your comments. I have this book added to my research from your comment on my last post and I do want to learn more about ME/CFS.
I only hope that others will take a moment to learn about IH and leaks because from my experience, it could be huge.
I do not know anything about chronic encephalitis, but I have seen it often noted as a cause of IH. I hope, truly, that these connections can be helpful to all of us.
“Intracranial hypertension (IH) is a build-up of pressure around the brain. It can happen suddenly, for example, as the result of a severe head injury, stroke or ruptured brain aneurysm. It can be caused by a condition that affects your brain, such as a brain abscess, brain tumour, meningitis or encephalitis.”
https://www.nhs.uk/conditions/intracranial-hypertension/#:~:text=Intracranial%20hypertension%20(IH)%20is%20a,brain%20tumour%2C%20meningitis%20or%20encephalitis.
Is there a research group that needs patients to test this theory for those with CFS?
If so, who and where?
I would love to see this but I have not heard of any coming soon. Most of the information I’m seeing is coming from the leak specialists who are performing patches and surgeries to leaks, tears, and other more complicated issues every week, as well as finding and repairing underlying causes of IH.
I hope that as people who follow the blog look into this, they’ll share their stories and we can see more and more data from that.
It’s very much worth pointing out, that Melissa fit the classic ME/CFS ‘personality’.
As she described herself: “Always busy. Unable to sit idle, constantly thinking or doing or making, then crashing into bed at the end of a long day. No matter the pain or fatigue, I pushed through.”
THAT’S what causes ME/CFS. That’s what led to her spinal leak(s). And if you’re newly diagnosed, or even later on in your illness, changing that fight or flight dominant state, to a rest and digest one — will be of HUGE benefit.
Is there any evidence that chemotherapy causes ICP? My husband had Hodgkins Lymphoma 19 years ago. He was diagnosed with ME/CFS 3 years ago. He also has severe depression now. We have zero support from our doctors. He sleeps 17 hours a day but not good quality sleep. This week he has had ‘soreness’ on his head and neck (can’t be touched) – he also has a lump behind his ear, which is openly weeping and a large lump on the back of his head. We went to an emergency appointment yesterday – the GP prescribed cream and then did a long speech on exercise and counselling. It left him exhausted. He gets regular headaches, blurred vision, pain in his back, kidney area. He is also diagnosed with POTS. His eyebrows have almost disappeared (thyroid blood tests okay); his skin is now elastic, stretchy around his ears and neck. He’s lost lots of weight. I’d welcome any thoughts/advice.
Yes, yes, yes. This is an extremely common side effect of certain cancer treatments. I should have said so in the post. I’m so sorry–every medical practitioner you’ve seen should have known this. I will never understand why so many doctors don’t seem to credit that having our brains crushed can make us feel unwell, but it’s particularly unacceptable in the case of cancer patients because at the very least it should be investigated to be certain it’s not being caused by a brain tumor.
In his case, I would think it likely the treatments led to increased intracranial pressure. I would recommend finding a doctor who takes it seriously (off the physicians directory below if you can) and please don’t give up if the first one you try dismisses it. It may be best to focus on the symptoms of headache and vision issues so that they can make an easier diagnosis because the hair loss may confuse them–a lot of the more unusual symptoms are not commonly known and the POTS connection was only discovered this decade.
I would also verify that any of the medicines he may be taking now do not contribute to increased ICP (those can sometimes be switched for other medications that do not). You can Google the medicine with “intracranial hypertension” or “cranial pressure” to check. Check the cream they gave him as well–many topical creams and steroids can increase ICP. If the lump is weeping clear fluid, that may be a CSF leak and a risk for meningitis.
This probably all sounds scarier than it is. High pressure can be treated very easily in a lot of cases and it’s possible some lifestyle adjustments and/or pressure medication will have him feeling greatly improved very quickly. You can try some of the tips to see if that gives him any relief while you wait for doctor appointments. The weight loss could be also be because of the appetite or nausea side effect of high pressure.
My mother and father both went through cancer treatment, so I understand how difficult this can be to navigate. I hope this is his answer and I wish you luck with finding a good care team.
https://spinalcsfleak.org/directory/
Coming back to say this could be either a spinal or cranial leak due to high pressure from the treatment (high ICP can cause leaks and over time spinal leaks can lead to brain sag) or it could be high pressure, depending on what’s happening with him. I have a post on leaks in addition to the post on high pressure, so please do look at both to determine whether he’s better resting on an incline or flat and if you should try for more hydration and salt or less. If he’s leaking, they can treat that via blood patch usually and then they can address the high pressure.
Thank you so much for your reply. I will look into this further. But, for now, I wasn’t happy with the doctor’s diagnosis of dry skin so we went to the Minor Injuries Unit at a local hospital (we are in the UK). A lovely nurse took a photo of the swelling behind his ear and head and sent to an ENT doctor, who diagnosed Shingles. He started on Antivirals straightaway. Next day I phoned our surgery and asked to see someone as I felt we needed more support and a proper diagnosis. Saw a nurse, who said it might now be infected; gave us antibiotic cream. He was in a terrible state. Hoping it will all calm down soon. Viruses keep cropping up for him; I believe his immune system has been compromised since birth and the cancer treatment exacerbated this. I am feeling desperate about his future now. Thank you for taking the time to reply. x
Thank you so much for this, life makes sense now. I had M.E after glandular fever but coped with it for 10 years before having a brain injury from a car accident, after that all hell broke loose and the med world wouldnt help at all. My resting heart rate was 110-120/min and they wouldnt even take my heart rate. I have been having some gentle chiro and physio and the improvements have been surprising and very welcomed but I have hit a plateau and I really think it is a chiari and Fluid pressure issue. I will never get our med world to help with this but I can do things myself now to help. Thank you
I’m so sorry that you’ve been unable to find the right care. I hope that soon this new information is caught by some drug company or someone who will actively raise awareness and get movement for the millions of us dealing with symptoms from pressure.
And I’m thrilled to hear it’s potentially an answer for you! Once I understood how pressure worked *everything* made sense. It allowed me to research and make changes that made a real difference in my symptoms. Knowing what was happening saved me, truly, and I hope it can bring you massive improvements at least (and someday, hopefully, a cure).
The “diagnoses considered rare are disregarded because they are rare” hit home for me. When I first discovered I was celiac, around 15 years ago now, I was told it was a rare disease, so I couldn’t have it. Oh, and only Europeans had it, they didn’t have it in the US. Both my parents are European, but that was considered irrelevant. Now, many years later, I can tell them I have the gene AND the symptoms, and that ends the conversation – but that wasn’t possible till the science caught up.
Exactly. They don’t even consider it because they’ve already made up their minds, never mind that rare doesn’t mean impossible. Then they say they’ve never seen a case when the truth is they’ve never *diagnosed* a case, so the stats never get updated.
The number of people who have these illnesses is staggering. I don’t know if I believe things will change with how certain patient groups are treated, but I do hope the medical code changes soon.
What a great article. It’s right on every level. I suffered for years with high pressure, eventually diagnosed by a neurologist called Dr Ellis, when all others just said I had a migraine. Last year I had shunt surgery which basically eradicated my symptoms. Unfortunately in October the shunt blocked and revision surgery didn’t go well. It caused a CSF leak which meant I went into low pressure. Now I’m struggling to get help to fix it as I go from high to low continually.