Ruhoy and Kaufman clearly enjoy the challenge of dealing with these complex diseases.
This blog follows the discussion between two ME/CFS experts, Dr. Ilene Ruhoy and Dr. David Kaufman, on their March 8th, 2023 edition of their Patreon channel: Unraveled.
Two chronic fatigue syndrome (ME/CFS) experts; Dr. Kaufman – an internist – and Dr. Ruhoy – a neurologist – began a more or less weekly Patreon channel called Unraveled: Understanding Complex Illness in December of last year. The Patreon broadcast provides the rare chance to see two experts who are deeply immersed in these diseases publicly dig into their many different aspects.
In an email, Dr. Kaufman spoke about their goal in doing the Patreon series – which is occurring at least once a week.
“Our goal, our reason for doing this, is to get the word out there and not just to patients but to their families and especially to other physicians. I am quite passionate about the desperate need to recruit more physicians to this work both for ME/CFS and the exploding epidemic of Long Covid. In addition to pulling/luring physicians into this work–which is incredibly challenging and rewarding–I want to help educate primary care physicians since they are–or should be–the backbone and foundation of healthcare in general and particularly for this patient population.”
The blog also contains findings not discussed in the talk. First, a bit on idiopathic Intracranial hypertension and complex diseases like ME/CFS/FM.
Idiopathic Intracranial Hypertension
Even though doctors have been doing lumbar punctures for decades in ME/CFS, idiopathic intracranial hypertension (IIH – increased spinal fluid pressure) didn’t seem to be on anyone’s radar until 2013 when Nicholas Higgins and two other Cambridge researchers reported that 8/20 ME/CFS patients exhibited high intracranial pressures during a lumbar puncture.
Another 2013 report found that while most people with ME/CFS did not met the criteria for IIH, but many reported improvements in symptoms after their cerebral spinal fluid was drained during a lumbar puncture.
High cerebral spinal fluid levels could potentially impact just about every symptom in ME/CFS/FM.
Higgins’s 2015 case report brought the IIH issue closer to home when he described a 49-year-old woman “with a long and debilitating history of chronic fatigue syndrome” who was diagnosed with “borderline idiopathic intracranial hypertension”. Her cerebral spinal fluid pressure was not particularly high, nor she did not have exhibit papilloedema (increased pressure in the eye) – two conditions many doctors require for an IIH diagnosis – but her symptoms improved dramatically for 4 days after a lumbar puncture had reduced her spinal fluid pressure.
After a catheter venography was done and stents were put in her pressure headaches and fatigue disappeared and her aches and pain were improved. Higgins reported that she experienced a “life-changing remission of symptoms with no regression in 2 years of follow-up.”
In 2018, Mieke Hulens, a Belgian researcher, and her colleagues proposed that all the symptoms found in ME/CFS and fibromyalgia (FM) could be explained by high cerebral spinal fluid pressures and proposed that doctors routinely monitor spinal fluid pressures during lumbar punctures.
IIH, though, really only became a big deal after a large Swedish study that IIH is almost ubiquitous (83%) in chronic fatigue syndrome (ME/CFS). We clearly need more studies, but if that finding is correct, IIH or IH is one of the most common manifestations of ME/CFS – and is certainly one of the most underdiagnosed.
Melissa Wright on Health Rising has produced several blogs on idiopathic intracranial hypertension and related issues.
The Patreon Talk
“We have to have a very high index of suspicion when seeing any of these patients (with regard to IIH) even if they don’t exhibit the classic symptoms.” Dr. Kaufman
“It’s an important diagnosis to rule in or rule out in these patients.” Dr. Ilene Ruhoy
It’s remarkable to see a new diagnosis – let alone one that could explain so much – suddenly pop up in chronic fatigue syndrome (ME/CFS) after so many decades. Maybe it’s not surprising, after all, though. Spine/brainstem findings (craniocervical instability, Tarlov cysts, tethered cord, cerebral spinal leaks, and idiopathic intracranial hypertension) have, after all, showed up all over the place in ME/CFS over the past ten years or so. There’s no denying that spinal conditions are playing a role – and sometimes a big role – in some people with ME/CFS.
Given that, it was not surprising to see Dr. Ruhoy – a neurologist – and Dr. Kaufman tackle it early in their Patreon series on complex chronic diseases.
Idiopathic intracranial hypertension (IIH) refers to increased spinal fluid pressure. Because it can affect your brain, and your cranial and spinal nerves, it could mess with a lot of fragile and pretty important real estate. Despite its potential for damage, IIH was first called “benign intracranial hypertension” (i.e. not harmful intracranial hypertension) and most doctors are probably not aware of its implications.
Dr. Ruhoy illuminated the kind of inadequate training she received in medical school regarding IIH. IIH got off to a rough start in the medical profession when it early on was associated with female gender and obesity – and weight loss was recommended – and the disease was largely considered “benign”.
Further research into leptin receptors suggested, though, that the obesity might have been related to the IIH instead of the other way around; i.e. IIH was helping to cause obesity in those who had it. Whatever the role obesity has in IIH, Ruhoy seemed to believe that the medical profession took a wrong turn when it focused on it. Neither she nor Kaufman have found obesity a factor in the IIH they see in their complex chronic disease cases.
“Arbitrary” Diagnostic Criteria?
Over time, we see the medical profession go down dry holes and get into restrictive and artificial thinking as it tries to nail down what’s going on in our very complex systems. Take papilledema – which refers to increased pressure on the optic nerve. Papilledema has been considered a key symptom of IIH, but Dr. Kaufman has found it not always present in his patients.
(Papillodema can be diagnosed in various ways including the opthalmoscope assessment that’s typically done during a visual exam. Some signs of papilledema include headaches that are worse in the morning, brief episodes of blurred, gray or black vision that last from seconds to minutes, double vision or seeing flashes of light, nausea/vomiting, pulse-like ringing in the ears (tinnitis), problems with thinking or movement. Given the possibly quite high incidence of IIH in these diseases, papillodema seems like good reason to get regular visual exams.)
Kaufman referred to the tendency to kind of black/white and non-rigorous thinking that says a condition is present if x finding is met but not if it’s not – when the condition really exists on a spectrum. Usually the markers for a condition are set too high – missing a substantial number of people who have it.
That applies to the intracranial opening pressure required to make a diagnosis of IIH. Both Kaufman and Ruhoy believe it is set too high for the ME/CFS population.
Both Higgins and Hulens agree that the normative values, and thus the diagnostic criteria used for IIH, don’t apply to ME/CFS or FM – or any other condition, for that matter. Even Higgins’s patients who exhibit high CSF pressure do not usually exhibit papilledema – which is enough to get them scratched off the IIH list for most doctors. Hulens has even gone so far as to call the diagnostic criteria “arbitrary” and Higgins believes a blind adherence to them has led to a “grossly implausible distortion of the epidemiology of IIH.”
Kaufman introduced a term that I think fits so well in these diseases – he said people exist with such a “high level of symptomatic noise” that they miss some of the symptoms that they have; i.e. their vision improves, but their vision problems had gotten buried in all the other symptoms they have.
Headache, visual changes, nausea, dizziness, back and neck pain, convulsions, visual acuity problems, visual field, double vision could all be caused by IIH.
In 2018 Hulens proposed that high spinal fluid pressure in the sleeves of the spinal nerve roots of FM patients was compressing those nerves causing a “polyradiculopathy” or pinched spinal nerves. The peripheral neuropathy symptoms found in FM (numbness, tingling, electric shocks, burning sensations, allodynia, weakness) as well as the reduced muscle strength studies could all be caused by increased spinal fluid pressure. These pinched sensory nerves are in turn causing pain and other symptoms across the body. The difference between these pinched nerves and the pinched nerves we ordinarily think of is that spinal fluid pressure, not structural problems, is the cause.
I
Empty Sella Syndrome
That brought Dr. Ruhoy to another and even less well-diagnosed syndrome in ME/CFS and related diseases called Empty Sella Syndrome (ESS). We have seen exactly zero studies on ESS in ME/CFS or FM (but one recent paper), yet Dr. Ruhoy said she sees ESS or partially empty Sella in 80-90% of her craniocervical instability patients. (Increased CSF fluid pressure causes flattening of the pituitary – possibly causing problems with pituitary hormone production (ACTH, TSH, H, FSH, PRL, oxytocin). Dr. Ruhoy has also found elevated prolactin in many of her patients which could be linked to the pituitary problems via compression on the hypothalamus stalk.
Dr. Kaufman extended the pituitary findings and the possible IIH relevance to them by stating that virtually everyone with ME/CFS (@90%) has a disorder of the hypothalamic-pituitary axis – which could be the result of the IIH pounding away at the pituitary. Thyroid problems, adrenal insufficiency, and pituitary problems – they all could be impacted by the IIH.
Doctors could get clued into these issues by asking some simple questions: “Do you have pain in your eyes?”, “Does it hurt when you bend over or sneeze?”, “Do you have tinnitus?”, “Do you have a headache that is worse in certain positions? That pulsing tinnitus, by the way, occurs because the head is so filled with fluid that you can hear the heartbeat through the fluid.
The possible complications continued to climb: migraine, cerebral spinal fluid leak (standing/sitting problems), Tarlov cysts, small fiber neuropathy (peripheral pain), dorsal root ganglia problems (peripheral pain/sensory nerves/ central sensitization), interstitial cystitis, irritable bladder, gut problems – all could be impacted by high spinal fluid pressure.
In short, the very complicated disease that ME/CFS and these other diseases are could all reflect problems with high cerebral spinal fluid pressure. They even suggested that, in some cases, the elevated cerebral spinal fluid pressure could be causing mast cell activation syndrome.
MRIs that measure the diameter of the optic nerve are particularly helpful in diagnosing IIH.
Kaufman and Ruhoy looked at issues like CCI and high intracranial pressure as welcome ways for doctors to understand why complex diseases like ME/CFS have so many symptoms – instead of just concluding their patients are crazy.
These problems are not easy to pick up. Doctors who have the knowledge and flexibility to order the right tests and track down what’s happening have the best chance of identifying what’s going on.
THE GIST
- This is the next in a series of blogs following the discussion between two ME/CFS/FM/POTS experts, Dr. Ilene Ruhoy and Dr. David Kaufman, on their Patreon channel “Unraveled”.
- This blog provides an overview of their March 8th, 2023 talk on idiopathic intrancranial hypertension (IIH) or high cerebral spinal fluid pressure levels. A large 2020 Swedish study suggested that IIH may be ubiquitous in the ME/CFS population. Dr. Ruhoy’s neurological background was particularly helpful in their discussion.
- The medical profession typically views IIH as a disorder afflicting obese women, and its cornerstone treatment is simply weight loss. ME/CFS researchers and doctors, including Kaufman and Ruhoy, believe the diagnostic criteria are set too high – resulting in many people with IIH in the ME/CFS/FM community being undiagnosed. They do not find IIH restricted to obese women in this community.
- Because increased cerebral spinal fluid pressure could affect your brain, and cranial and spinal nerves, it could potentially produce virtually every symptom found in ME/CFS including cognition, many different types of pain including head, neck, bladder, and gut pain, tinnitus, migraine, sympathetic nervous system functioning, HPA axis problems, empty sella syndrome – the list goes on and on.
- While the cause of idiopathic intracranial hypertension (IIH) is unknown, another condition called transverse venous stenosis, which refers to a narrowing of the veins in the brain, can help produce it.
- Various tests such as CT myelograms can help with diagnosis, and MRIs that assess the diameter of the optic nerve sheath are particularly helpful. Lumbar punctures can help assess pressure levels, but what’s considered high pressure is, Dr. Ruhoy said, “not necessarily applicable, or reliable or even accurate”.
- Lumbar punctures that remove 20-30 ccs of fluid and which result in an improvement in symptoms are a clear indicator that IIH is present.
- Both Drs. Kaufman and Ruhoy first use Diamox (acetazolamide), which Dr. Ruhoy stated is fairly well tolerated and safe. Dr. Ruhoy uses topiramate if acetazolamide doesn’t work out and may add a little furosomide (Lasix) (to topiramate but not acetazolamide) to make topiramate a little more potent.
- It was a joy watching these two ME/CFS experts – who clearly enjoy each other and love the challenge of dealing with these complex diseases – toss their ideas back and forth.
Take these histories… Some of Dr. Ruhoy’s patients with a long history of high intracranial pressure sprang a cerebral spinal fluid leak. A CSF leak is not a good thing, but it did relieve their high intracranial fluid pressure. Their intracranial pressure plummeted and then rose after their leak was fixed. As it approached normality, their symptoms improved dramatically for a time – only to worsen as their IIH returned. Their cerebral spinal fluid pressure, then, went from too high, to too low, to normal, to too high.
Transverse Venous Stenosis
“Transverse venous stenosis” is a condition related to IIH that Dr. Ruhoy has found in some of her patients. Transverse venous stenosis describes a narrowing of the veins that flow out of the brain, causing fluid to back up in the brain (contributing potentially to IIH). (Stents – which Dr. Ruhoy described as “not great”, but which can help, are used to open the veins and increase the venous blood flows).
Dr. Kaufman – who described himself as something of a newbie in this area – said that once again, the more they look for venous compression syndromes, the more they find them.
Diagnostics
Assessing the diameter of the optic nerve sheath is an excellent way to check for IIH. CT myelograms can help as well. The bottom line is that the tools are out there – they just need to be used and unfortunately, Dr. Kaufman stated, they are not easy for a doctor to get.
Reduced symptoms after a lumbar puncture, which removes 2-30 cc’s of cerebral spinal fluid, is diagnostic for IIH.
Lumbar punctures can be helpful, but what’s considered a high pressure is, Dr. Ruhoy said, “not necessarily applicable, or reliable or even accurate”. Plus, in people with connective tissue disorders such as Ehlers-Danlos Syndrome (EDS), lumbar punctures can create a chronic CSF leak. (Dr. Ruhoy, though, finds that doing them slowly and carefully in the clinic helps to avoid the leaks and has not had problems with leaks.)
A positive response to a lumbar puncture that takes 20-30 cc’s out – thus reducing the spinal fluid pressure – clinches the diagnosis for Dr. Ruhoy. Nothing more is needed. I had a spinal tap which left me feeling better for hours. That was promising for me, but I know of someone whose POTS completely disappeared and whose mental clarity zoomed for almost 3 weeks after a spinal tap. Her symptoms resumed after that.
Diamox (Acetazolamide)
Both Drs. Kaufman and Ruhoy first use Diamox (acetazolamide), which Dr. Ruhoy stated is fairly well tolerated and safe. Acetazolamide (Diamox) is also used to treat channelopathies which can contribute to IIH. She uses topiramate if acetazolamide doesn’t work out and may add a little furosomide (to topiramate but not acetazolamide) to make topiramate a little more potent.
Acetazolamide inhibits the enzyme (carbonic anhydrase) that makes cerebral spinal fluid. Interestingly, Diamox can affect so many symptoms that Ruhoy and Kaufman noted that patients can respond to Diamox because of something other than IIH. When it works, though, it can make a “huge difference” and be “life-changing” for these patients.
Dr. Kaufman reported that one of the biggest problems he has with Diamox is that some of his patients get very fatigued. Noting the dive in pressure that occurs when high CSF pressure triggers a cerebral spinal fluid leak, he wondered if he was simply catching some patients at the wrong time – when their cerebral spinal fluid pressure was low.
In this scenario, the high CSF pressure causes a leak to occur – which relieves the pressure and drives it too low. Under those circumstances, the drug would further lower CSF pressure, causing fatigue.
The drug is not expensive, has been used for decades and its safety profile is well known. This is the kind of finding one would hope would prompt the NIH to finally allow clinical trials for ME/CFS to be funded through its panel on ME/CFS.
Both Kaufman and Ruhoy believe IIH is probably showing up in long COVID. Kaufman, though, wonders if it takes years and years of inflammation to produce conditions like craniocervical instability and IIH.
Kaufman and Ruhoy agreed that having an MRI done to measure optic sheaths was incredibly useful. It’s this kind of recommendation that underscores how important it is to have doctors with experience in this area. Let’s hope some of them are watching these podcasts.
Ruhoy and Kaufman noted that the dramatic improvements some patients experience during a traction trial (to lift their heads off their brainstems) may not always be due to relieving craniocervical instability. High cerebral spinal fluid pressure can, as it can do with cranial or spinal nerves, tweak the brainstem as well, causing it to respond with a sympathetic nervous system (fight or flight) surge state. In some patients who respond well to traction, it may reduce cerebral spinal fluid pressures by restoring normal cerebral spinal fluid flows.
Jeff reported on the Phoenix Rising Forums that his neurosurgeon will often recommend a Diamox trial before surgery to see if surgery can be prevented.
The Medical Detective
A self-described medical detective Dr. Ruhoy loves the challenge of figuring out what’s going on with her complex patients.
Dr. Ruhoy ended by saying that she regards each patient as a “project” – a puzzle that she as a kind of medical detective wants to solve. That’s what she enjoys in medicine.
Boy is she in the right place! This disease practically demands practitioners who remain curious and enjoy taking on complex challenges. Kaufman noted how the “Septad” formulation – presented in Health Rising’s first blog on their Patreon channel – allows doctors to make sense of these thought-provoking illnesses.
It was a joy watching these two ME/CFS experts – who clearly enjoy each other and love the challenge of dealing with these complex diseases – toss their ideas back and forth. Check out more blogs in Health Rising’s series
- The “Septad” – a Treatment Roadmap for ME/CFS, POTS, Long COVID : From “Unraveled” a Patreon Broadcast from Dr. Kaufman and Ruhoy
- Patreon #4: Kaufman and Ruhoy on Craniocervical Instability in ME/CFS and Allied Disorders
Check out Melissa Wright’s blog on IIH.
I have long thought I have IIH. I do wonder about the possibility of Intermittent IIH (IIIH??) And therefore, a lumbar puncture could be normal at that time due to a) physical position during the puncture (some get symptomatic with position changes) or b) not being symptomatic (for any reason) at the time of the puncture. And, it is my understanding that any lumbar puncture can contribute to arachnoiditis, another cause of wonky CSF flow and odd neuromuscular symptoms.
Very very interesting Cort. I suspect this to be a big part of the picture for many of us
What a complex condition .
Perhaps people with m.e. the threshold for passing into intercranial hypertension readings due to the weajebing , eds type tissue
Another comment. Could this explain not only sensory neuropathic discomfort; but cause muscular “jerks,” spasms, isolated myoclonus? Motor symptoms? I get those lightning shooting electrical pains accompanied by totally involuntary movement of my leg. And I don’t have MS.
I really don’t know. Hulen proposed that it could be causing numbness, tingling, electric shocks, burning sensations, allodynia, weakness….muscular jerks? I don’t know. I remember Dan Moricoli suffered from those – he eventually got over all of it.
Hi
Sudden muscular jerks are very common in ME/CFS. In pediatric experience over many years about 80% have them. Most often one sided (unilateral) han/ arm and/ our foot. Just one (not series). Most often a couple/ week. Much less common (about 1/5 of the 80%) are two-sided, synchronous (at the same time), single, jerks in both arms/ shoulders. These are both myoclonic jerks (not tics which are slower). They are definite “brain symptoms”. Not causing trouble (I have seen some with 100+/day in the beginning of ME/CFS a couple of times). They are actually old news..
Yes, absolutely! It has to be noted that CSF pressure readings are not always an accurate measure of dysfunction. A ton of factors can change the readings, including the position the patient is in for the test. I personally think because ICP was dismissed by doctors for so long that they assume pressure is not sensitive. But blood pressures and heart rates can vary in people so it’s not great assumption to make.
But also over time, the body learns to compensate for pressure issues and some of us swing wildly during different activities over the course of a day. If you suspect you have this at all, I recommend looking into it with someone up on the current info—maybe on the Spinal CSF Leak Foundation physician directory, because leak specialists generally also understand high pressure.
Thank you! Dr Bateman has referred me to one. It is now my task to summarize my journey, including all the imagings over the last 9 years (including my upright MRI with flexion and extension views.) This will be submitted for review. I hope I pass the audition! Thank you fot validating our chosen pathway!
Oh this is fantastic news! I hope this is your answer! When I was leaking I cycled between high and low pressure as my body tried to compensate, but now with only high pressure, it still fluctuates quite a bit. I’m personally affected greatly by sodium intake but many factors can change it.
I recommend a printed symptom list that includes any positional aspects, like if your heartbeat whooshes in your ears bent over or if your nose runs when you lean forward. Think about whether you need to be totally flat when lying down or if you’re better with your head on a pillow and whether your symptoms are worse in the morning or night. Those details may help. But the great thing about a pressure specialist is that they know the right questions to ask. I wish you luck!
I don’t understand the reference to the Bonilla study – did he treat a subset of patients with Diamox as well as Abilify?
I am confused by that too, was that a mistake, Cort?
Yes, it was a big brain fart! Don’t know how I got that mixed up – must have been the long A…….. word. I don’t know it showed up in my search. Darn! It looked promising! Thanks for catching that so quickly…
Cort, you are amazing, I don’t know how you do all this with ME!!
With your help I will hopefully get things more right in the future. 🙂
I have a pretty rare condition called Superior Canal Dehiscence, a hole in the superior canal of the ear. Suffered for 5 years from 2005-2010 until I found a doctor in Louisiana who operated . He always does lumbar punctures before operating and my pressure was fine. This is another condition that can cause pulsatile tinnitus (hearing your heartbeat but also other things like your footsteps….) and, dizziness. 2 hours in OR and 5 years of those awful symptoms were gone. Recently went back and although do have a good sized hole in other ear the tests were mostly ok so is trying me on diamox. Interesting to read that it has caused some improvement in CFS…….
In the Diamox page attached to this blog one person says Diamox is another doctors go to drug for tinnitus. Good luck.
I can hear the movement of eye muscles when shifting my eyes. Though it’s now less clear due to an increased volume of constant hissing. I hear (very tense) muscle tone generally as a kind of low pitched background drone. And I used to hear a very high pitched electronic scream that I took to be nervous system noise, but that’s gone with age-related hearing loss (82 in a few weeks’ time). I’ve had FM/CFS for 22 years: together with cervical & lumbar-sacral osteoarthritis diagnosed in 1976 with shocking pain at the back of my neck which has recently returned with a vengeance, accompanied by instant exhaustion & urgent need to lie diwn if I stand for more than a few minutes & forget to stretch my neck away from my shoulders & rotate my head, Alexander Technique style.
This is not the cause for ME/CFS, totally bogus. What is the connection with starting after an infection? Or long stress. It doesn’t even explain the symptoms of ME/CFS.
Patiënts who have this ‘condition’ don’t have ME/CFS.
Many of us have post-vial ME/CFS and IH, as well as other structural and venous issues.
Well….Dr. Kaufman and Ruhoy beg to differ I guess – and so do the Swedish researchers. Interestingly, though, except for the inflammation idea – which is enough I guess – no one is saying anything about cause….It’s very “idiopathic. Inflammation, could, I suppose be blocking up the veins and the lymphatic drainage causing the CSF to build up.
Ray Perrin has talked a lot about that.
I’ve had CFS/ME /autoimmune relapses for 35 years, mostly managed well with diet and homeopathy and supplements and meditating on self-compassion. In the last 5 years however there’s been a buildup of fluid at the base of my skull, and for a few years using a roller on my spine and neck helped, but no more.
Recently I felt like I’d become a ‘zombie’ and have been feeling increasing confusion, balance and bladder problems and strong tinnitus with cranial pressure – so I looked up the HR blog about Perrin technique and followed some YouTube videos, and after one try I had remarkable results. I was hoping for a quick fix.
Not having discipline though, I haven’t done it 3 times per day but now I have no choice because the symptoms are starting to look like early NPH – normal-pressure hydrocephaly, and I’m in Sri Lanka for the winter.
My sister-in-law had it and after getting a shunt in her brain, within 10 days she went from a wheelchair to walking again.
If I don’t clear the brain’s lymph myself, I sense this is just getting worse. I used Chat GPT to compare the symptoms of IIH and NPH and I’m more in the NPH category. Maybe this will help someone else recognize what’s going on and get tested. ME/CFS is multi-layered and if we can recognize and peel off one layer, it’s worth it.
Perrin technique – https://theperrintechnique.com/about-us/
Perrin lymphatic self-massage video 12 min. https://www.youtube.com/watch?v=ccV24hCOe5A
Short version – Perrin lymphatic self-massage 5 min.
https://www.youtube.com/watch?v=Vd7xz9Pzjw4
I do the perrin technique. I, at one time, could feel lumps aprox 2″ under my skin.they are very, very small now since doing perrin.
because I had an ulcer way back in 81′ I believe I have a deep rooted infection, maybe from having a tooth pulled,as the socket took forever to heal,this at the age of 15. I take a native American herb that seems to be making headway.
I believe cranial pressure to be caused by infection weakening the body ,causing people’s disc’s to shrink or disintegrate, causing the spine and its supporting muscles to no longer prop the entire body up.
think about it…if the spine is damaged or weakened how could the fluid pass through
You could technically call it not a “real ME/CFS” since ME/CFS definition requires that no other condition explains the symptoms. But some people with IH have been reporting ME/CFS symptoms complete with PEM and brain fog. So, maybe it’s possible that IH, through neuroinflammation or something else, causes the MECFS symptoms.
That said, lumbar puncture or the drug would’ve been a part of the standard therapy by now if they worked well enough for enough number of patients. That they aren’t seems to tell me that only a small number of patients have MECFS symptoms caused by IH and therefore benefit from the therapy.
Many of the assumptions about intracranial pressure were in fact incorrect, so many patients were 1) never truly ruled out as leakers because their doctor considered leaks too rare to bother 2) never tested for high pressure because they did not present with papilledema—which has now been discovered is not a requirement, as well as obesity (many leakers are thin) or 3) noted as having normal opening pressure when normal appears to vary for everyone 4) ruled out from a normal MRI a which is now also considered not accurate in many cases and 5) not seen by a pressure specialist with an understanding of the proper imaging techniques and how to read the imaging for this specific condition.
If an ME/CFS diagnosis is a diagnosis of exclusion then IIH has to be *properly* excluded. And that is not happening.
Maybe slew of researchers over the past 40 years overlooked IIH? Maybe CDC and MECFS organizations should update their guideline according to what you say? Well, here is to hoping that IIH be properly excluded and many more patients released from the ME/CFS hell.
It does seems likely. Because the diagnostic criteria was incorrect and because they had no way to know CSF pressure could affect the brain this way. And now that they can see inside the brain, they’re quickly discovering how those systems work and finding new information that will help treat pressure as well as countless neurological issues.
I’m incredibly hopeful.
Lumbar puncture isn’t a therapy. They are danger and painful. LP’s are used for diagnostics only.
Many illnesses raise intracranial pressure, including the ones common to ME/CFS. In addition, many of the medicines used to treat those conditions can raise pressure. Intracranial pressure can be affected by blood flow, CSF flow, or inflammation. Anything that adds or removes pressure inside the skull is pressing on the brain and nerves—our control center for the body and its functions.
Hi Melissa, I have a 7 mm nerve root cyst at T1-T2 does this mean anything?
Hi Nancy. Yes! Information and resources here: https://www.tarlovcystfoundation.org/faqs/
And here: https://spinalcsfleak.org/resources/resource-documents/
And tons of videos from a recent symposium at the Spinal CSF Leak Foundation YouTube channel. So much of this is really (relatively) new data and they’re constantly making advances and publishing new studies so make sure you look for current dates on your research and find a doctor who’s up on the new imaging and tests. I hope this is your answer!
Gijs,
I totally agree with you, Because the cause of ME/CFS has not been identified, all kinds of conditions are being tossed into this diagnosis.
A 17 year study in Taiwan found ME/CFS following infections in a very large patient group compared to a control group.
Are we ignoring these important data because the study wasn’t done in this country or Europe?
https://translational-medicine.biomedcentral.com/articles/10.1186/s12967-023-04636-z#:~:text=Conclusion,increased%20risk%20of%20developing%20CFS.
The increased brain pressure can cause the following symptoms:
Headache. This is strongest in the morning and increases with sudden head movements, such as coughing.
Tinnitus.
Dizziness.
Nausea.
Double vision (diplopia) and blurred vision.
Temporary blindness. This can occur if the patient changes the position of his head.
No typical ME/CFS presentation. It could be secondary, but certainly not primary. In rare cases just like you can have ME/CFS and also diabetes.
That’s not an inaccurate list, but leaves out many of the less commonly recognized symptoms, which the medical community has come to acknowledge after recent advancements in testing and equipment. A more accurate list is below, but it should be noted that pressure on the brain and brainstem can cause any systems that those control to have issues:
Symptoms of intracranial hypertension vary. Not every person will have headache, or vision issues, or any of the less common side effects of high pressure. Assumptions otherwise is the reason many cases are misdiagnosed.
Key Symptoms are:
Headache, head pressure, or head pain, as with low intracranial pressure, is considered a key symptom of IH. Intracranial pressure headaches may not be relieved pain medication. IH headache may not occur in one specific area. Pain may be located behind eyes, forehead, one side of head, back of head, base of skull, top of head, or may be burning or pressure type pain, and may be made worse by eye movement. Not everyone with IH has all head pain symptoms.
IH headache may share migraine characteristics, such as light and scent sensitivity, and worsening with exertion, cough, or straining. Migraine, however, should not be accompanied by pulse synchronous tinnitus or visual issues (excluding aura–temporary flashing or flickering objects, usually lasting about 20 minutes prior to migraine onset, and disappearing after head pain begins).
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 caused by CSF pressure on the optic nerve and blood supply can lead to vision loss. Not everyone with IH has all vision symptom and lack of papilledema does not rule out IH.
Pulse-synchronous tinnitus (whooshing, whistling, humming or marching noises in one or both ears that correlates with your heartbeat).
Other Symptoms are:
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 nose (often misdiagnosed as allergy or sinus issues)
Endocrine issues (due to empty sella syndrome and the flattening of the pituitary gland)
Malaise
Exercise intolerance
These symptoms may appear in a variety of patient groups, yet many do not realize they may be attributed to raised ICP. It’s important to remember diagnoses considered rare can be disregarded because they are considered rare.
(On a more personal note, I experienced all of the ME/CFS symptoms including fever and illness issues with my low pressure spinal CSF leak.)
There are many diseases with a similar list of symptoms. I.E. Lyme disease, thyroid diseases, adrenal gland diseases etc… These are often general complaints. The key symptoms often make the distinction. In this case it doesn’t resemble the presentation of ME/CFS. That’s why a specialist is needed to properly address a patient’s complaints. An objective test can then provide supporting evidence or a strong indication. Based on the current research, you can’t say that IIH is the cause or plays a role in ME/CFS, nor even what percentage of ME/CFS patients have IIH. More research is needed for that. But I don’t rule out that some people have this condition in a ”benign form”. Because the primary form is a serious condition that normally requires surgery or medical intervention.
Hi Melissa – thank you for posting this! Do you happen to have a link? I’d love to read more.
Hi Ann. I’ve been collecting research I’m interested in here: https://pressureresources.com/research
But there is much more out there, including how venous compression and other issues affect intracranial pressure. It’s often dismissed because the older data isn’t accurate, but ICP issues can be severe and disabling and cause issues in many other body systems that I’ve experienced myself. Fortunately new data is out there every day. I hope it’s helpful!
Mellissa this is fantastic, thank you! I also have multiple venous compressions affecting my ICP so this really resonates with me. I appreciate you replying and I can’t wait to dig in!
Sorry I spelled your name wrong, Melissa!
It isn’t known whether the cause of ME/CFS is intracranial pressure because the cause of ME/CFS is unknown. But intracranial pressure is certainly the cause of many symptoms experienced by the patient community. And that’s incredibly important for quality of life and for finding a cure.
I agree, we are nowhere near finding out the cause of MECFS as there is very little research being carried out. It maddens and upsets me when I see some of the posts which state there is a cure..I have had this illness for 30 years and my GP has started to take an interest and is sending me for various tests at the hospital. Wish me luck!
I lost 40 lbs. a few years ago. It made NO difference in ME/CFS symptoms that I remember.
The weight has crept back, as it does, even though eating and food are no longer of any interest, especially as I now don’t use salt because of edema problems if I do.
Maybe I should try again.
This rings very true for me.
I’ve had ME/CFS for 30 years after an EBV infection but have also been diagnosed with CCI (11 motorcycle crashes) & a partially empty sella.
I had a hormone panel done late last year & my Growth Hormone came back as 0.2. My GP said that was ‘normal’ but the range is 0.2 to 15.0. Hardly normal. Sub optimal doesn’t even describe that properly. It’s 20 cents out of a possible 15 dollars.
I did a 7 day ketamine infusion in hospital & came out feeling superhuman. No symptoms, boundless energy & no brain fog. I crashed after 3 days but pulled out of it the same day, much is almost unheard of. I was good for about 2 months then went back to my illness baseline & have been sick, sometimes worse since then.
Ketamine has been shown to reduce intracranial pressure (PMID: 19569909) in TBIs. So this all adds up.
Also, I know of 2 people with severe ME/CFS who were completely healed after having the top vertebrae fused because they had CCI.
Now to find a GP that will actually believe this..
Why don’t we just try Diamox?
It is cheap,relatively safe…. I am going to,give it a try.
Cort did the dr mention the dose of Diamox?
Thanks a lot.
My number one complaint besides severe fatigue was and is the reeling in my head like pressure. My iq has dropped dramatically from the start over 40 years ago… I would try to explain like my head was a heavy bowling ball being held up by a toothpick.
Anyway shortness of breath (oxygen hunger) and the pressure in my brain along with low oxygen feeling to brain has been ongoing since illness began.
I have had several brain MRI’s over the decades and initially I was,told I had white spots all over my Brian similar to MS but not MS. My last MRI I had a brain bleed? My small vessels in brain are damaged and my brain has shrunk a lot…. ;(. I have trouble standing… the longer I do the weaker I get. I am mostly bed ridden…. My HPA has been off since age 10 that I know,of. My feet burnt every night since age 14. They finally stoppped when I began a new form of B1. I iced them every night for almost 4 decades,,.. not being able,to sleep because of the burning…. I cant believe at least that symptom is gone : unless I stop taking the activated b1. So this sounds easy to try ….:). Just need the dose, my doctor will prescribe :). Th ask again.
I would like to know how many patients have bulging cervical intervertebral discs with CF/ME symptoms. I am doing a research project on the reliability and validity of reporting on MRI and CT scans of the spine. A good percentage of radiologists seem to ignore bulging discs that are not impacting the cord.
I suggest that the reports do not define the fact that scans are more often than not taken in the recumbent position and are therefore scientifically invalid.
Further upper cervical spine instability is difficult to detect. AS the ligaments that attach the cranium to the spine are very fragile there is a further problem that when a person sleeps there maybe chronic cord irritation causing a lot of the symptoms of CF/ME
I would be very interested n the number of CF/ME patients who have suffer disc injuries
The proper specialist for imaging is absolutely critical. So many people are dismissed because a tech not familiar with these conditions didn’t know what to look for. Two people in my immediate family found their high pressure symptoms improved after surgery, just from bone pressing against the dura and interrupting flow. It’s mind blowing to think about how many people this affects and they have no idea.
I have a multitude of spinal issues & a C4-6 fusion due to complete disc breakdown from wear caused by severe hypermobility. Also was told after my tethered cord release that I had a huge amount of stenosis. I have suspected IIH for a loooong time due to the constant feeling of head pressure & whooshing. I also have a LOT of abnormal vision issues that I know are not common bc I was.an eye care professional. Have been unable to find a doc interested I helping though. Easier to just say it’s all EDS related.
Lynn, B1 has helped my IIH a bit too — I take TTFD. What form of B1 do you take?
Diamox is hardly innocuous which is why it’s often referred to as DIE-amox. I’m trying other thigs like B1 in an effort to avoid Diamox — it’s a good backup plan though!
Hello, I remember taking it several times for preventing high altitude sickness when climbing high up (Years ago, before ME/CFS). The doctor who prescribed it wasn’t too worried about side effects, and I don’t remember particular bad side effects…
I wouldn’t try pressure medication without doctor supervision. It would not be worth risking blindness, kidney damage, or some other not-repairable issue when the high pressure itself may be entirely repairable. But if you’re able to get a specialist then you’d know exactly what your underlying cause is and how best to treat or manage it.
There are a couple of IIH and Spinal CSF Leak Facebook groups where you can search for people who have had good experiences with doctors in your area. I saw so many doctors who had no idea what intracranial pressure even was. The right care team makes all the difference.
I have had high eye pressures and have been watched for glaucoma. I don’t have any nerve damage. I’ve received laser treatment twice. Then in 2017 once I began Mestinon, my eye pressures have been in the normal, albeit higher end range. Are these the type of eye pressures they are referencing?
They generally look for papilledema in high pressure, which is not quite the same, but the assumptions in this field are changing quickly so it absolutely could turn out to be–and if so possibly related to low pressure/leaks. I found this if it’s helpful: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7002772/
“Multiple studies have found intracranial pressure to be lower in patients with glaucoma than in control groups, which supports a role for low ICP in the pathogenesis of glaucoma”
I have a diagnosis of intracranial hypertension, lupus, Graves, partial empty sella, mild papilledema, degenerative disc disease , probably some CCI, and mild hypermobility. I’m now investigating the possibility of a spinal csf leak. Among the various Facebook groups for these different entities, there are always numerous patients with Ehlers-Danlos/ hypermobility in each and every group. Dr. Ruhoy has been on several panels discussing csf leaks/Ehlers-Danlos and intracranial hypertension and cci. There is much fatigue associated with both the high and low pressures in IH and csf leaks and in hypermobility/EDS, similar to a ME/CFS patient. One patient that may have been mentioned here before, Whitney Dafoe has been diagnosed with severe ME/CFS but also has Ehlers-Danlos. He has his own Facebook page. I feel like the major factor is the hypermobility issue/weak connective tissue. The patients have issues with IH,CCI, cranial and spinal csf leaks, dysautonomia, Eagles Syndrome, cerebral vein stenosis, and jugular veinous congestion as well. I’m looking forward to more research to bring these all together one day.
I ask this as a physicist and not a physician!
The absolute value of pressure within the cranium doesn’t matter. It would have to rise very high indeed before it changed the chemistry of biological processes. What does matter is the balance of pressure between different volumes of fluid, in this case CSF versus Blood.
It seems to be accepted that PWME have reduced blood volumes. What if the problem arises because cranial blood pressure is too low rather than because CSF pressure is too high? Is that possible?
If pressure becomes too low in the skull then the brain isn’t floating—it’s knocking around the skull or settled into the base and getting pressed down by gravity. If pressure becomes too high then it presses the brain downward. Both of these squish the brainstem—the body’s control center that directs automatic processes—and the nerves.
It’s absolutely a mechanical/hydraulic issue. Think about how your sink backs up into the basin if your drain is slightly blocked. This can be how CSF pressure raises in some cases. And CSF leaks can act as a pressure relief valve. Brains are extremely complex. Squishing them is pretty easy.
My question was whether too low a blood pressure could be causing the problem rather than too high a CSF pressure. Rather than too high CSF pressure constricting the brain, too little blood pressure within the brain could result in constriction?
Sorry—I think so, yes! Because of this:
The skull contains a closed system of brain, blood, and cerebrospinal fluid and a delicate balance must be preserved to maintain intracranial pressure
When something goes wrong—with, for instance, the mechanisms that absorb or drain CSF, illness, or injury—pressure can increase inside the skull, and bone is unable to accommodate expansion.
So if you lower one fluid or the other, either the body is going to try to compensate or the brain is going to occupy the space that was left empty (and things won’t be where they should). We also know that CSF pulsations are driven in part by heart rate and breathing. I personally believe this is behind the POTS-like symptoms of tachycardia and air hunger while the body tries to correct CSF flow during a leak. It stands to reason that either fluid (blood or CSF) would be highly regulated to protect the brain for just that reason.
Too low pressure or too low volume either one. There’s just no data on it yet.
(And yes—it can be blood volume, CSF volume, or brain volume like tumors or inflammation. Anything that displaces brain parts or nerves, or interrupts flow.)
The answer is yes because when you have a CSF leak (or lumbar puncture) the CSF pressure drops and causes a whole different set of issues from the high pressure. It’s a different headache but just as bad and other symptoms like your brain is squishing the pituitary hypothalmus, etc. People report severe symptoms on the rebound after a lumbar puncture to drain fluid. That’s why the relief of symptoms is often very short.
In the past few dsys, given my intermittent and extreme feeling that I have low brsin nliod flow, I also wonder if it is high CSF causing intercranial pressure or hypoperfusion. Last year, on spinal imaging, rather large Tarlov’s cysts were identified and summarily dismissed despite lumbar parasthesias. Cervical degeneration also present. ME/CFS symptoms preceded these fundings by forty years.
I have many of the IIH symptoms, if not all, and noticed years ago that consuming any high-sodium foods would increase head pressure, brain fog, head pain, balance issues and POTS. I completely went off all processed foods, meat and dairy, and only eat the blandest of natural vegetables and grains and seeds. After reading about a young woman who started eating like I do, who then got off the kidney transplant list and now has normal kidney function, I can’t help but wonder if IIH and fluid retention and many other symptoms of ME/CFS are also related to kidney function. It seems to be absent from any research I’ve read so far, and the only person I’ve heard from who must severely restrict sodium due to IIH is Melissa Wright, through your website. I have yet to meet someone else who has to so severely restrict sodium in order to reduce ME symptoms and all the other symptoms that go with it. It’s not an easy diet by any means but might be worth people experimenting for a few months to see if there are improvements.
I am desperate for data on the relation to kindey function! My mom had surgery to repair a C4-C5 issue in her neck that was compressing her spinal cord and blocking CSF flow, which caused intracranial hypertension. After the surgery, her kidney function has improved dramatically and I haven’t been able to find much science on this.
I’m wondering if it has to do with the brain directing what the kidneys should dump or if we’ve just overtaxed our bodies, but I have no idea.
I will say that if this works for you then you should definitely look into IIH patient groups because all of us restrict sodium. Low salt and diuretics are the main treatment for those doing non-prescription management of high pressure (also avoiding vitamin A, certain pressure raising meds, and bending or straining activities). We also sleep on an incline (wedge pillow or raised bed).
Thanks so much, Melissa! You are right about multiple systems being affected. It takes a rather holistic approach that American medicine lacks. As for why the kidneys may be involved, there was an article about each neuro thread passing from brain through neck that controls a different organ. I believe there was a researcher trying to map them…but there are thousands. Perhaps your mother’s neck was blocking the nerve that goes to the kidneys? I know in my situation there are so many things going on, including a great deal of unavoidable stress, and of course there’s the gut-brain axis, too. My approach has been to opt for daily PT exercises, a pure diet, and now that I’m walking twice a day instead of just once, that has been very helpful, at least in the cold weather before summer heat brings on sweat and hyponatremia. It’s a delicate balance but without side effects that drugs and lumbar puncture can bring about. Sometimes meds and procedures can reduce the clarity of the original problem(s).
I am THRILLED to see this! Absolutely fantastic. Thank you, Cort, for all that you do!
So many people with pressure issues are dismissed because of outdated information and there are so many things that can cause pressure issues (accident, illness, injury, or compression issues from birth or that come with age) and evidence that they can cause damage to connective tissue that worsens spine and blood vessel conditions, or possibly MCAS. The more I learn about these connections, the more it all makes sense. But because so many body systems can be affected, there are a lot of places where diagnosis can go wrong—we should always be looking for the underlying cause when we have a group of symptoms!
Recognizing pressure issues is going to help a staggering percentage of the population. I’m so grateful and hopeful.
LOVE the learning / info from this ‘forum’ and SO appreciate the ability to ask questions and actually receive answers 🙂
I was diagnosed with ME/CFS in the ’80s and while I’ve managed to survive, there has been no real quality to my existence.
Becoming my own ‘doctor’, learning to muscle test myself and using homeopathy, acupuncture and other natural modalities, e.g., meditation, breath work, ad nauseam, have kept me going.
My eldest granddaughter was diagnosed with IIH two years ago AFTER COVID VACCINES (I don’t do vaccines) and now has the same symptoms as I, so we are interested in this information and how these complex diagnoses are somehow related.
Are you aware of ANY doctors around Seattle / Western Washington who are savvy about any of this? The docs who treat IIH, are not aware of the possible relationship to these other diseases or we have not found any.
Sending many, many thanks, Cort, for your dedication and for just being ‘there’.
Thanks, Katherine! Ilene Ruhoy is based in Seattle 🙂
I had no idea IIene Ruhoy was based in Seattle–thank you again and again for that information and, of course, for your reply!
Another thing that can lower IP temporarily is oral glycerol. Thats because of its dehydrating effect. Food grade can be ordered on Amazon. Might be worth a test.
Results of decreased IIH with low energy diet..
https://www.bmj.com/content/341/bmj.c2701
Sending appreciation!
Very very interesting Cort. I suspect this to be a big part of the picture for many of us
What a complex condition
No kidding!
If you Google IIH, the info is remarkably indicative of just about every symptom. (50+) of ME/CFS. Cause or effect? I presume effect. What concerns me about this article is the fact that so many of the studies are pretty old. How can this NOT be followed up? First I’ve heard of it, but certainly indicative of what I have gone through. Have they not studied it more because it doesn’t really have a cure? If the cause is Dr. Naviaux’s research, then this would surely be effect. If IIH is worsened by obesity, are ME/CFS patients typically overweight? This IIH must surely be at least tagged to some ME/CFS subgroups. I have been trying to see a neurologist for years, but in Canada, there is a shortage of neurologists and they don’t like to see ME/CFS patients because they either don’t have a lot of help for us, or they just still consider ME/CFS a minor illness compared to what they normally see. I’ve waited for years before they cancel my appointments. One even prescribed meds without even seeing me.
Cort. My NeuroQuant MRI (CortechsLabs Triage Brain Atrophy Report) showed my brain size was in the 96th percentile and my fluid filled ventricles were only in the 32nd percentile. My specialist said I have a swollen inflamed brain. This causes high intracranial pressure. This neuroinflammation I believe is the main causes of high pressure in ME/CFS. The high pressure is just a symptom. It’s not idiopathic. Some people have mechanical issues as the cause like Jennifer Brea did.
https://www.cortechs.ai/solution/neuroquant/
I have two questions:
1) can viral infections cause CSF leaks?
2) can a CSF leak spontaneously repair itself?
i am heartened by the research thatn is going on, into ME?CFS. i have had symptoms for 11years and am desperate to get a cure and live a normal life again.One thing i have noticed is that my core temperature is always feeling hot, and my balance is affected and i have fallen.
i declare this info in case it might help.
thankyou and good luck with research
yours, maggie stirling, uk.
Diamox significantly changed my level of functioning. I started taking it in 2020 just before being diagnosed with CCI. At that time I would wake up feeling like I was dying every morning and I was bedridden 20 hours per day. It was horrific and I would cry almost every morning upon waking. Within days of starting Diamox, I woke up feeling like myself. I was still severely debilitated from CCI, but my quality of life improved dramatically without the severe head pressure.
I had my head and neck fused and tethered cord surgically fixed in 2022. I tried going off of Diamox, but I was still severely debilitated by head pressure. With Diamox, a craniocervical fusion and tethered cord surgery, I am now fairly functional. I am driving, cooking, cleaning, etc. I am upright all of my waking hours these days. I am not 100% or working, but I am significantly improved. Without the Diamox I was having trouble functioning, even following the surgeries.
I also meant to add that I think the Perrin self exercises help the head pressure as well. I noticed a correlation between hormone regulation and doing the exercises. I do them about 3-4 times per week.
Thank you, Cort, for highlighting “high cerebral spinal fluid pressure,” a topic I’ve been discussing for years but which has yet to be acknowledged by PCPs or neurologists. I’ve mentioned CSF issues several times in the forum.
After confirmed issues with my thyroid, adrenal insufficiency, and Tarlov cysts, I discovered a CSF leak from my nose and ears, which alleviated some of my symptoms.
Another often overlooked condition that might influence CSF circulation is poor lymphatic drainage, such as lymphatic obstruction or chyle blockage. For more discussion on related conditions, see this thread: https://forums.phoenixrising.me/threads/proteomics-of-fibrin-amyloid-microclots-in-long-covid-post-acute-sequelae-of-covid-19-pasc-shows-many-entrapped-pro-inflam-kruger-et-al-2022.88621/#post-2416058