Kaufman and Ruhoy

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.

cerebrospinal fluid

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.

Under Pressure: Large Spinal Study Finds Intracranial Hypertension Common in ME/CFS

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.

Pressure Mounting: Is Fibromyalgia Caused By High Pressure in the Brain (Intracranial Hypertension)


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.

Eye anatomy

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.


  • 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 a supposedly key sign – that IIH makes you feel worse when you lie down. It doesn’t hold true in the complex disease group. Some people feel worse when they stand up. Doctors, they asserted, have to take a really good history – it all comes down to that.

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.


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.

lumbar puncture

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.

Diamox (Acetazolamide) For ME/CFS, POTS and Long COVID


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

Dr. Ruhoy

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

Check out Melissa Wright’s blog on IIH.

High Intracranial Fluid Pressure, ME/CFS, Fibromyalgia and Long COVID


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