Studies suggest that a different form of intracranial hypertension may be common in chronic fatigue syndrome (ME/CFS), fibromyalgia and migraine.
After digging around several times, the doctor was able to access my spinal fluid and get a sample and I was able to stop squirming. Then Dr. Baraniuk said, “Your lumbar pressure was high.”
The first part of my first – and, to date only – lumbar puncture was a bit torturous, but the aftermath was nothing but good. Getting rid of some of that spinal fluid seemed to help. In fact, I was having a very unusual experience – I felt relaxed and my mind felt clear. In fact, I couldn’t remember the last time I’d felt this calm. I should do these lumbar punctures more often, I remember thinking.
Apparently, I’m not alone. Dr. Baraniuk said he sees high lumbar pressures “a lot in ME/CFS”.
The Case For IIH in Chronic fatigue syndrome (ME/CFS) and Fibromyalgia (FM)
The idiopathic intracranial hypertension (IIH) story in ME/CFS begins with 2013 with a Cambridge doctor named John Higgins. Higgins found that 20% of 20 people with ME/CFS had high cerebrospinal fluid pressures. He suggested that an obstruction to venous outflows (another blood vessel issue) was inhibiting normal cerebrospinal fluid (CSF) flows and causing high CSF pressure.
In 2014, using a local anesthetic, Higgins performed a jugular venoplasty (insertion of a balloon) in the jugular veins of 14 women with ME/CFS who had evidence of jugular vein narrowing. All patients reported immediate improvement, but all returned to baseline within a couple of weeks.
In 2015, Higgins described a 49-year-old woman with a 20-year case of ME/CFS, triggered by an infection. CT venography revealed narrowing of her transverse venous sinuses – a possible sign of IIH. Her CSF pressure was borderline normal. Draining some of CSF during a lumbar puncture left her feeling well, headache-free, with more energy and less pain for 4 days – after which she returned to her typical ME/CFS state.
After a surgical implantation of stents, she reported that her pressure headaches and fatigue were gone and her aches and pains were improved. She could also concentrate normally again. Interestingly, her CSF pressure only dropped minimally. Higgins suggested intracranial pressure is different and is a more important factor than CSF pressure taken during a lumbar puncture. Two years later, she was still doing fine.
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. He noted that his 20-person study found high lumbar pressures in 20% of his ME/CFS group but 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 was energy improved. The heightened sense of well-being and health lasted for weeks for some.
Then, in 2019, using indirect measures (eyeball diameter, optic nerve diameter), the first large study assessing IIH in ME/CFS occurred. Bragee did not use the gold standard – a lumbar puncture – but he found evidence of IIH in no less than 55-83% of 200-plus ME/CFS patients in Sweden.
(Studies indicate that ultrasounds with optic nerve sheath diameter (ONSD) greater than 5.5 or 5.6 is indicative of moderate to high spinal fluid pressure.)
Now step into a different world – a world in which IIH is rare, is often associated with obesity in young women, and is diagnosed using complex criteria requiring expensive testing.
The current accepted diagnostic criteria for IIH are convoluted and reflect some of the uncertainty in the field. They state that 1-5 below must be met for a full diagnosis. A probably diagnosis is made if 1-4 are met.
- Normal neurological examination except cranial nerve abnormalities.
- Neuroimaging: normal brain parenchyma without hydrocephalus, mass, or structural lesion, and no abnormal meningeal enhancement or venous sinus thrombosis on MRI or MR venography; if MRI is unavailable, contrast-enhanced CT can be used.
- Normal CSF composition.
- Raised lumbar puncture opening pressure (>25 cm H2O).
Alternate criteria #3: If papilloedema is not found but a sixth nerve palsy is present, and other criteria are met, IIH is diagnosed.
Alternate criteria #4: If both papilloedema and sixth nerve palsy are absent, but criteria 2-5 are fulfilled, a diagnosis can be made if flattening of the posterior globe, empty sella, perioptic subarachnoid space distension, and transverse venous stenosis are found (whatever the heck all that means). The authors noted that the last criteria may be too restrictive.
Hulens suggests other testing including: sensory testing using a pin-prick test, strength testing, knee and Achilles tendon reflexes tests, a tandem gait test, an MRI revision to detect possible nerve root dilations and TCs, NCS/EMG, and measurement of the retinal nerve layer thickness using an optical microscope.
The 2016 Markey Lancet review states that the symptoms of IIH are very variable. Hulens asserts that many of the symptoms in ME/CFS and FM could be explained by high cerebral spinal fluid pressures. He calls the condition “chronic postural idiopathic cerebrospinal hypertension” – apparently because when you stand, your pain usually worsens. Standing or sitting up causes the sleeves of the nerve roots across your spine fill up with cerebral spinal fluid, putting pressure on the nerves and causing everything from facial pain to gut pain to leg pain. Even the cognitive issues could come into play. Hulens also believes that leaky blood/brain barrier, in conIunction with high CSF pressures, causes issues in the brain.
- Stiff Neck – Upper body pain is very common in both ME/CFS and FM. Hulens believes this symptom occurs when the CSF fluid stretches the spinal nerve sheaths at the back of the head and neck, causing the spinal nerves to exit the spinal cord and touch the muscle tissue (ouch!).
- Nerve pain – pinched sensory nerves may be producing pain in the arms, shoulders/upper back, hips/lower back, and legs. Children with IIH used anvils, hammers, and a vise grip to describe their head pain as pounding. Some also experienced photophobia.
- Hulens believes increased IH may be causing degeneration of the sensory nerve roots and could be contributing to the small nerve fiber losses in FM and ME/CFS.
- Gastrointestinal problems – the gut problems in ME/CFS/FM could derive from nerve compression in the lower spine.
- Dizziness and balance problems – cause unknown.
- Endocrine problems – not common in IIH but may occur when CSF causes flattening of the pituitary glands and empty sella syndrome (ESS).
- Pulsatile Tinnitus – too high or low CSF pressure alters inner ear pressure.
- Memory and cognitive difficulties
- Exercise Intolerance
Grossly Implausible Distortions?
“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
Both Higgins and Hulens concluded 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. Hulens calls them “arbitrary“. Both believe that instead of being a static condition, that IIH – as one would think any condition that involves liquid flows – exists on a continuum.
Both believe that chronic, mildly increased cerebral spinal fluid pressures are enough to cause symptoms, that the medical profession got off track with IIH early on, and that the condition is likely much more prevalent than believed.
Even if you meet the current criteria, good luck getting diagnosed. For one thing, because the condition is considered to be rare, it’s rarely looked for. A 2012 review stated that:
“Idiopathic intracranial hypertension (IIH) is a rare disorder that typically affects obese women of childbearing age.”
The upshot is that if you go to a doctor with headache, nausea, and other symptoms, if you’re not an overweight woman of childbearing age, IIH probably won’t be suspected.
Both believe doctors have been too focused on a symptom called papilledema – a swelling of the optic nerve that causes it to bulge into the back of the eyeball which doctors can diagnose in their office with an ophthalmoscope. Most of Higgins’s patients, even those with high CSF pressure, did not exhibit papilledema.
The present criteria do allow people without papilledema to get an IIH diagnosis, but papilledema is so associated with the disease that most doctors probably will not consider IIH without it. Even then, you must meet multiple other criteria such as an empty sella, narrowed veins, etc.
Then, if a lumbar puncture is taken and you don’t meet the criteria (>25 cm H2O), you won’t be diagnosed with IIH. Yet Higgins describe multiple instances when cerebral spinal fluid evacuation significantly helped people with ME/CFS. I know of one person whose severe ME/CFS and POTS virtually disappeared for two weeks following their lumbar puncture.
According to the Intracranial Hypertension Research Foundation, many factors can cause IIH including, interestingly, infections and infectious mononucleosis, and autoimmune disorders. So can head trauma, stroke, blood clots, drugs (including tetracycline antibiotics), hypoparathyroidism and Addison’s disease.
The easiest treatment option is that, if you’re obese, losing weight can help.
Otherwise, the treatment involves some heavy-weight drugs including consists of carbonic anhydrase inhibitors (such as acetazolamide (Diamox), Methazolamide (Neptazane), furosemide (Lasix), topiramate (Topamax) and Octreotide.
Treatments have not been well studied. Acetazolamide or Diamox, the most commonly used drug to treat IIH, is used to treat altitude sickness, glaucoma, water retention, non-glaucoma associated eye pressure, seizures, epilepsy and others. Diamox can cause nausea, vomiting, tingling and numbness, fatigue and diarrhea. Topamax had similar efficacy to Diamox, but with fewer side effects.
Surgical interventions can be helpful but are usually only done in more extreme cases where visual loss is occurring.
- Two authors, Higgins and Hulens, propose that increased cerebral spinal fluid pressures; i.e. intracranial hypertension – are common in ME/CFS and fibromyalgia
- Hulens proposes that by pressing on nerve roots and affecting the brain, IIH could cause all the symptoms present in these diseases including pain, fatigue, dizziness, gut issues, cognitive problems
- Both Higgins and Hulens propose that the current criteria of IIH miss many cases of IIH in ME/CFS and FM and other diseases. They assert that IIH exists on a continuum and is far more common than is currently believed
- Bragee found that from 55-83% of ME/CFS patients have IIH
- Higgins studies indicate that even those who do not meet the IIH criteria often benefit from reductions in cerebral spinal fluid
- A migraine study produced a similar outcome
- Treatment options are not particularly inspiring and include strong drugs such as Diamox
- More studies are clearly needed for both diseases.
Conclusion (and the Swirl)
Idiopathic intracranial hypertension is another of those whacky and frustrating conditions which can be triggered in a staggeringly large number of ways. Why it occurs is not clear. On the whole, the condition has not been well-studied, is believed to be rare – and probably isn’t – and doesn’t have a lot of good treatment options.
Recent IIH reviews ignored the work done on IIH in ME/CFS and FM (no surprise, there), but if the Bragee study can be validated (and published in a medical journal), it would certainly raise some eyebrows. While placebo-controlled studies are surely needed, on the face of it, it’s hard to refute a study like Higgins’s which found significant improvement following a dreaded lumbar puncture, of all things.
More studies like Bragee’s and Higgins’s would suggest that IIH – a supposedly rare condition – may actually be a relatively common disorder which badly needs a redo of its diagnostic criteria.
Meanwhile it’s, intriguing to see another issue in ME/CFS that’s characterized by possible circulatory/connective tissue problems. The narrowed veins often found in IIH could derive from a connective tissue issue – an interesting proposition given the high incidence of hypermobility and Ehlers Danlos Syndrome that Bragee recently found in ME/CFS. One of the five clusters of ME/CFS patients identified in a 1,700 person Spanish ME/CFS study were characterized by increased levels of ligament and subcutaneous issues, ligamentous hyperlaxity, endometriosis, low Vit D levels and mood disorders.
Could the increased spinal fluid pressure reflect problems in the brain? The cerebral spinal fluid is one of the brain’s main dumping grounds for toxins. Perrin believes that inhibited lymphatic and cerebral flows from the brain in ME/CFS and FM result in increased toxin levels with the limbic system (hypothalamus, thalamus, basal ganglia) most affected.
ME/CFS and fibromyalgia are not the only diseases in which under diagnoses of IIH may occur. Migraine – commonly found in both FM and ME/CFS – presents another intriguing disease intersection. A 2015 study asserted that many treatment-resistant migraine patients actually have what the authors called IIH without papilledema (IIHWOP) – which may be the most common form of IIH found in ME/CFS as well.
The authors found that normalizing intercranial pressures by removing cerebral spinal fluid was an effective treatment for about a fifth of the patients in their study. They stated that IIH (without papilledema) “is much more prevalent than believed, (and) is commonly misdiagnosed as chronic migraine…”.
Orthostatic intolerance may be another condition in which IIH is hiding out. One study found that standing up makes IIH worse, while another, which probably used a questionnaire, found that 9% of OI patients had IIH. That number could rise significantly if the effects of spinal fluid removal were assessed.
Idiopathic cranial hypertension, ME/CFS, fibromyalgia, migraine, hypermobility, EDS…these conditions seem to be swirling around and mixing into each other and that’s a good thing. At some point, the circle will complete.
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