Large Spinal Study Finds Intracranial Hypertension

Under Pressure: Large Spinal Study Finds Intracranial Hypertension Common in ME/CFS – Health Rising by Cort Johnson | Dec 2019

This article explains how lax spinal joints in the neck can “kink” the vessels holding our cerebrospinal fluid produce common symptoms of Fibromyalgia, CFS/ME and Hypermobility/EDS.

A couple of years ago, there was hardly any discussion of spinal issues in ME/CFS. It’s become clear, though, that spinal issues are present in some patients and can even, in some instances, produce virtually all the symptoms found in this disease.

From cerebral spinal fluid leaks, to spinal stenosis, to intracranial hypertension, to craniocervical instability, the spine is now of intense interest.  

The Study

Signs of Intracranial Hypertension, Hypermobility and Craniocervical Obstructions in patients with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome – Björn Bragée, Anastasios Michos3, Brandon Drum, Mikael Fahlgren, Robert Szulkin, Bo C Bertilson

Created in 2017, the clinic worked quickly to gather what may be the largest array of MRIs collected for ME/CFS.  These 200- plus MRIs were used to produce easily the biggest assessment of spinal issues in ME/CFS. 

The group used the MRIs and a clinical examination to assess

• intracranial hypertension,
• joint hypermobility,
• Ehlers Danlos Syndrome,
• various conditions that can obstruct spinal fluid flows,
• spinal issues and
• craniocervical instability.

The Conditions Assessed

Joint hypermobility – When the joints become too flexible, they can produce

• pain and stiffness,
• clicking joints,
• easily dislocatable joints,
• recurring muscle/joint injuries,
• constipation and irritable bowel syndrome (IBS),
• fatigue,
• dizziness and fainting,
• thin or stretchy skin.

Intracranial hypertension occurs when high cerebral spinal fluid pressure is present. Its symptoms are similar to those produced by a brain tumor and include

• frequent headaches,
• blurry or double vision,
• poor peripheral vision,
• dizziness,
• nausea and/or vomiting,
• stiff neck,
• gait and coordination problems,
• tinnitus (ringing in the ears),
• forgetfulness and
• depression.

Craniocervical Instability refers to a condition where lax ligaments which hold the head up over the body allow the brain to impinge upon the brainstem, spinal cord or nerve roots. Symptoms can be diverse but generally include

• headache,
• neck pain,
• numbness,
• heavy feeling head,
• “bobble-head”,
• dizziness,
• vertigo,
• gait and coordination problems,
• muscle weakness,
• problems with sleep and visual disturbances.

Jeff, Jen Brea and others diagnosed with ME/CFS were found to have CCI. Jeff and Jen Brea made full recoveries from severe cases of ME/CFS after surgery.

Chiari malformation occurs when the cerebellum dips down into the spinal column cutting impeding spinal fluid flows. Its associated

• pain (especially headaches aggravated by coughing/straining),
• weakness – especially in the hands, neck, and arm – and leg pain,
• numbness,
• temperature insensitivity,
• unsteadiness,
• double vision,
• slurred speech,
• trouble swallowing,
• vomiting and
• tinnitus (ringing in the ears).
• Spinal issues that could disrupt spinal fluid flow

The group also looked for structural problems which could result in reduced spinal fluid flows and IH. They included:

• Lowered foramen magnum – The foramen magnum is a large hole at the base of the skull through which several arteries, membranes and ligaments and a nerve passes.
• Lowered position of cerebellar tonsils – the bottom half of the cerebellum can dip down into the upper spinal canal – cutting off spinal fluid flows. In more severe cases, this is called Chiari malformation.

Craniocervical Instability

The authors used something called the clivo-axial angle (CXA) to assess craniocervical instability (CCI).  Experts agreed in the 2013 Consensus Statement on Craniocervical Instability that four tests can be used to assess craniocervical instability. The Consensus states that a clivo-axial angle (CXA) 135º or less is possibly pathological, and that, in some circumstances, stress the brainstem and spinal cord. It is not a definitive test of CCI, but indicates that further investigation is warranted.

Results

An astonishing 96% of patients fulfilled the criteria for fibromyalgia. That number is quite high and may reflect the more severely ill group of ME/CFS patients the authors stated made up the study.  A 1000 person ME/CFS Spain study found that 54% had fibromyalgia.

The Swedes found joint hypermobility (Beighton score >4) in a remarkable 49% of ME/CFS patients. Most of those (41% of total) fulfilled the hypermobility criteria (Beighton score >5) for hypermobile Ehlers Danlos Syndrome (hEDS). Twenty percent of ME/CFS patients met the full criteria for EDS (including hyper-elastic, extensible skin/fragile tissues).

Similar findings have shown up in fibromyalgia.  A large (150 person +) study, though, found joint hypermobility in 64% of FM women. A 236 person study found joint hypermobility in 46% of FM women.

Why the focus on joint hypermobility? Because connective tissue and lax ligament problems also underlie many of the spinal problems the authors looked at.  Lax ligaments, for instance, are responsible for craniocervical instability.

Connective tissue problems can contribute to a lowered cerebellum, foramen magnum, as well.

While more studies are needed, hypermobility and therefore connective tissue problems appear to be common in ME/CFS. We will surely learn more about the incidence of hypermobility and EDS incidence from the CDC’s multisite study.

Intracranial Hypertension

Signs of intracranial hypertension (IH) were common with 55% having high ODNS ( >5.8 mm) and 83% having an ODNS/EDS ratio of greater than .25. The author reported that an ODNS/EDS ratio >.25 was associated with IH with severe symptoms.

Drooping Cerebrellar Tonsils – Fifty-six percent of patients (as opposed to a general population prevalence of 25%) had evidence of drooping cerebrellar tonsils which could be obstructing spinal fluid flows.  Seventeen percent had evidence of the more severe Chiari malformation.

Spinal Issues

The authors reported that eighty percent of the patients had one or more obstructions in the cervical spine. More than one vertebral segment from the neck to the lumbar region was obstructed in 64% of participants.

Craniocervical Instability

A Clivo-axial angle (CXA) angle of less than 150 degrees was found in 114 of the patients (56%). Normal CXAs are reported to range from 150-170 degrees. Henderson reported that “the CXA has a normal range of 145° to 160° in the neutral position”.

Conclusions

A very high incidence of fibromyalgia comorbidity and the almost 50% incidence of hypermobility suggested that this patient population was indeed severely ill. 

The incidence of Ehlers Danlos Syndrome (EDS) appeared to be quite high (20%) as well.

The big news was the very high rates of intracranial hypertension found.  Depending on which measure was being assessed, estimates ranged from a whopping 55% to over 80% of the group.

Plus, the study suggested that a strikingly high percentage of patients (17%) relative to the general population (<1%) may have Chiari malformation.

It seems the more ME/CFS is studied, the more possibilities turn up. Since IH can produce or contribute many symptoms found in ME/CFS, this study, while unpublished, will hopefully incentivize others to assess spinal issues in ME/CFS.