Understanding jugular venous outflow disturbance

JenB

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I just wanted to share an article that was recently posted to our Facebook group on ME/CFS and structural diagnoses. A woman in it who meets ME/CFS criteria was recently diagnosed with bilateral internal jugular vein stenosis (IJVS). This is considered a congenital condition, however she never had symptoms before contracting Dengue Fever. Her doctor hypothesizes that since Dengue Fever can thicken the blood, her infection took a previously asymptomatic congenital condition and made it symptomatic. This hypothesis is supported by the fact that a drug called Stugeron, a vasodilator, improves her symptoms.

IJVS, as this article describes, can cause symptoms that “mimic” intracranial hypertension including altered CSF flow and cerebral hypoperfusion:
https://onlinelibrary.wiley.com/doi/full/10.1111/cns.12859


This pings for me given the conversation in another thread about how there might be a range of diagnoses that can cause intracranial hypertension and/or cerebral hypoperfusion, resulting in ME/CFS symptoms.

It also pings for me as it is an example of a concept I keep encountering: that there are patients who have a congenital, anatomical abnormality that prior to an infection, is completely and largely asymptomatic, and that only becomes apparent after a major inflammatory event. That was the case for me with tethered cord syndrome and, apparently, in this patient with IJVS.
 

SlamDancin

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A couple really quick initial impressions @JenB, and thank you for posting this here as I don’t use Facebook for several reasons, are that in all my reading of intracranial hypertension so far they mention a drug called Topiramate as the current gold standard treatment, and I’ve started as of a few days ago as I have several symptoms resembling it, but that what’s happening is a breakdown in a process called Neurovascular Coupling (NVC). It’s the CNS telling the peripheral vasculature to meet energy demands by supplying the raw materials (blood, CSF) and Dr. Van Elzakker is showing that even holding our breaths causes major immediate deficits in that process taking place compared to healthy controls. He hasn’t named NVC by name yet but my research suggests this is the process that is screwed. It involves a lot of players we are well familiar with like glutamate, NMDA, nitric oxide, GABAA, etc. I think he would be a fascinating person to reach out to Jen. He’d find your recovery story fascinating considering he is convinced the brainstem is the epicenter of this disease!!
 
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*Reviving this thread as I'm interested in the topic.*

I have recently been diagnosed with IJVS, significant on one side and severe on the other (the vein disappears from CT imaging because it's so pinched!). My jugulars both appear to be squashed between the styloid and the transverse process of the C1 vertebra.

I'd be really interested to know if anyone has any knowledge about IJVS and its relation to ME/CFS.

Some thoughts and questions:
  1. Could IJVS on its own be enough to cause ME symptoms? A neurosurgeon in the UK has put my CXA and a few other measurements at just in the abnormal range, but not very significantly.
  2. I'd love to hear anyones experiences of having a styloidectomy, shaving of the C1, or muscle "trimming" to make space for the jugular vein.
  3. I wonder if it is possible to create more space for the jugular by improving posture? I have slight kyphosis and forward head posture, and my ME symptoms started after landing on my head during a backflip(!). Perhaps this caused a misalignment of bones that could be corrected.

I'm hoping to get an appointment with Dr Higgins, who specialises in this area, so hopefully I can update for anyone who has the same issue in the future.
 

JenB

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*Reviving this thread as I'm interested in the topic.*

I have recently been diagnosed with IJVS, significant on one side and severe on the other (the vein disappears from CT imaging because it's so pinched!). My jugulars both appear to be squashed between the styloid and the transverse process of the C1 vertebra.

I'd be really interested to know if anyone has any knowledge about IJVS and its relation to ME/CFS.

Some thoughts and questions:
  1. Could IJVS on its own be enough to cause ME symptoms? A neurosurgeon in the UK has put my CXA and a few other measurements at just in the abnormal range, but not very significantly.
  2. I'd love to hear anyones experiences of having a styloidectomy, shaving of the C1, or muscle "trimming" to make space for the jugular vein.
  3. I wonder if it is possible to create more space for the jugular by improving posture? I have slight kyphosis and forward head posture, and my ME symptoms started after landing on my head during a backflip(!). Perhaps this caused a misalignment of bones that could be corrected.

I'm hoping to get an appointment with Dr Higgins, who specialises in this area, so hopefully I can update for anyone who has the same issue in the future.
Having had “internal jugular vein compression” due to my omohyoid muscles, a problem that developed after my craniocervical fusion and tethered cord release surgeries, I can say for me personally that the symptoms were very different from the symptoms due to CCI and TC, let alone ME. However, they can be very disabling, can probably interact with the symptoms caused by the other pathologies, and can contribute to intracranial hypertension, which can cause many ME-like symptoms.

Given your history, it sounds like in addition to Higgins, working with an EDS-literate physical therapist could be really helpful. There is also an upper cervical chiropractor in the UK that many have found useful (see the FB group). In short—it is possible to make improvements on all of these conditions, without surgery, depending on the severity and context of your case. But you need to consult with additional practitioners and try to get differing views/treatment modalities to inform your plan of care, a significant financial and logistical challenge everywhere, but particularly in Europe.

Do you have Eagle’s Syndrome symptoms? Generally, you should be asymptomatic when your head is in neutral. I have hypertrophic styloids (> 2.5 cm) but was found not to have Eagle’s Syndrome.
 
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Hi Jen,

Thanks for your response. Yes I hope to see that chiropractor in due course. I'd love to find out if anyone has had styloid/C1 compression of the jugular and resolved it non-surgically, but as I understand it this kind of compression is somewhat rare/under-researched. And you're right, the costs can add up!

I have been assessed by a hypermobility expert who saw no signs of anything along those lines.

I don't have any Eagle's symptoms, apart from tinnitus and headaches (which are so common). However I measured my styloids on my CT scan and I'm not certain about my technique but I make one of them to be 3-3.5cm (corresponding to the more compressed jugular). The other is around 2.5cm.
 

JenB

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Hi Jen,

Thanks for your response. Yes I hope to see that chiropractor in due course. I'd love to find out if anyone has had styloid/C1 compression of the jugular and resolved it non-surgically, but as I understand it this kind of compression is somewhat rare/under-researched. And you're right, the costs can add up!

I have been assessed by a hypermobility expert who saw no signs of anything along those lines.

I don't have any Eagle's symptoms, apart from tinnitus and headaches (which are so common). However I measured my styloids on my CT scan and I'm not certain about my technique but I make one of them to be 3-3.5cm (corresponding to the more compressed jugular). The other is around 2.5cm.
It’s possible changing your posture could create a little more room there, and thus reduce symptoms, but it entirely depends on the architecture/morphology of that area, for you personally. In this respect, I am not sure that more research would be very helpful here as compared to assessments from a skilled neurosurgeon/ENT.

Has anyone you consulted with done a 3D reconstruction of your CTscan? When my doctor was trying to rule out Eagle’s, they looked at both venous outflow (using CT w/ contrast + venous passes, and also an invasive angiogram—unnecessary for Eagle’s, but helpful if trying to rule out many different possible causes for the vascular problem) and the bone structure of the space. Theoretically, they should be able to tell you whether postural correction is likely to be of benefit, e.g., by changing the angle of the styloid slightly.

I believe if you don’t have rotational symptoms, but rather permanent compression of the internal jugular veins at C1, this is called “pseudo-Eagle’s.’
 
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...it entirely depends on the architecture/morphology of that area, for you personally. In this respect, I am not sure that more research would be very helpful here as compared to assessments from a skilled neurosurgeon/ENT.
Sensible thinking, as always :). I am definitely seeking some professional assessment.

Has anyone you consulted with done a 3D reconstruction of your CTscan?
Not that I'm aware of, but I have, and I can take that to show any specialists.

I wonder if my slight kyphosis and forward head posture has been an unconscious effort by my body to create more space for my jugulars. It makes sense to me in terms of geometry, and I think would make it less likely that postural correction would help with this particular issue.

“pseudo-Eagle’s.’
Thanks! This will help direct my learning