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Spinal cord myelopathy attributed to bilateral jugular vein occlusion and impaired venous drainage....
This gal had demyelinating lesions (just like MS) on her spinal cord because her jugular veins were blocked. This is the disease model we are seeing in patients tested by Drs. Zamboni and Dake.

http://thejns.org/doi/pdf/10.3171/spi.2007.6.1.60

Ventriculoperitoneal shunt failure causing myelopathy in a
patient with bilateral jugular vein occlusion
Case report
WILLIAME. HUMPHRIES, M.P.H.,1PETERM. GROSSI, M.D.,1LINDAG. LIETHE, M.D.,2
ANDTIMOTHYM. GEORGE, M.D.3
1
Pediatric Neurosurgery Service, Division of Neurosurgery; 2Division of Neuroradiology, Duke University Medical Center, Durham, North Carolina; and 3Pediatric Neurosurgery, Children’s Hospital of Austin, Texas

The authors describe the case of a 36-year-old woman with bilateral internal jugular vein occlusion, hydrocephalus, and Dandy–Walker variant who presented with myelopathy that was ultimately attributed to ventriculoperitoneal (VP) shunt failure. Computed tomography (CT) angiography of the head and neck revealed epidural venous engorgement
within the cervical spine, greater that 50% narrowing of the C2–5 spinal canal, and compression of the cervical spinal cord. After successful shunt revision, postoperative CT angiography revealed decreased venous engorgement as well as decompression of the cervical spinal cord, and the patient’s myelopathy improved. This case represents a fascinat-
ing clinical presentation of VP shunt failure, highlighting the physiological importance of the external jugular pathways involved in cerebral venous drainage.


Authors of previous studies have suggested that the vertebral venous system should be adept at tolerating increased venous drainage when internal jugular venous flow is compromised, thereby raising the question as to if there were physiological consequences associated with bi-lateral IJV resection including facial cyanosis and edema.5
These complications illustrate that although the vertebral venous system can drain the cranial vault, it may not be as adept as the jugular venous system at tolerating large increases in flow. In addition, events that alter or impede distal outflow through the vertebral venous system can further compromise the ability of this system to tolerate increases in venous flow. Of interest, our patient underwent resection of a thoracic arachnoid cyst 3 years prior to presentation. Removal of this cyst may have disrupted the distal outflow of the vertebral venous network in the thoracic spine, thereby decreasing its ability to tolerate sub-
stantial increases in flow. Therefore the pathophysiology of this patient’s presentation can be attributed to a primary anatomical predisposition and a secondary acquired insult that ultimately led to spinal cord myelopathy in association with VP shunt failure.
This case illustrates a fascinating clinical presentation of VP shunt failure that highlights the physiological importance of the external jugular pathways of cerebral venous drainage.

And after correct drainage was restored...the myelopathy began to heal!!!
cheer

123

Great post Cheer -

And after correct drainage was restored...the myelopathy began to heal!!!

Heal - has a nice sound to it!
Sharon

But is this myelopathy comparable to that seen in MS lesions?

As I understood this case study, high pressure in the veins that run inside the vertebrae had them engorged and crowding, even crushing, the spinal cord in the space they shared. (Cervical Spondylotic Myelopathy)

Then when the drainage was allowed to flow through the jugulars instead, the internal veins slimmed down and the decompressed cord could heal.

zap wrote:But is this myelopathy comparable to that seen in MS lesions?

As I understood this case study, high pressure in the veins that run inside the vertebrae had them engorged and crowding, even crushing, the spinal cord in the space they shared. (Cervical Spondylotic Myelopathy)

Then when the drainage was allowed to flow through the jugulars instead, the internal veins slimmed down and the decompressed cord could heal.

Terrific point...zap. The collateral veins that formed on this patient put pressure on the spine- the mechanism of demyelination is not exactly the same, but engorged veins also leak and can damage tissue. The main point I wanted to make about this study is that we will hear from doctors that the IJVs are not essential for drainage, that the vertebral and collateral veins can take the load just fine. What these doctors are saying is that is not true. Without jugular drainage, collateral veins will leak, or become engorged and put pressure on surrounding tissue and bone.

What my husband and this patient share is this:
bilateral jugular stenosis from the base of the skull to the jaw
cervical spinal myelopathy (both created by collateral veins)
Increased intracranial pressure

-but they have 2 different diagnosis...because my husband's intracranial hypertension created lesions in his brain, and got him diagnosed with MS. These doctors knew the patient's history with venous issues, so they looked to her jugulars first. And the mechanism for healing is the same...get rid of the engorged collateral veins and let the jugs do their job, and myelopathy can heal.
make sense?
cheer

This is way too cool cheer...thanks i am going to copy and forward to spread the word more

Great paper!! This paper is good and helpful for us but not quite understood correctly I am afraid... forgive me Cheer but I would like to pick the paper apart a bit...

There are a couple of things in that paper that need clarification. First this person had a ventriculo peritoneal shunt and it had failed. That is the biggest problem with this patient. This person has "water on the brain" (hydrocephalus) and they put a tube from the ventricles to the abdomen to drain the extra fluid. That tube had plugged up so her fluid was building up in her brain, but after her shunt was repaired....

After successful shunt revision, postoperative CT angiography revealed decreased venous engorgement as well as decompression of the cervical spinal cord, and the patient’s myelopathy improved

Note they did not repair the jugulars.

Spinal myelopathy means any damage to the spinal cord, not specifically an MS lesion. It can be trauma of any kind that causes a change in function in the areas of the body associated, in this case the cord was compressed by the venous pressure that was impacted by the shunt failure not just the jugular issue, but the really great thing is that she recovered some function! that's good news!

But I would have highlighted this next bit because it is hugely important for us and it may be a good thing to add to the thread for arguments to make to insurance companies regarding our coverage...

Authors of previous studies have suggested that the vertebral venous system should be adept at tolerating increased venous drainage when internal jugular venous flow is compromised, thereby raising the question as to if there were physiological consequences associated with bi-lateral IJV resection including facial cyanosis and edema

This is going to be an issue for us and for all of the people trying to do this. I have seen many of these papers suggesting that the jugulars are not critically important to drainage of the cerebral circulatory system because the vertebral veins supposedly can take the load. These authors show however that is not true after all if there is an increase in flow as there was in this woman with her various issues including a dandy walker issue along with hydrocephalus and shunt failure.

These complications illustrate that although the vertebral venous system can drain the cranial vault, it may not be as adept as the jugular venous system at tolerating large increases in flow

so they are attributing her inability to have the vertebral veins accommodate the flow to the fact of her very heavy load because of her issues.

However,

In addition, events that alter or impede distal outflow through the vertebral venous system can further compromise the ability of this system to tolerate increases in venous flow. Of interest, our patient underwent resection of a thoracic arachnoid cyst 3 years prior to presentation. Removal of this cyst may have disrupted the distal outflow of the vertebral venous network in the thoracic spine, thereby decreasing its ability to tolerate sub- stantial increases in flow.

She had an additional issue in her chest/spinal area that added to her issues (thoracic cyst)--an azygos stenosis sure adds to a persons drainage issues if they have such a thing.

Basically they are sort of saying that probably the jugular system can be removed and compromised and the vertebrals will take the load most of the time--BUT if there is an increase in flow at all then it will not work adequately and potentially venous issues may even impact the spine...

That does not in any way though say that LONG TERM jugular stenosis is fine, no where can I find a paper that follows someone for a long time after a jugular comprimise to see if it was still OK...what seems to happen is someone has let's say a terrible accident then loses their jugs....next week they do a venous CT exam and they say the vertebrals are working just fine. NO one looks like 10 years later to see if they developed any MS leisons or anything.

Frankly if this woman developed a lesion in her brain it would be attributed to her other rather severe brain issues rather than being understood as "MS".

but the most important part Cheer pointed out that a venous congestion can cause a spinal myelopathy. That is the key...

I crossed paths with my answer, that's what happens when you take a long time to send cause you're cooking dinner!
CHeer said:

The main point I wanted to make about this study is that we will hear from doctors that the IJVs are not essential for drainage, that the vertebral and collateral veins can take the load just fine. What these doctors are saying is that is not true. Without jugular drainage, collateral veins will leak, or become engorged and put pressure on surrounding tissue and bone.

Yes right on!!

HERE is a paper on what the problem is regarding how the jugular stenosis is seen

Venous outflow of the brain after bilateral complete jugular ligation.

Ensari S, Kaptanoğlu E, Tun K, Gün T, Beşkonakli E, Celikkanat S, Dere H, Cekirge S.

Ankara Numune Education and Research Hospital, Department of Otorhinolaryngology, Ankara, Turkey.

A case of a patient with bilateral internal, external, posterior external and anterior jugular vein ligations and excisions performed in the neck due to a larynx tumor is presented. Radical neck dissection is a standard otorhinolaryngological procedure in the management of head and neck cancer patients with bilateral lymph node metastasis to the neck. Sacrifice of both internal and external jugular veins bilaterally has been recognized as a dangerous approach leading to intracranial hypertension with subsequent neurological sequela and death. In this report, we aimed to demonstrate how venous outflow from the brain diverts after jugular venous system obliteration. After bilateral jugular vein ligations, digital subtraction angiography (DSA) showed that the venous drainage route of the brain had been diverted from the jugular veins to the vertebral venous plexus.

This is pretty well commonly thought that the jugs are not necessary the vertebrals can just take up the slack no problem, but once again these people test it out mere days after they do their work on the person. This one had cancer, did she even live 10 more years? If she did and she had lesions in her brain after time was it attributed to MS or some post cancer thing that just happened? the assumption that it is fine because it looks OK the week after surgery may be very short sighted. The abstracts that follow affirm the idea that an overloaded venous system causes damage contrary to the idea offered here that it works just fine, and in support of CHeers paper that shows taking out the jugs may not be OK

My own stenosis showed significant pressure differences and relief of presssure after the stents were in.

HERE is a paper on venous hypertension causing myelopathy in the cervical cord

STUDY DESIGN: Case report. OBJECTIVE: We describe a patient who developed a myelopathy associated with a noncompressive herniated cervical intervertebral disc at the same level. We provide clinical and radiological evidence that reveals that even though the disc herniation did not compress the spinal cord, it diminished venous blood flow out of the spinal cord, possibly resulting in a venous hypertensive myelopathy (VHM). SETTING: Baltimore, MD, USA. CLINICAL PRESENTATION: A 29-year-old woman developed a cervical radiculopathy, followed by a slowly progressive cervical myelopathy associated with a herniated C5-C6 disc. Magnetic resonance imaging showed a noncompressive disc herniation, a swollen spinal cord with increased T2 signal most prominent at the site of the herniated disc, extending several levels above and below the disc. The patient was diagnosed with acute transverse myelitis (ATM) and was started on IV steroids. However, unlike most cases of transverse myelitis, spinal fluid analysis was noninflammatory. In contrast, several features suggested that the patient instead had VHM. We suggest that the disc herniation resulted in impaired drainage of blood from the spinal cord through compression of the venous plexus near the intervertebral foramen. INTERVENTION: Although the patient did not recover function following high-dose steroid administration, she recovered completely following C5-C6 discectomy and fusion. CONCLUSION: To our knowledge, this is the first report of likely VHM in the absence of a spinal arteriovenous malformation. We suggest that some patients diagnosed with ATM in the setting of extrinsic spinal column abnormalities may actually have a noninflammatory myelopathy associated with impaired spinal venous drainage.

ANd a stunner HERE

Diagnosis of venous circulation disorders in the cervical portion of the spine and spinal cord using selective phlebography]

[Article in Russian]

Ogleznev KO, Tsuladze II.

Selective phlebography of the major branches of the vena cava superior, supplemented, if necessary, by selective catheterization of the spinal vein, was employed to study the disorders in the cervical section of the spine and cord. Twenty-five patients with myelopathies of obscure origin were examined. The main symptoms were stenoses and atresias of the orifices of the internal jugular veins and compression and stenoses of the brachiocephalic trunks. The possibility of a reverse venous blood stream from the nonspinal veins to the epidural ones and backward was confirmed. Therefore, a disorder of the venous outflow from the spinal channel, on the one hand, and intensification of the venous blood inflow, on the other, may develop under certain conditions, resulting in intrachannel hypertension. Eight patients with pathologic changes in the veins (decompressions and angiolysis) were operated on.

These people had myelopathies caused by venous backflow............ that were fixed after surgery to relieve the atresia (blockage from being formed wierdly) or stenosis (another kind of blockage).

anyone hanging out on this forum knows this is exactly what we are talking about here. 1993. Not specifically MS but "myelopathies of unknown origin"

HERE

Spinal cord oedema due to venous stasis.
F C Henderson
H A Crockard
J M Stevens


Department of Surgical Neurology, National Hospital for Neurology and Neurosurgery, London, UK.
Venous hypertension and stagnant hypoxia in the human spinal cord are poorly understood. We report a case in which a partial Brown-Séquard syndrome resulted from obstruction of venous drainage on one side of the spinal cord. Neurological deterioration and eventual recovery paralleled the formation and clearance of oedema. The clinical and MRI findings support the contention that spinal cord injury arising as a result of obstruction to venous outflow occurs primarily in the white matter, spreading secondarily to the grey matter, and observations that obstruction of venous flow in the high cervical spinal cord results in changes in the lower cervical cord. MedlineID:
PMID: 8492903

Primarily in the white matter----secondarily to the grey matter.....

Uh, BINGO!!
is this fun or what?
marie

That last paper is a winner, Marie! (BTW...never feel bad for correcting my inaccuracies! Many things are still flying over my head...)

But I get this....

The clinical and MRI findings support the contention that spinal cord injury arising as a result of obstruction to venous outflow occurs primarily in the white matter, spreading secondarily to the grey matter, and observations that obstruction of venous flow in the high cervical spinal cord results in changes in the lower cervical cord

Showing first in white matter, then grey....the same kind of damage we're able to detect in the MS brain, now thanks to 7tesla MRI. It's not just demyelination...venous obstruction effects all of the brain or spine...that's why we don't need to see lesions to have damage.
Good find, Marie!!
cheer

Holy myelopathies Batman

mrhodes40 wrote:HERE is a paper on venous hypertension causing myelopathy in the cervical cord

STUDY DESIGN: Case report. OBJECTIVE: We describe a patient who developed a myelopathy associated with a noncompressive herniated cervical intervertebral disc at the same level. We provide clinical and radiological evidence that reveals that even though the disc herniation did not compress the spinal cord, it diminished venous blood flow out of the spinal cord, possibly resulting in a venous hypertensive myelopathy (VHM).

The following text is from one of my MRI reports that was done 2 years after I was initially diagnosed. The above reference makes me wonder if there's a connection in my own case?

Impression:
MR imaging of the cervical spinal cord demonstrates only minimally increased T2 signal intensity within the spinal cord at the C6-C7 disc space level, the site of the prominent diffuse disc bulge. Findings are less prominent than on the prior examination. Imaging of the cervical spinal cord is otherwise unremarkable.

MR Cervical Spine.

Technique: Sagittal and axial T1 and T2 images were obtained of the cervical spine with and without contrast.

Findings: There is straightening of the cervical lordotic curvature. At the C6-C7 level there is a broad-based, disc bulge which causes anterior extradural indentation of the thecal sac without compete effacement of CSF anterior to the spinal cord. There is not a focal disc herniation.

There is mild narrowing of the neural foramen, bilaterally. There is no alteration in spinal cord contour. There is minimally increased T2 signal intensity within the spinal cord posterior to the inferior aspect of C6 and the superior aspect of C7 which is distinctly less prominent than on the prior examination.

Spinal cord at other levels is normal. There are no structural alterations of the foramen magnum.

I should probably add that at 2 years post diagnosis that I was doing pretty good. I was still riding my bicycle 8+ miles and walking 2 miles in only 30 minutes. I don't remember exactly, but I believe that this was either a follow-up MRI or one that was done for other symptoms that I was having (I remember having a problem with my foot which felt like I had a piece of bark stuck just under the ball of my foot. I was taking Vioxx at the time in order to block the effects of Avonex and it worked great for this however a colleague shared a paper with my that stated that Vioxx was linked to aseptic meningitis (i.e., nonbacterial inflammation of the meninges) so I stopped taking it and my symptoms disappeared shortly afterwards, this was before Vioxx was pulled from the market and I'm grateful that I only took it for a short time).

In any case, at least on the surface, the MRI report seems to be consistent with the CCSVI hypothesis. It looks like the pieces of the puzzle are starting to fit together or maybe I'm just looking at it with CCSVI colored glasses.

Comments on this are of course welcome as always.

NHE

Man,

Miss a day and miss a lot!! This is all so heartening to me as I'm just trying to limp over the finish line. All kinds of bad stuff happenning here. The 'roids worked for a few days, but things are back and with some new stuff, so I'm reading this and hoping it allows me a good day. The last few have been almost an hour at a time. I'm getting by, but I've got new hurdles. Last night (this is after over a year of successful cathing and almost through the night sleeping) I peed the bed 4 TIMES. I'll spare all the details, but the fluid intake was the same as always. To give an idea (I think it may be my kidneys) I cathed at 11:00 after no fluids after 8:00 and had already done it by 12:00, and to boot I cathed again and had 350ml still in there AFTER the incident. My kidneys are pulling overtime for some reason. Something got whacked in my last relapse and my level of function has dropped; this is two days in a row of this. It's been getting worse and now is like an open line from my kidneys to the sheets.

So sorry to hijack; really, this is just giving me a little rosy outlook that hopefully helps me calm down today. I have the worst timing. I was doing so good until a few weeks ago and am so close to getting this done. Sucks to be missing so much sleep too. I can't believe I scheduled this out six weeks so I could make my camping trip next week. I probably will enjoy that like root canal.

Once again, great news and sorry to Eeyore this great news, but I'm just needing inspiriation!

Lew, There are so many others here who are so much better with words of inspiration than I am. Right now I'm feeling the injustice of this all for you -- you're so close, and now to be hit with all this crap...it just sucks. See, that's not inspirational, so I really hope someone else jumps in, but if it's any help to you, I'm hating the MS puppeteers on your behalf with everything that's in me. So I'll take that monkey off your back so you can concentrate on the good stuff that's coming and get yourself over that finish line. I'll leave it to someone else to say it will all work out, you'll get to Stanford and you'll move ahead from there.

Lew I am glad that these papers help out with the mood. I got more coming in just a sec, but what a DRAG . You know what though? strokes can whack a whole lot of brain and people can still get better...and polio people could recover a whole lot too. here's the good thing the flare is so close to the procedure that you will have a better chance of recovering something important. THe thing about us MSers is that we keep getting whacked all the time, buying the model here, with more venous pressure. SO I think this should change the face of relapse recovery altogether I am glad it is so close for you.

Also given that you have been on tysabri it makes a good case for the standard therapies simply not cutting it for you (or me) personally. Thank God for Dr Dake and a new approach available. I mean if you are doing fine, go ahead and wait for the research to play out, but some of us got no wait! But we are lucky!

HERE is a paper on arterio venous fistula and we've had a bunch of them on this forum but I used the TIMS search function and I do not see that we had this specific one.......

Spinal dural arteriovenous fistulas (SDAVFs) are the most common type of spinal vascular malformation. The arteriovenous shunts, located entirely outside the spinal cord, cause a clinical picture of chronic progressive myelopathy believed to arise from the effects of increased venous pressure and impaired venous drainage on the spinal cord. Despite their well-described clinical and angiographic features, no reports have documented the spinal cord pathology in a case of angiographically or pathologically proven SDAVF. We report such a patient in whom a spinal cord biopsy supported increased venous pressure as a mechanism of neurologic dysfunction

OOHHHH!! Another one!!

OBJECT: Spinal cord edema is a rare radiological finding in chronic degenerative disorders of the spine. Between 1997 and 2001, the authors treated six patients with cervical spondylotic myelopathy in whom postoperative spinal cord edema was demonstrated. The authors describe the radiological and clinical features of this unusual condition. METHODS: The six patients were all men, and ranged in age from 44 to 72 years. All patients presented with mild quadriparesis and underwent laminoplasty or anterior fusion. Preoperative magnetic resonance (MR) imaging revealed marked spinal cord compression with intramedullary hyperintensity on T2-weighted sequences and spinal cord enhancement at the compression level after administration of Gd. After surgery, spinal cord edema was observed in all patients; the spinal cord appeared swollen on the postoperative MR images. Preoperative and postoperative Gd-enhanced MR imaging demonstrated clear enhancement of the white matter at the compressed segment Neurologically, five of six patients experienced good improvement of symptoms; however, the spinal cord edema as documented on follow-up MR imaging persisted for several months after surgery. CONCLUSIONS: The radiological characterization of spinal cord edema was based on the reversible white matter lesion most likely caused by disturbed local venous circulation induced by chronic spinal cord compression. Such unusual MR findings in cervical spondylotic myelopathy should be differentiated from intramedullary spinal cord tumors, demyelinating disorders, or inflammatory processes.
MedlineID:
PMID: 12859052

ANd that one adds to the paper NHE was commenting on .....another one.

NHE I am with you on your findings. IT does seems to be coming together like a puzzle there. Maybe we do have rose colored glasses but given that the other explanation for what ails us leaves these details hanging out like stupid anomalies that are somehow just coincidental, this looks pretty good.

Island Girls is my friend she fits this issue too---several spinal cord herniations and problems thereby. She was also told it was unrelated but connecting the dots with the earlier paper wherein the herniation can obstruct a vein--which is exactly what IG said was going on in her neck--and cause white matter then grey matter issues..........well geez.

I can't call her yet its too early...can't wait to tell her to read this too...she goes the 6th to Stanford
marie

More...............
HERE

Venous manifestations of spinal arteriovenous fistulas.

Andersson T, van Dijk JM, Willinsky RA.

Department of Neurosurgery and Neuroradiology, Karolinska Hospital, S-171 76 Stockholm, Sweden.

Spinal dural AVFs, the most common type of spinal arteriovenous malformations, are symptomatic because of venous hypertension and congestion. This has been referred to as venous congestive myelopathy. The typical MRI findings that reflect venous congestive myelopathy include peripheral T2 hypointensity that outlines a T2 hyperintensity within a swollen spinal cord. Enlarged perimedullary vessels are typically present. Contrast-enhanced MRA has become instrumental in localizing the site of these fistulas. Spinal epidural AVFs and the perimedullary spinal cord AVFs may also present with a congestive myelopathy and have similar findings on MRI. Angiography remains the gold standard for characterization of the angioarchitecture of spinal vascular malformations. [figure: see text] Multidisciplinary treatment planning is mandatory and requires knowledge of the natural history of these vascular lesions.

It occurs to me that the neurologist who orders the head and neck MRV is thinking of this situation and thinking to eliminate this known problem rather than understanding that it is not a arteriovenous fistula that is causing the congestive myelopathy in this case it is a stenosis further down.

They are not understanding that part but are leaning on what they know already about things they need to differentiate for the MS patient. This is one of those differential diagnoses to make and they are aware of it. They are not appreciating the difference between this and what we actually are talking about....venous congestive myelopathy but from a venous stenosis.

but note the findings there with regard to the hyperintensities on T2 weighted MRI....
marie