New procedure to treat pseudotumor cerebri

Published on March 13, 2013 at 9:28 AM · 8 Comments

A team of interventional neuroradiologists and neurosurgeons at Johns Hopkins reports wide success with a new procedure to treat pseudotumor cerebri, a rare but potentially blinding condition marked by excessive pressure inside the skull, caused by a dangerous narrowing of a vein located at the base of the brain.

The Johns Hopkins team's latest study, to be published in the Journal of Neuro-Ophthalmology online March 14, is believed to be the first to show how directly lowering pressure inside the vein alleviates the condition and improves vision.

The study report on 12 patients describes the team's novel use of intravascular ultrasound imaging to delicately thread an expandable metal stent, roughly two inches long and attached to a catheter, through an opening in the groin, all the way to the main blood vessels in the neck and shoulders draining fluid from the brain. The goal is to precisely position the stent across the narrowed portion of the vein, called the transverse sinus, where it expands, allowing blood to drain more freely and relieving fluid pressure in the brain. The vein narrowing, or stenosis, is considered to be the leading cause of pseudotumor cerebri. The condition earned its name, which translates to "false" tumor of the brain, because in the era before detailed brain imaging became available, surgeons used to operate on people with similar optic nerve swelling, suspecting brain tumors, yet they would find none.

Because the transverse sinus drains cerebrospinal fluid, constriction of the vessel causes fluid backup and raises intracranial pressure, which, if left untreated, can lead to permanent damage to the optic nerve, blurred vision and eventual blindness. Traditional treatments include drugs that reduce pressure and the surgical placement of a straw-like shunt between the lower spine and brain, or between the lower spine and stomach cavity to drain excess

According to lead study investigator and Johns Hopkins interventional neuroradiologist Martin Radvany, M.D., the new, minimally invasive procedure, known as transverse sinus stenting, takes about two hours to perform, and could serve as a long-term, if not permanent fix to what he says is a vexing and growing problem, seen mostly in obese, premenopausal women between the ages of 18 and 40.

Radvany, an assistant professor at the Johns Hopkins University School of Medicine, says the new stent procedure has the potential to supplant the current standard of care using shunts, which come with risk of infection, including meningitis, and concerns about too little or too much drainage, and the need for shunt replacements every few years.

"Our study results, if validated in more patients, give us more than an alternative to shunts and bypassing the consequences of pseudotumor cerebri," says study co-investigator and interventional neuroradiologist Philippe Gailloud, M.D. "Our latest research helps us get to the root of the problem so that we can stop and possibly prevent the vein from narrowing in the first place," says Gailloud, who is also director of interventional neuroradiology at Johns Hopkins.

Study results showed that 10 of 12 patients treated had a complete and lasting recovery, with internal vein and spinal pressure readings returning to normal, loss of most symptoms, if not total reversal, and CT scan images showing no return of narrowing in the transverse sinus, nor any worsening optic nerve damage.

Pressure readings taken from inside the narrowed vein immediately before and after transverse sinus stenting showed initial differences between the healthy and constricted areas of the vein as high as 28 millimeters of mercury. These pressure gradients dropped back to and remained at 2 millimeters of mercury or even equalized with normal vein pressure at 0 millimeters of mercury.

Swelling in the eye went down to normal and never returned in 11 out of 12 patients treated. Extensive vision testing, including line-reading tests for visual acuity, distinguishing colors, and peripheral vision, found that eight patients' vision returned to normal. All had an immediate disappearance of headaches and hearing noises inside their heads.

One patient's symptoms later came back and deteriorated after treatment, and another's condition failed to show lasting signs of improvement, but did not get any worse. Both ended up having surgical shunts placed.

Gailloud, an associate professor at the Johns Hopkins University School of Medicine, points out that pseudotumor cerebri is one of many kinds of idiopathic intracranial hypertension, whose origins are unknown. But, he says, the immediate healthy rebound in vein pressure due to the stenting proves that the narrowing of the transverse sinus vein was not due to forces outside the vein, but instrinsic to it. "Now that we know more about what is actually happening, we can orient our research towards finding out what causes the transverse sinus to narrow."

In the study, 11 women and one man were offered transverse sinus stenting as an alternative to surgical shunting, but only after weight loss, dieting, and drug therapy with acetazolamide, or Diamox, a medication that lowers pressure in the brain, had failed to alleviate their symptoms. Participants came from the mid-Atlantic states, and ranged in age from 21 to 55; all were obese, with an average body mass index of 32.6.

All were treated at The Johns Hopkins Hospital between January 2008 and June 2011, and had spinal fluid pressure tests, angiograms and CT scans to confirm that pseudotumor cerebri and vein narrowing were causing their symptoms, the most serious of which is blurred vision, a sign of permanent nerve damage. Most study participants, Gailloud says, experienced initial symptoms of severe headache, with over half reporting "a whooshing noise" inside their heads. All were monitored in the hospital for two days after IVUS, which only required general anesthetic, and were followed through periodic checkups for between one year and as long as four-and-a-half years.

Senior study investigator and neurologist Abhay Moghekar, M.D., says the team will continue to monitor patients who choose transverse sinus stenting instead of surgical shunting, and to compare their progress over several more years.

Moghekar, an assistant professor and director of the Center for Cerebrospinal Fluid Disorders at Johns Hopkins, says having alternative and permanent therapies for pseudotumor cerebri is increasingly important, as the condition appears to be tied to obesity, now an epidemic in the United States. He says that a decade ago, he saw less than two dozen cases per year, but now sees well over a hundred.

"Pseudotumor cerebri is a serious condition, in which the prospect of going blind is very real," says Moghekar. "Until now, we had little evidence of why surgical shunting or IVUS stenting worked," he adds, pointing out that further studies can use pressure gradients to distinguish between people who respond best to one procedure or the other.

Source:

Johns Hopkins University School of Medicine

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Comments
  1. Dennis Gonterman Dennis Gonterman United States says:

    To any one paying attention this is what my medical condition is. I thought my eyesight was going to be history but with large amount of drugs (diamox) my eyesight slowly improved and got much better than it was prior. The remaining symptom are still with me. Intercracial pressure and pain have become a daily part of my life.

  2. kristal gonsalves kristal gonsalves United States says:

    My sister n law has  this  condition  since  2011, and  now  has  no medical coverage  and  has  no  way to  see any  doctors. She  lives  in  pain  each  day  and  recently  she  has  started  to  have  memory  loss.  her  employer  does not offer  any medical  coverage  and has cut her  work hours  in the hopes  that she  will resign  and  be  left with no income. I  was able to  find  a free doctor clinic in her  area but  they were unable to  help  her  because she  needs to  see  a specialist  they  were  able to  give  her a RX  for  pain  that  she  was  given at  the time  of  her  diagnosis in 2011.  she is  37 years  old and  a mother  of  4. She  was  denied disability  because  she  worked and  now with  her  hours  being cut  I  have  no idea  how  she can get  treatment. Does  anyone  know  of  any  Neurologist  that  offer emergency   treatment  to people in  need? we  can't reapply  to  disability  without  current  documentation  regarding  her  condition.

  3. Ria Robinson Ria Robinson United States says:

    I've had this condition since 2010. I've had two spinal taps and I take diamox. I no longer have migraines but I still suffer from double vision. All in all I'm doing much better

  4. Sherri Mendenhall Sherri Mendenhall United States says:

    I was diagnosed in 2009, I'm allergic to diamox so that option was out quick.  In 2010 i received my first VP (ventricular peritoneal) shunt, which had to be revised in 2011 due to scar tissue.  After my first surgery the relief was immediate and amazing, I honestly had no idea how much pain I had been in until it went away.  Unfortunately I have had a very hard time getting the weight off, primaryily because it is hard to exercise with the vertigo and double vision.  I have been having spinal taps every 2 - 3 months where they pull off 40ccs.  I really dont want to crack my brain open again for yet another revision.  Has anyone actually had this stent surgery and if so how is it working out?  I am reallt tired of the pain, vertigo double vision and "gray" vision zones that come along with this.

    Hopeful in the NW

    • Karen Pooley Karen Pooley United Kingdom says:

      Hi Sherri,
      I had my first stent back in 2000, this procedure is not new here, my second one was placed in 2001 and I had eleven years where things were pretty damn good Smile The operation itself is non invasive in comparison to having any kind of shunt fitted, the stent goes in through the neck and I was only required to stay in hospital overnight.
      I have since had three more stents placed as in 2012 my pressures went up again, have to say though I haven't had to worry about infections or revisions, so very happy being stented, I hope this small piece of information helps you Smile x

  5. Michelle Goebel Michelle Goebel United States says:

    I went through all of 2012 telling my primary doctors that I was having severe migraines, which I never have had before, my vision was very blurred and I felt disoriented for periods of time.  My short term memory was going as well as the horrible ringing in my ears. My doctor said it was just stress because I was going through a divorce.  Then on 12/17/2012 I lost my vision completely while at work, I was taken to the hospital and they found that I have a small brain tumor but that wasn't causing the blindness.  It took until 02/2013 during a lumbar puncture to find that I had extremely elevated spinal fluid in my brain.  I have been on Diamox for over a year which has caused me to be so low in Potassium that my heart is now not pumping sufficiently.  My kidneys are also being impacted by the Diamox.  I have had 7 lumbar punctures and have been having 3 different types of seizures.  I had a grand mal seizure where I was passed out on my left leg for over 7 hours and cut off the circulation to my leg so I now don't have full feeling or use of my leg.  Pseudotumor has caused me to be permanently disabled and my neurologist is working to help me get to John's Hopkin's in hope that I can get the help I need.  The neurosurgeons in Orlando will only do a shunt and that is just not an option for me.  I need help and I'm a single mom who lost her job after 14 years with my company because I was missing so much work because of this.  I can't keep going like this, this is not living it is suffering everyday.

  6. lucky steph done lucky steph done United States says:

    I continue to be disappointed with the medical fields notion that obesity has caused IIH.  If this were true then it would not be idiopathic.  Granted the article did not directly say obesity caused the IH it was implied in at least 3 times.  It is easy to blame the patient for being obese and in my opinion is often used as a way to shirk responsibility if a patient dosent respond to treatment.   It is akin to saying "Ms Doe dosent want to get well enough to lose weight so it is her fault".  I was thin when the daily headache started and gained a massive amount of weight through the years.  I want every physician to sit down a minuet and think about how hard it is to be active when it feels like you have a marching band in your head.  
      
       I am also confused by the last statement about "Until now, we had little evidence of why surgical shunting or IVUS stenting worked,".  It is possible I am misreading the statement or not comprehending what the intent of the statement is.  It was my understand it was a proven fact that shunting was understood to work from the removal of the excess spinal fluid.  

    I am glad to see some success stories of the stenting here, alas the majority of people with IIH that I  know do not qualify for the stenting process.  So we return to square one.

The opinions expressed here are the views of the writer and do not necessarily reflect the views and opinions of News-Medical.Net.
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