Syrinx or black hole?

[pokey]

I have had symptoms since 1998, such as  R arm/leg numbness, tingling, bladder spasms, blurred vision. Recently had a few episodes of vertigo. All intermittent. Needless to say, I have had a few MRI's to rule out MS. One T2 hyperintensity near left frontal horn. 1 Cervical MRI normal.

Recent MRI of brain and throacic spine read as small mid-thoracic cord syrinx. Exact borders are difficult to define, but it appears to extend from appx lower T7 to mid T9. Measures appx 2mm in diameter. The abnormaility is centrally located and follows cerebral spinal fluid on T1/T2 sequences. No abnormal enhancement. No fracture. There is a T8 superior endplate Schmorl's node. No paraspinal abnormailty is detected. Impression: Very small T7-9 cord syrinx. No definite etiology is demonstrated. The cerebellar tonsils are boderline low, but DO NOT have the classic configuration of a Chiari I malformation.

Questions:

Could the syrinx actually represent a "black hole" rather than a syrinx? What would be the MRI differences between the 2?

After having several prior MRI's, this is the first time it has been mentioned about the cerebellar tonsils which decend appx 5mm below the foramen magnum. My guess, because its not significant?

I understand the T7-9 thing be responsible for the leg and bladder problems, but really don't explain the arm and vision problems. Any ideas?

I know without seeing the actual films it is difficult to say much but any suggestions as to next steps that sould be taken will be greatly appreciated.

Thank you in advance.
Pokey

[CCF-Neuro-M.D.-JT]

I don't think there is such a thing as a black hole in the spinal cord. If there is indeed a small fluid filled space in the center of the cord visualized on the MRI, it is likely to be a syrinx. You're right about the arm and vision problems not explained by the syrinx in the middle of the back, as these symptoms represent higher regions of the spinal cord and brain. Also, I'm not quite sure as to whether or not the thoracic syrinx is really causing your other symptoms as the size (2mm) is very small. Can't comment on the possibility of Chiari as I would have to see the films for myself. If MS is really a consideration, further evaluation such as visual and somatosensory evoked potentials can be of help which can assess the vision as well as numbness/tingling symptoms. Also, a spinal tap can be considered. This of course all needs to be taken into clinical context and discussed with your neurologist so that unnecessary testing is not ordered.Good luck.

[justy]

Please see a Chiari expert. One of the best is Dr. Thomas Milhorat In Manhasset New York.

[Annika]

Dear Pokey,

I agree with Justy.  There is a lot of new research now on how to diagnose a Chiari.  Dr. Milhorat has published some excellent papers on this — I don't know him personally and am not necessarily saying he's the one to see, but it is definitely true that not all neurusurgeons and neurologists are up to speed on the evaluation of this very difficult disorder.  The criteria for diagnosing a Chiari have been undergoing some serious revision in the past few years.  

I am in touch with many other patients with tethered cord (which I have).  Many have Chiaris that meet the diagnostic criteria by the old standards.  But a sizeable portion of us (this includes me) have very definite Chiari symptoms, yet with little or no tonsillar herniation on MRI.  Some have been diagnosed with what is called "Chiari 0", or "borderline Chiari", a controversial new category that Dr. Milhorat is at the center of defining.  Some of us may have a problem in which the brainstem is pulled down into the foramen magnum only in certain spinal positions (like sitting, or bending the head forward), which would not be seen on a supine MRI, but which could cause the same brainstem dysfunction from either transient compression or alterations in CSF flow.

Good luck in finding someone who can make sense of this for you.  I figure you've had MRI's of your whole spine to rule out cord tethering, since this can be a cause of both a Chiari and a syrinx.  I have many of the symptoms of  syrinx, and am waiting to get a cervical MRI this weekend to rule that out.

Best wishes,
Annika

[To CCF Neuro MD From Pokey]

Thanks for the info CCF Neuro, Justy and Annika. My MRI report was read as "not a classic configuration of a Chiari I malformation". So, I really don't know what to think of this yet. I did have a pretty bad car wreck in 1994, and didn't have any symptoms until 98. Guess there is a possiblity that this came from the car wreck. The differnetial diagnosis is "prominent central cord, but would be unusual". Another thought by the neuroradiologist was possibly an old lesion, but that again would be unusual. Never claimed to be normal! Follow up appt today and hopefully more information today. It would be interesting to see if MS and Chiari can co-exsist. One lesion, and clinical ON....very fustrating.... If this is truly a Chiari, I wonder after all these MRI's is it just now being mentioned....again, fustrating. Well again, thanks to everyone that has taken the time to read my post and respond.

Pokey

[justy]

Hi Pokey,
Just as Annika said the criteria has changed for Chiari 1 Malformation but the fact that they mentioned it at all in your report is reason for further investigation with a Chiari Expert. The average neurologist and even most neurosurgeons are not usually aware of the changing criteria and the very fact that you have a syrinx and the symtptoms you mentioned is suspect. I have a syrinx and have been told I do not have Chiari. I am seeing a well known Chiari expert in January to get his opinion. I will also be given a Cine MRI which is a special MRI that shows the flow of CSF to see if there is a blockage. I do know of many who were told they did not have Chiari and were given the Cine MRI which proved otherwise.

[To CCF Neuro MD From Pokey]

Hi Everyone,

Just to let you all know what my Doctor said. He says I do not have Chiari. He also said that the syrinx is not causing my symptoms since some of them are related to the brain, and cervial region. So were back onto the MS probablity....again. We did talk about treatment, but without the MRI confirmation of more than 1 spot, he didn't feel we should. I am going to follow up with my PCP and see how he feels. Justy, I feel the same way you do, the fact that it was even mentioned may be important. I'm starting to feel like an airplane in a holding pattern and it's running out of fuel. These past couple of days have been very trying to say the least. I have seen an MS specialist before, maybe I should try to see him again. Maybe he would be able to lead my PCP to the right connections to find out if this is a Chiari or not. One good thing though, atleast a Chiari is fixable!

Pokey

[justy]

Your welcome Pokey,
There have been many Chiarians who have previously been misdiagnosed with fibromyalgia, MS, and Chronic Fatigue. The ctiteria for which most neurologists and radiologists diagnose Chiari is a 3-5mm herniation of the cerebellar tonsils. Dr. Milhorat's 1999 clinical study "Chiari 1 Redefined" which can be read at www.wacma.com in the "on site info" states that this criteria may be too restrictive. You can also talk with other Chiarians many who have gone through what you are going through right now at http://groups.yahoo.com/group/chiari/ I'm sorry if I seem persistant but I know of many who have gone through this. Good Luck.........

[To CCF Neuro MD From Pokey]

Thanks again Justy. I do meet one of the criteria for Chiari and that is the 5mm measurement. But they also stated in the report that the cerebellar tonsils maintain a rounded configuration. When I know that my family Doctor has all the reports, the MRI the consultation with the neuro; then I will make an appointment with him to go over everything. I am going to check out that website. I also did read that article about diagnosing Chiari. Very interesting. I have an x-ray backround, so I do like to look at the pictures on the net, especially the cases that don't give you what it is. I came across info on what they term "Gibbs artifact" which can represent a syrinx on MRI, but since mine showed up in the sagittal plane and the axial plane it makes that unlikely. Anyways, I just have to wait until my Doc has the reports so he can make his decision. I think there will be another opinion in the future. I'll keep ya posted. I'm sure that I'll be posting again. This is the best website!

[Annika]

Dear Pokey,

Everything Justy has said in his past two posts is right on target with what I have learned about this problem.  I wrote a longer note to you yesterday, but it was lost when my computer crashed.  Thankfully, Justy said all the main things I wanted to point out.  

In particular, the issue of confusion with MS is critical.  Without saying that someone does or does not have MS, it is very common for the comparatively rare spinal disorders (such as syringomyelia, tethered cord and Chiari) to be misdiagnosed as MS.  First, misdiagnosis happens frequently before the spinal anomalies are found — there are cases reported of patients who went along for years with a standing diagnosis of MS until the right MRI was done of the right area of the spine.  Most of the time this seems to have happened because the treating doctor feels the symptoms are "so classic" for MS that they neglected the exhaustive searching through the entire nervous system that may be necessary to find these disorders.  Alternatively, they may not be familiar enough with these uncommon disorders to know that there's something else out there they should be looking for.  

What I find equally frustrating is the second category of misdiagnosis, the "double diagnosis."  This comes up all the time — I'm not saying this is you, but just reporting what comes up with a lot of patients.  Even when clear spinal cord abnormalities are found (like a syrinx), symptoms and neurological deficts often pop up that the doctor feels "couldn't possibly be related" to this abnormality.  They then start looking around for a separate neurological illness to explain the symptoms that don't fit with what they would expect.  I'm not saying this is always wrong — certainly, it is possible for a single unlucky person to get two independent nervous system disorders.  But I have heard this same story again and again, and what seems to be more common is just that the treating physician has misunderstood some key feature of the basic spinal disorder.  

One thing to possibly explore is that this could all be related to your car accident in 1994.  There's a documented mechanism by which this could theoretically happen, which could link both your borderline Chiari and your syrinx.  When there's spinal trauma, sometimes there's a contusion and bleeding around the cord, inside the spinal canal (into the CSF).  Bleeding inside the spinal canal can sometimes lead to the formation of adhesive scar tissue.  This "sticky" scarring can then cause the nerve roots and/or spinal cord to get permanently welded to the side of the dura, causing a tethering effect on the cord.  Normally, the spinal cord moves up and down inside the canal as you move your body during the day.  When the cord is stuck, though, it can't move normally - instead, a great deal of tension is generated.  This tethering effect on the cord has been shown to be associated long-term with both syrinx formation, and with pulling the brainstem and/or cerebellum too far down into the foramen magnum (one of the basic abnormalities of the Chiari).

If there's some sort of scar tissue having a tethering effect on your cord, this could also explain why you have symptoms in areas other than what would be predicted strictly from the location of the syrinx.  One of the difficult things is that this kind of scarring can sometimes be very hard to pick up on MRI.  In some cases it's very obvious, but in other cases patients undergoing operations for a syrinx were found to have adhesions or bands of scar tissue that could not be seen on MRI.  

Neurosurgeons who have a decent amount of experience with these problems can often help sort things out based on your history and symptoms.  The key, though, is that they really need to have direct personal experience seeing these patients, and be up-to-date on the literature.  If the Chiari teachings are being overhauled, the syrinx literature is going through even more basic changes, with some superb papers coming out in the past few years.  The most fundamental change in our understanding of syrinx formation is that many researchers now feel a syrinx is almost always a secondary effect of some other spinal abnormality (the biggies: Chiari, tethered cord, spinal tumor, adhesive scar tissue — also called "arachnoid adhesions.").

There's a clinical trial through NIH that aims to find out how to best treat a syrinx by addressing the underlying causes.  Here's the link to that study:

http://www.clinicaltrials.gov/ct/gui/show/NCT00011245;jsessionid=6351766B4711D80D74E7F4D4CD3FB183?order=1

I got this link through this website, so you may already have seen it.  For anyone else reading this, I don't know anything about the quality of that clinical trial, but I think their written summary of the new understanding of syrinx formation is very good.

Good luck,
Annika