POTS and Chiari I malformation

[lthomas521]

Prilipko O, Dehdashti AR, Zaim S, Seeck M. Orthostatic intolerance and syncope associated with Chiari type I malformation. J Neurol Neurosurg Psychiatry. 2005 Jul;76(7):1034-6.

http://jnnp.bmjjournals.com/cgi/content/full/76/7/1034

Presurgical Epilepsy Evaluation Unit, Program of Functional Neurology and Neurosurgery, University of Lausanne, Switzerland.

The Chiari type I malformation (CM1) is characterized by herniation of cerebellar tonsils to at least 3-5 mm below the plane of foramen magnum and can present with a wide variety of clinical symptoms, frequently including occipital headaches, secondary to bulbar and/or medullary distress. Rarely, syncopal episodes have also been described and attributed to either compression of the midbrain ascending reticular system, or vascular compromise (vertebrobasilar artery compression, hypotension). We report the first case of a CM1 patient with frequently recurring syncope due to postural orthostatic tachycardia syndrome (POTS), a form of orthostatic intolerance, whose symptoms resolved completely after surgical intervention. It is important to stress that it is not clear whether the described association of POTS and CM1 in our patient is a fortuitous finding in an isolated case or a reflection of a more systematic association between the two pathologies.

[Michelle Sawicki]

I have not read this entire article yet, but did previously read the abstract. I advise caution in this area. I have heard from several patients who had miraculous recoveries or great improvement after chiari surgery only to get ill again in the following weeks, months or years. There are some, like in the chiari story posted on our website, who do get better. I believe there is a connection between chiari and dysautonomia, but I don't believe sugery is an automatic "fix" for everyone.

Michelle

[Guest Julia59]

Chiari----just like POTS-----is a chronic condition. Surgery should only be a last resort. I have read many many stories of patients who have had surgery and had improvement for a few months or even a couple of years only to have a return of POTS symptoms---and other CNS symptoms.

However, I have heard of some who have improved and stayed that way.

Sometimes the ANS damage is not reversable and it will still be there after surgery, but the surgery may stop the progression of ANS dysfunction. And of course we know that dysautonomia has other causes that are still unknown.

Chiari with minimal or no herniation can cause a person to have symptoms also, brain stem compression is brain stem compression regardless of herniation into the (foramen magnum)----or opening of the skull. I know this as I have minimal herniation, and a small posterior fossa-(lower skull). I also have a malformation of the odontoid bone causing instability in the cervical/cranial junction---and added pressure to my brain stem along with the pressure already there from the chiari.

A lot of people with chiari malformation will have some form of ANS dysfunction or another. From my experience with meeting people both on line and in person----their symptoms seem to differ from "typical" POTS. There are usually other complications with motor function ect. , and the syncope is more related to exertion and aggravation of the upper spine and brain stem area from the force of gravity---usually taking longer for the syncope, if it indeed happens. It takes quite a lot to get me there-----(near syncope), but it has happened many times.

If POTS is from Chiari----it's just another sub-type, but not necessarily cured when one has surgery. The chiari in itself is chronic meaning that some patients who have this, may have repeated surgeries, especially those who have had surgery from an inexperienced surgeon, or who have complications from EDS---( Ehler Danlos Syndrome)---a collagen disorder where the ligaments and joints are too stretchy and unstable.

I would advise studying the experiences of many patients who have had surgery for chiari---and who have also had ANS dysfunction with their chiari. I have heard good things and bad. Another thing to pay attention to is OTHER symptoms outside of typical POTS symptoms like (difficult swallowing), (drop attacks without passing out)----those are usually warnings that the hind brain compression is starting to become severe, or even life threatening. This is a time to think about seeing a very good experienced neurosurgeon in the area of chiari--or chiari related disorders.

One woman I know had surgery, but waited too long, and her swallowing difficulties are permanent. She was already having drop attacks with both syncope and non-syncope. However, I don't hear of this type of thing that often.

There are a lot of gray area's with chiari & ANS dysfunction regarding the necessity for surgery. If my "POTS" symptoms stayed the same I would not have surgery. But now I have other symptoms that might be trying to warn me that more is going on besides my "POTS". Still, I remain cautious.

Please keep in mind that I am a laymen in the medical world, and this is strictly views from my own experiences, and from researching patient experiences.

Julie :0)

[DancingLight]

julie,

how timely this discussion is, huh? this whole things scares the bejeebers out of me...i gotta stay in the moment, breathe, emily, breathe, and hope i don't get to the surgery point!

thanks for shedding so much light on this julie...your post was well put and very helpful. you have clearly read all sides of the story. thank you for using your precious energy to write that all out...i know that writing a post like that can wipe me out!

speaking of being wiped out...time to try and nap...

emily

[MightyMouse]

Ditto the replies about long term outcomes for Chiari docompression surgeries. I have several friends who've struggled terribly, inlcuding one who has had more than a half dozen surgical procedures after the first decompression, including at least 3 new plates and/or screws, removal of scar tissue, reshaping of plates, repair of dura, etc; each one has given brief relief, but in the end still has terrible symptoms on a daily basis, inlcuding vision problems, severe headaches with vomitting, etc. Short term, meds have helped, long term, she's ended up getting numerous spinal punctures to bring down her intracranial pressure. Chiari decompression surgery is still a very imprecise art and from many accounts, has a very long way to go before giving a reliable outcome.

Just a word to the wise to do a great deal of leg work investigating about Chiari BEFORE allowing anyone to chop out a piece of your skull... like other procedures, (i.e. ablation), it's not reversible.

Nina

[DSM3KIDZ]

Just a question because I know nothing about Chiari, how is it dx? Is it just based on symptoms or through MRI testing or something different?

Dayna

absorbing as much info as possible

[MightyMouse]

Dayna, it's diagnosed via any of several types of imaging, inlcuding MRI. To read up on it:

http://www.ninds.nih.gov/disorders/chiari/chiari.htm

http://www.pressenter.com/~wacma/info.htm

[Guest Julia59]

Most of the time an MRI is needed because it is a difficult diagnosis. C-scans with contrast----and a special CINE MRI are often used. Here is a link---with some information regarding chiari malformation.

http://www.nfra.net/ChiarMal.htm

Julie :0)

[Ernie]

Hi Julia,

Thank you very much for your interesting explanation.

Ernie

[DancingLight]

nina, julie or anyone else...

what's the difference b/w having the chiari surgery and the decompression surgery for cervical spinal stenosis? are they different in risks, etc? just wondering...

thanks...you can guide me to a website instead of typing!

emily

[MightyMouse]

Em, yes, there are different risks. Spinal decompression doesn't usually involve puncturing the dura, or contact with brain matter. Also, the lower back portion of the brain carries the signals for most automatic functions of the body--breathing, swallowing, etc. Damage to this area can be catastrophic.

Chiair decompression involves opening the back of the skull to leave more space for the brain to sit w/o blocking fluid flow (cerebrospinal fluid). Any mess ups with this surgery can lead to stroke or death.

Spinal decompression risks aren't exactly minor either, but there is more of a likelihood of paralysis, parathesias, or other nerve conduction problems, as opposed to death (my understanding is that c-spine surgery carries typical risk from general anesthesia.). In this case they basically clean out any bone matter that's not supposed to be there, but they don't remove the basic spinal structures, only widen the spinal canal and or nerve root canals. The dura can be punctured accidentally, and nerves can be damaged accidentally, even with neurological monitoring.

Lastly, if you've a history or suspected dx of EDS, surgery should be your absolute last resort. Bone healing and EDS can be problematic (I, being a prime example).

Nina

[DancingLight]

nina,

thank you so much.

surgery WILL be a last resort. i am just so freaked out about it even being a possiblity. i know how hard a time you have had and julie too. eeeekkkk.

we are doing everything and i mean everything in our power to avoid surgery...i'm in good hands as you know!

emily

[Guest Julia59]

Ugg,

Emily--Nina,

AS I sit hear and read this i'm in so much pain and my lower part of my face is twiching. I have had problems lately---swallowing, trouble breathing and virtually no bowel motility---all the things that point to further brain stem compression.

It is literally hard to hold my head up---and my ears hurt so bad.

I can't move anything on my upper body without feeling one area or another popping out---or something moving to a place where it shouldn't be.. UGGGG--I don't really know how to explain this. I have been staying at home---as I find it too taxing to go out anymore. I avoid stress as much as possible. I avoid people who don't support me---where before I could deal with that----now I just stay away from them. Which means some parts of my family are avoided and in turn I can't see the ones who are supportive.

My niece came over tonight and I gave her a bunch of clothes I used to wear when I worked as an Office mgr.. She is going for interviews and needed the proper business attire. I was glad to help, but sad to see I won't be missing them at the same time. I'm also a size or two bigger because of the inability to exercise, and the lovely weight gain from the Wellbutrin. On a happier note, my niece went from a size 18-20 to a 10-12----I'm so proud of her. She had troubles with weight since puberty and now she is so happy----and her once skinny aunt is now actually a little bigger then she is. You know---i'm so happy she is the one i'm bigger then now---it didn't bother me in the slightest---just made me happy for her. I was lucky all those years to be thin---it's her turn now.

I gave her some really nice Gap Khakis I can still wear, but they looked so good on her and they fit her better. I only wore them twice. I'm too depressed to care if I dress up to go out anyway. Really, I do count my blessings, but now it's a tough time, and the reality is that I am officially depressed. The good thing is that i'm getting the help I need. I can't tackle this on my own anymore----I don't just snap out of it anymore.

I don't know what to do, I wish these symptoms weren't progressing, but they are, and it's scarey. Still, i'm checking every avenue before I ever even consider surgery. I know surgery for my problems won't be pretty----as we could be talking decompression from the brain stem down my entire cervical spine. I am told I have stenosis in my whole cervical spine as well as the other issues near the brain stem. I guess it depends on what they would see when going in there, as I would imagine they would try to be as conservative as possible. I just know what is seen on the MRI's---could be worse---or maybe not as bad. Note this is all what-if's ------things I prepare myself for if it comes to surgery.

Chiari's and cervical spine issues are tricky. And the surgery for these area's are very difficult. It's not as simple as going in and taking a tumor out. I really have to commend the neurosurgeons who venture into this area. The mal-practice insurance alone dictates where they live.

My main fear is cranial settling---and the instability. I pray this is not the case ---and that it just feels that way. Maybe Dr. Heffez will say it's not that bad----and conservitive measure can be used for now. The only thing to do for that is to fuse the neck to the cranium. Since I have had this virus that never really fully went away---I noticed when I was coughing a lot---if I turned my head to the left---I had to turn it back quickly as it felt like it was cutting off my air.

This is just all too much for me. Sorry----rambling get's it off my chest.

You guys are great.

As you can see---all this chiari--and chiari related stuff can really mess with your mind.

Julie :0)