Chirari 0 malformation and congenital cervical spinal stenosis any relation?

General
1

Hello:

My tonsils droop down only 4.36 mm instead of the 5 mm that is required by medical doctors to call it Chiari 1 malformation, I have been to so many neurologists and neurosurgeons (none of which are chiari specialists) none of them have felt that my symptoms are related to Chiari. I was looking through old medical records of my cervical MRI and saw that on the report it was mentioned that I have SEVERE Congenital cervical spinal stenosis, so my bones of my neck were formed larger than usual and are putting pressure on my spinal cord which affects the low back and legs from what I have read and also can be causing my bowel and bladder problems.

Does anyone think that this congenital deformity could be in association with the cerebellar tonsilar ptosis that doesn't meat the strictest criteria for chiari 1 malformation?

Also, recently I found out I have skin cancer so I will be having treatment on July 30th so this just adds to the myriad of problems.

The doctors have decided that my chiari isn't causing problems, but my stenosis of the spine and degeneration of the spine is and I have arthritis and degeneration of the spine and they have said my aches and pains in my body are fibromyalgia and I should see rheumatology.

First things first, Skin cancer treatment and repair, then I will tackle this puzzling problem again. This morning my cane barely got me out of bed I was in so much pain and weakness in the low back and legs. Soon I will graduate to a walker, what next a wheelchair? I will accept whatever happens...but it would be helpful if one doctor would determine what is really going on, if the Chiari is a problem or not; what is .64 mm (barely over 1/2 mm)?

Thanks for reading this.

2

Emmaline when you talk about hypermobile, what does that mean? Is that Essential tremor disorder which I also have which I am on medication for? or is it the leg jerks/jumps and restless leg at night which I also have. What specialist would look into EDS? My HMO insurance doesn't cover other doctors outside of their system and I have seem multiple docs that stick up with one another on diagnoses because they are in the same group. It seems I don't have a fair shot. My primary that I currently have told me that he would refer me to Mayo clinic if this next neurologist couldn't determine what was wrong, but because they diagnosed me with carpal tunnel syndrome he doesn't want to refer to mayo. I have only had this primary doc for one year because we moved a year ago, my new friend told me to see another doc for primary care so I am meeting a new doctor the middle of this month to see if she can direct me in other directions. It is like I am spinning my wheels, soon it will be wheel chair wheels if something isn't done soon.

We have no money to go to a Chiari specialist somewhere and because we have insurance that is an HMO mayo clinic wants $5,000 up front, our vehicles aren't even worth that much.

3

Hi DMJMMHH,

I wanted to respond to your questions. They are actually really complex. You are such a great person & have been through so much. I am sorry for your additional skin cancer dx. Do they know what type yet and have they scheduled have several friends and family members that has had skin cancers. They all have done well with heir individual treatments.

These are great links to information on EDS & Hypermobility. Please read them , then I suggest you discuss them with your Dr. If you think the symptoms may pertain to what you are experiencing.

You will get further with the following specialists with EDS and Hypermobility on diagnosis , treatment and continued care than a NL or NS:

Orthopedist, Dermatologist, OD, PCP, Geneticist, Physiologist or Rheumatologist.

There has been other Members that has raised the same question about the difficulties with physicians in the same practice not wanting to publically disagree with each other's diagnoses or treatment. I would recommend a totally different practice with a new dr. of any kind. If you are having those problems. I also know quite a bit about HMO's and can help you with referral or insurance questions.

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002439/

http://www.ncbi.nlm.nih.gov/books/NBK1279/

http://www.medicinenet.com/hypermobility_syndrome/article.htm

http://hypermobility.org/

Ben's Friends also has an excellent NEW EDS Discussion Forum:

http://www.ehlersdanlossyndromesupport.org/

Essential Tremor Disorder can be a symptom or related disorder of EDS . Tremors are symptoms of many different CNS Disorders & many Chiarians even have Tremors. I have occasional tremors. Is all this connected. Yes. That is where everything gets complicated. CNS Disorders have many of the same symptoms. Having a Low lying Cerebellum, Surgery, Brain Damage or other problems in the Cerebellum can cause so many Disorders and attribute to others. Some are genetic, some are not. I did massive research on the Cerebellum, Cognition, CM & Schizophrenia a week or so ago for another Discussion. That lead me to additional disorders and symptoms and the common factor was the Cerebellum and Brain Stem and their connection with other physiology in that immediate area. I still find myself processing all that information in my thoughts, because it leads to so many additional questions and light bulb moments. I plan on discussing it all with my brilliant medical contacts in different fields. That was how far reaching and thought provoking it was for me. I will message you some information I have saved. It's massive, but one article specifically was brilliant and connected many neurological disorders.

Just a question....Have you had your vitamin B12 Levels & Thyroid checked?

Info on EDS & Essential Tremor Relationship:

http://en.wikipedia.org/wiki/Essential_tremor

http://www.mayoclinic.com/health/essential-tremor/DS00367

http://symptoms.rightdiagnosis.com/cosymptoms/hand-tremors-similar-...

http://en.wikipedia.org/wiki/Ehlers-danlos - see signs & symptoms

Abby (Head Moderator on this forum) and Puggles are both Chiarians and have EDS and are Moderators on the EDS Forum. They are incredible ladies and can answer any questions you may have & very informative & supportive. I am knowledgeable about EDS but not nearly as much as these ladies, since I haven't been diagnosed as long as they have.

I worked for BC&BS and had an HMO when I became very ill. They approved and paid the total charges, except for one $400 copay, for me to go to another state for two surgeries involving multiple brain & CNS diagnoses, C Spine reconstruction, diagnostics, treatments, therapies, office visits , hospital stays, CSF Leak (from MVD surgery) and Chemical Meningitis treatment & hospitalization & all follow ups. The only thing they wouldn't cover was the acupuncturist the NS brought in the hospital for my severe motion sickness, because acupuncture is never a covered service, especially on HMO's or fully insured Group Policies. They didn't approve my care because I was an employee. They approved it because the specialist was the best for my collective issues and had vast experience in neurosurgical issues. He was the 52nd Specialist I consulted with and the 1st that could correctly diagnose & treat me.

Please never think navigating and working with HMO's are impossible. They key and a very valuable ally is your PCP when you have a HMO.

We can find you a good NS that participates or will be approved by your HMO. I know you are worried but it can be done. When you have stenosis and CM it makes it more difficult. Most NS only specializes on the brain or neck or back or specific disorders. There are a lot of Cranial Nerve Disorder specializing NS's & clinics popping up. My NS developed the Microvascular Decompression surgery and is the preeminent expert on Trigeminal Neuralgia. I was first diagnosed with Bilateral Geniculate Neuralgia. That lead to my CM & C Spine dx's & emergency surgeries and everything else.

I know this is a long message but I tried to explain every question you asked. I also hope you and your family are having a nice 4th of July !!!

Tracy Z.

PS: I will respond to your CM0 questions. We have a great article but our Discussion Search is not working and has been reported. I will ck. on another laptop and see if I have a link to it.

4

Positional cervical spinal cord compres - PubMed Mobile http://www.ncbi.nlm.nih.gov/m/pubmed/18499527/?i=3&from=/12048419/related

5

Agreed with Emmaline that you need a new doctor. I have been told that you can actually have chiari & NO herniation & have worse problems than people with herniation. The "new school" of thinking, is that there is no definate corralation between size of herniation & symptoms or need for surgery. I would be very curious to know if you have a syrinx. I am so sorry for you insurance situation. I am also having problems with that. What state do you live in? I have learned a lot from the great people here, & if there is not a chiari qualified doctor in your network, then you have to call insurance & insist they make provisions for one at same cost as an in-network one. They have to put a case worker on it. If they do not handle it fairly & quickly, you can start writing your congressperson, council person, & every other local representation for your area & insist they contact your insurance carrier, & they HAVE to!! In my state, there is also a department of insurance that will advocate for you. who knew! maybe your state also? We also have county medical clinics that have to accept you & are affiliated with good hospitals. You may want to start looking into all that. I know its downright maddening, but dont give up the fight!! If you reach out here, people have all sorts of great success stories of how they fought & won with insurance. That being said, I am very sorry about your condition, & I really hope you will find relief real soon! feel free to message me anytime! -Jessica

6

All of you have been so amazingly helpful and supportive and I am in the process of reading through the links you all posted and studying them. Last night I listened to Dr. Andrew J. Holman's you tube presentation, wow; seems to make much sense! I have sleep apnea and restless leg syndrome and even with a CPAP I find myself still extremely tired all day. I haven't been to an orthopedic surgeon, that is on my list of things to do, along with another rheumatologist. And I will be going through more information that you all have provided before I see my new possible primary doctor, I have heard great things about her from more than one person so I am hopeful. My cancer surgery on my nose will be done the end of this month so then I will begin to pursue once again to get to the right medical doctors/specialists.

I will keep everyone posted.

thank you very much!

Dawn

7

I also live in Wisconsin,
The Wisconsin Chiari Center is a part of the St.Mary’s hospital system . They are now St. Mary Dean Ventures, so they accept Dean Health Insurance primarilary, but do work with others. Dean is an HMO but as Tracy has said they have my greatest ally since my multiple dx’s.




Mandy said:

Hello,

This sounds like a nightmare, I am so sorry :frowning:

I see from your profile you’re in Wisconsin, have you checked with the Wisconsin Chiari Center yet (I haven’t been able to find what insurance they accept)? They are very highly rated. Finding a doctor familiar with Chiari can be difficult.

My previous rheumatologist (he just retired) had a very strong theory about fibromyalgia and spinal cord compression. Here is a YouTube video you can watch, and an article he wrote about why intermittent spinal cord compression can cause fibromyalgia symptoms.

http://www.youtube.com/watch?v=Ei8LbUDkQno

http://www.positionalcordcompression.com/images/PacificRheumAssoc_P…

And here’s the study he did on pramipexole (Mirapax). I was just prescribed pramipexole for RLS (restless leg syndrome). I have had no side effects other than a little more sleepiness. My pain is better except on the nights that I forget to wear my CPAP.

http://www.ncbi.nlm.nih.gov/pubmed/16052595

Dr. Heffez at the Wisconsin Chiari Center does the positional MRI (head forward, head normal, head back). They have shown that for some people who look normal in an MRI, when they put their head forward or back, they pinch the spinal cord. With the intermittent “pinching” or even just touching, it irritates the brain stem. This can cause the brain stem to be overactive, which means you don’t sleep well at night, and this can cause widespread pain.

With all that being said, for a portion of people with Chiari, their brain stem is going to be compressed by the tonsils being squished into the same space. So, from what Dr. Holman has said, the brain stem irritations don’t stop until the existing problem is corrected. If your spinal stenosis is pinching your brain stem, then that needs to be addressed. Have you already worked with an orthopedic surgeon? They should be able to tell you what they can do for you. I am not sure how your insurance works, but you should be able to ask your primary if they can refer you to an ortho surgeon to check on your neck.

Along with brain stem irritation or compression, tethered cord can cause bowel, bladder, and leg problems. A full-spine MRI can usually show if you have tethered cord. Tethered cord is where the bottom of your spinal cord attaches to a vertebrae or muscle, and the tension can cause damage which affects the lower half of your body. It can also “pull” the cerebellar tonsils, and can be the cause of the Chiari. Here’s an article from the Wisconsin Chiari Center: http://www.wichiaricenter.org/oth/Page.asp?PageID=OTH000008

As for EDS, that is Ehlers-Danlos Syndrome, or hypermobility. Your rheumatologist should be familiar with hypermobility. Hypermobility has a bunch of symptoms, but the main ones are extended flexibility of joints, scars that stretch wide after healing (thin wide scars), flat feet, bumps on the backs of arms and legs (kerastosis pilaris), excessive stretch marks, being able to touch tongue to nose, and the whites of the eyes (sclera) having a bluish color to them. Here is an article, it’s pretty technical, but the pictures are great. They show what some of the physical stuff looks like.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3512326/

Hypermobility and EDS are very common in Chiari, and in my opinion, most if not all patients with Chiari have some form of it. It can cause the neck bones to not stay in place very well, so it’s a good thing to know before you have surgery.

Please let me know if you have any questions, I know some of this stuff doesn’t make sense the first few times around, and it’s a ton of information.

I think it’s a good idea to see a rheumatologist, ortho surgeon, and a neurosurgeon, as long as your insurance covers that without too much out of pocket. The rheumatologist can check for hypermobility/EDS, fibromyalgia, and the status of your arthritis. The orthopedic surgeon should know about spinal stenosis, and the neurosurgeon about the Chiari.

Let us know how it goes :slight_smile:

Mandy

8

Hi Dawn,

I live in Madison! I’ve known about my Chiari since I was 18 years old (don’t really like to admit it but 29 years now! Lol!) I was told for so many years that it was nothing to worry about. Well it was! I had sub occipital decompression/dura pasty (with bovine patch/C1-2 laminectomy surgery in 2006 with great results. I lived in Montana at the time and knew no one else ever had any of these crazy symptoms! It wasn’t until I move here that I really educated myself about it because some of my symptoms returned. Not as bad, but I knew something wasn’t right. I’ve received so much better care here it’s like night and day. I’ve since learned that I also have EDS, POTS and many other problems also. I also suffer from hemiplegic migraines, which aren’t related to any of my other dx’s but we are managing everything now. It just makes so much more sense now! Now my PCP is not an expert in any of my conditions but he sends me to the correct specialists that are needed, and we are both learning as we go! He always tells me that I’ve taught him more then he ever learned in med school! My rheumatologist was too afraid to even work with me so I was sent to a geneticist for my dx of EDS and some ideas for my plan of care for my PCP. It’s really hardto find a PCP that is willing to listen and learn about you because no one knows better about how you are feeling, it’s his job to know or at the very leas listen to you and learn or send you to the correct doctor to help you.

I also have an HMO, first time ever in my life. I always heard horror stories about them, but have found that they have helped me out greatly!

If you have any questions don’t hesitate to ask, or even pm me or send me a message. I’m usually here (still recovering from 2 knee surgeries since March 6, gotta’ love EDS! Lol!).

Barb

9

Hi Barb:

so did I understand you right that the Chiari Center is now a Dean HMO system? I have Dean HMO...I wonder if I need a referral to go there...I will have to call my insurance plan. First things first though, I must get this skin cancer taken care of on July 30th and if it looks like I need plastic surgery that will occur in August and then if all is well; I begin again on this journey of finding answers. Did you know that there was a support meeting for Chiari held in Middleton (near Madison) this past spring? I think they were hoping to plan one again for this summer, but haven't heard anything yet. It was very helpful!

Dawn


Puggles said:

Hi Dawn,
I live in Madison! I've known about my Chiari since I was 18 years old (don't really like to admit it but 29 years now! Lol!) I was told for so many years that it was nothing to worry about. Well it was! I had sub occipital decompression/dura pasty (with bovine patch/C1-2 laminectomy surgery in 2006 with great results. I lived in Montana at the time and knew no one else ever had any of these crazy symptoms! It wasn't until I move here that I really educated myself about it because some of my symptoms returned. Not as bad, but I knew something wasn't right. I've received so much better care here it's like night and day. I've since learned that I also have EDS, POTS and many other problems also. I also suffer from hemiplegic migraines, which aren't related to any of my other dx's but we are managing everything now. It just makes so much more sense now! Now my PCP is not an expert in any of my conditions but he sends me to the correct specialists that are needed, and we are both learning as we go! He always tells me that I've taught him more then he ever learned in med school! My rheumatologist was too afraid to even work with me so I was sent to a geneticist for my dx of EDS and some ideas for my plan of care for my PCP. It's really hardto find a PCP that is willing to listen and learn about you because no one knows better about how you are feeling, it's his job to know or at the very leas listen to you and learn or send you to the correct doctor to help you.
I also have an HMO, first time ever in my life. I always heard horror stories about them, but have found that they have helped me out greatly!
If you have any questions don't hesitate to ask, or even pm me or send me a message. I'm usually here (still recovering from 2 knee surgeries since March 6, gotta' love EDS! Lol!).
Barb
10

Mandy:

Dr. Holman sounds great!!!!!! I am very impressed and I hope that his retirement will lead him to many discoveries through his research; I think he is onto something very very big and helpful for many people. Let's hope!

Mandy said:

Hi Dawn, isn't Dr. Holman great?? I am terribly sad he has retired, he's wanting to focus on research was my impression.

Best of luck on the new PCP, I am in the search for a good one as well.

Keeping you in my thoughts for the upcoming cancer surgery.

Take care,

Mandy

11

Yes the Wisconsin Chiari center is part of the Dean system. You will be covered. If you go to their site there is a questionaire to print out and a list of things that they request (copies of MRI, ct’c and any tests you may have had, and a photo copy of the front and back of your insurance card). You fill it out and send the diskss of the tests. Then every Monday they have a meeting to review the cases. You do not need a referral to send your info, and it costs nothing for them to review them. Then they will call you back. There is also a list of phone numbers so you can contact them, and email them with any questions.
I hope all goes well with your appointment on the 30th. Praying for good results.

12

Hi Dawn,

I just wanted to see how you are doing?

Tracy

DMJMMHH said:

Hi Barb:

so did I understand you right that the Chiari Center is now a Dean HMO system? I have Dean HMO...I wonder if I need a referral to go there...I will have to call my insurance plan. First things first though, I must get this skin cancer taken care of on July 30th and if it looks like I need plastic surgery that will occur in August and then if all is well; I begin again on this journey of finding answers. Did you know that there was a support meeting for Chiari held in Middleton (near Madison) this past spring? I think they were hoping to plan one again for this summer, but haven't heard anything yet. It was very helpful!

Dawn


Puggles said:

Hi Dawn,
I live in Madison! I've known about my Chiari since I was 18 years old (don't really like to admit it but 29 years now! Lol!) I was told for so many years that it was nothing to worry about. Well it was! I had sub occipital decompression/dura pasty (with bovine patch/C1-2 laminectomy surgery in 2006 with great results. I lived in Montana at the time and knew no one else ever had any of these crazy symptoms! It wasn't until I move here that I really educated myself about it because some of my symptoms returned. Not as bad, but I knew something wasn't right. I've received so much better care here it's like night and day. I've since learned that I also have EDS, POTS and many other problems also. I also suffer from hemiplegic migraines, which aren't related to any of my other dx's but we are managing everything now. It just makes so much more sense now! Now my PCP is not an expert in any of my conditions but he sends me to the correct specialists that are needed, and we are both learning as we go! He always tells me that I've taught him more then he ever learned in med school! My rheumatologist was too afraid to even work with me so I was sent to a geneticist for my dx of EDS and some ideas for my plan of care for my PCP. It's really hardto find a PCP that is willing to listen and learn about you because no one knows better about how you are feeling, it's his job to know or at the very leas listen to you and learn or send you to the correct doctor to help you.
I also have an HMO, first time ever in my life. I always heard horror stories about them, but have found that they have helped me out greatly!
If you have any questions don't hesitate to ask, or even pm me or send me a message. I'm usually here (still recovering from 2 knee surgeries since March 6, gotta' love EDS! Lol!).
Barb