I live in West Tennessee and I just went to a neurologist for the second time. The first time was four weeks ago and he told me that my MRI had shown the cerebullar tonsils to be 5mm. I had a lumbar puncture done and today was my follow up appointment. Well today he tells me I just have bad headaches. He gave me Topamax and said come back in a month. I have so many of the symptoms described here for the Chiari Malfomation- sudden intense headaches that start at the back of my neck and go up to my head. Just slight movements in my head triggers severe headaches that I've coined "brain pains" because I can't think of anything else to describe them. I have extreme fatigue and my head seems heavy all the time. I have that swallowing issue and many others. So- my question is what do I do now- my neurologist is saying I am "JUST having bad headaches" He spent maybe 5 minutes with me today! I feel this is something serious that he is not taking seriously! Does this sound like Chiari Malformation to any of you? Any suggestions I would greatly appreciate!
What is a Chiari Malformation?
Chiari Malformation Type I (CM) is a neurological disorder where part of the brain, the cerebellum (or more specifically the cerebellar tonsils), descends out of the skull into the spinal area. This results in compression of parts of the brain and spinal cord, and disrupts the normal flow of cerebrospinal fluid (a clear fluid which bathes the brain and spinal cord).
Does the size of the malformation matter?
Traditionally, Chiari Malformation has been defined as the cerebellar tonsils descending more than 3-5mm out of the skull. However, research has shown there is no real correlation between the amount of descent (or herniation) and clinical symptoms. Some people with herniations of less than 3mm are extremely symptomatic and some people with quite large herniations are symptom free. Because of this, doctors are now focusing on whether the cerebellar tonsils block the normal flow of cerebrospinal fluid (CSF). The current theory is that disruption of CSF flow is a more important measure than the size of the herniation.
How is Chiari treated?
If the symptoms aren't severe, doctors may recommend just monitoring the situation with regular MRI's and treating the symptoms individually. However, if symptoms are interfering with quality of life, are getting worse, or if the nervous system is being impaired, doctors may recommend surgery. The most common surgical treatment, performed by a neurosurgeon, is known as decompression surgery. An alternative surgery involves placing a shunt (a tube like device) to channel the flow of CSF and relieve pressure.
You do have a Chiari malformation it is a small one. Generally surgery is not needed unless it is around 8mm. But how small doesn't matter if you are exhibiting symptoms and if your symptoms progress.
I have a great neurologist and neurosurgeon so I will give you advice based on what they have done for me.
My CM was found in March, mine is 7mm. The MRI was of my head and c-spine (neck) with and without contrast (they inject you with dye). The MRI showed the CM plus a syrinx between my 6th and 8th vertabret. Upon diagnosis another MRI was ordered for the rest of my back to be sure no other syrinx's were present. After the diagnosis my nuerologist and I consulted with a neurosurgeon who decided decompression surgery was not needed at this time, but he ordered a follow up MRI to be done in July (4 months after 1st one). Once I had the follow-up MRI my neurosurgeon saw no growth in my Chiari, a better flow of fluid around the tonsils, and a small shrinkage in my syrinx (the improvements are attributed to a new pillow I'd been using - tempurpedic contoured memory foam pillow). My next follow-up MRI is going to be in 6 months, if there is no change it will be in a year and then after that I may need one every three to five years, unless my symptoms progress.
Side Note: A syrinx is like a cavity or hole in your spine which develops from the pressure of your overgrown brain tonsils pushing down on your spine. This syrinx fills up with CSF (cerebral spinal fluid) and can cause a lot of problems on its own. Now here's the tricky part. You can get more than one syrinx and you can get them anywhere in your spine so you should request an MRI of your whole spine. The syrinx can be caused by chiari, injury, or even just birth. They are not always dangerous, it just depends on where it is, how big it is, and whether or not it is growing.
So basically, I'm saying find a neurologist who will recognize this as a substantial ailment, and watch it closely. Have follow-up MRI's and check your entire spine for syrinx's. If your insurance will pay for it what should your doctor care if you have 100 MRI's? Also, have a consultation with either a Chiari specialist or a neurosurgeon to determine if surgery is necessary (It shouldn't be, but that really does depend on your symptoms and their severity). It's your health, be proactive about it. If you don't like your doc find a new one, you have options.
About the symptoms and surgery: I do have symptoms but nothing I can't live with, and at this point my chiari and syrinx are not life threatening, and they are not growing so if I can live with my symptoms, it is better than having a very dramatic brain and spinal surgery. It's almost like making the decision to have chemo therapy for a mole that could one day become melanoma. You don't have a life threatening disease yet so why have a potentially life altering surgery. Live with the symptoms if you can, but document them so you can see a progression and be sure if surgery is right for you. As for the terrible pressure head pains, the pillow really made all the difference for me. It's expensive, but if your chiari is small and you are going to live with the headaches (which surgery doesn't always cure anyway) then get a good pillow and/or mattress - they also have chiari pillows on www.medpillows.com
Thank you so much for such wonderful advice! I am already searching for a new neurologist. I just want to find someone that takes it seriously. I am like you- my symptoms are annoying, but I can live with them right now. I have read many of the stories online of people with this, and I feel very blessed that mine is not as severe. I think it would validate everything that I am feeling just to find a neurologist that would say- yes this is what you have, it is a mild case but it is Chiari! So just a good neurologist can give that diagnosis- right? It wouldn't have to be from a specialist would it? Because I live in rural West Tennessee, and I think the closest specialist is in Chattanooga. Again- thanks for the response!
Well- from the research I've done- I agree with you! I have lost some peripheral vision in my left eye. My eye doctor is the one that got the ball rolling for me to see a neurologist. Has anyone ever heard of your optic nerve swelling with this? Because my eye doctor detected that also. From what I gather through the research I've done- once you become symptomatic there is some damage being done- right? Thank you for your time and advice. The first neurologist seemed clueless- so I am in the process of finding another one!
Thank you so much for your recommendation! It is so hard to find a compitent and good neurologist- especially in rural West Tennessee! I wrote your doctors name down and I am going to check on my insurance to see if he is covered! Thank you again so much!
Yes, raised intracranial pressure (which is caused by the disruption of csf flow) can put pressure on your optic nerves which can cause them to swell. This is known as papilledema. It doesn't always happen, but it can.
Once a patient begins to have symptoms, he/she should be evaluated by a Chiari specialist to evaluate whether or not damage has been (or could be) done.
Thank you so much for your comments. It has been so helpful! It amazes me how much I am learning from you guys- My eye doctor orginally thought that I had pseudotumor cerebri because of the swelling of the optic nerve and the loss of perphial vision, but when I had the lumbar puncture my pressure was normal which is completely opposite of what my eye doctor expected. Now it is beginning to make sense that both those can be related to Chiari- Thanks again Rebecca for all the comments. I really appreciate it!
At first, I also thought that my daughter had pseudo tumor cerebri. And now I am thankful that she was not diagnosed with this. If she had been, we may not have pursued having her evaluated for Chiari, we might still be trying to find out what is wrong with her.
We were thinking maybe I had both- and that was really upseting me! I am just so glad I took a proactive part on behalf of my health. Maybe now I can take all of this info in to my new neurologist- whoever it might be! :) I am so thankful that I have realized all these symptoms are associated with Chiari. Thanks again! I appreciate it!
God Bless you too,
I looked at my insurance about the doctor in Nashville, Dr. Carl Hampf and he is on my insurance. I was really excited that he was on it. My eye doctor is going to call tomorrow and try to get me in to see him. So- he is knowledgable about Chiari? Will he do the surgery or would he refer me to something else. Thank you for recommending him- maybe I can jump right to a good doctor and not have to spend years searching for one. So- can you give me any info on what to expect? I was just making sure he is a good one since my eye doctor is calling tomorrow.
Thanks so much,
About your eyes, you may want to look into an opthonerologist to help you with your problems. Mine was able to tell me very quickly and easily which symptoms were chiari related and which were not. Good luck to you!
About the argument of size of herniation, symptoms, and surgery - here is some info I found online that may help you. This supports what was said previously, the biggest indication for need of surgery depends on symptoms, not size. I think what dana above means by surgery is usually not needed until about 8mm, is that STATISTICALLY most chiari malformations do not present symptoms until about 8mm. That is not always the case, but you can either be given only the scary information that makes people want to run and have unnecessary surgery or you can be given useful information based on surveys, scientific studies, and normal human behavior and development.
Treatment of Chiari malformations and syringomyelia is very dependent on the exact type of malformation, as well as progression in anatomy changes or symptoms.
Chiari I malformations that are asymptomatic should be left alone. There is no indication for "prophylactic" surgery on these. If the malformation is defined as symptomatic, or is causing a syrinx, treatment is usually recommended.
Chiari II malformations are treated if the patient is symptomatic, and physicians have determined that there are no complications from hydrocephalus. In some patients, consideration of a tethered cord is also explored. In many infants who become symptomatic from a Chiari II malformation, the symptom onset and progression are severe and rapid, and this requires an urgent or emergency approach.
Surgical treatment of these malformations depends on the type of malformation. The goal of surgery is to relieve the symptoms, or stop the progression of the syrinx or symptoms.
Chiari I malformations may be treated surgically with only local decompression of the overlying bones, decompression of the bones and release of the dura (a thick membrane covering the brain and spinal cord), or decompression of the bone and dura and some degree of cerebellar tissue resection.
Decompression is performed under general anesthesia. It consists of removing the back of the foramen magnum and often the back of the first few vertebrae to the point where the cerebellar tonsils end. This provides more space for the brainstem, spinal cord, and descended cerebellar components. A tissue graft is often spliced into this opening to provide even more room for the unimpeded passage of CSF. Occasionally, the cavity within the spinal cord resulting from hydromyelia can be drained with a diverting shunt tube. This tube can divert the fluid from inside the spinal cord to outside the cord, or be directed to either the chest or abdominal cavity. These procedures can be done together or separately.
Chiari II decompression is treated similarly, but is usually restricted to decompressing the tissues in the spinal canal and leaving the back of the skull alone.
The goal of Chiari surgery is:
Optimal decompression of nerve tissue
Reconstruction of normal CSF flow around and behind the cerebellum
The benefits of surgery should always be weighed carefully against its risks. Although some patients experience a reduction in their symptoms, there is no guarantee that surgery will help every individual. Nerve damage that has already occurred usually cannot be reversed. Some surgical patients need repeat surgeries, while others may not achieve symptom relief.
My advice to you is - don't listen to every crazy person out there who says "you have chiari you need surgery" because that isn't always the case. This isn't a death sentence for you, and your herniation IS small, which simply means - Statistacally you are less likely to have life altering symptoms and need surgery than someone with a larger herniation. But this is definitly more serious than as your doc said "severe headaches" so research, educate yourself, and don't make decisions based on panic or fear, make decisions about your treatment based on your specific case. Every chiari case is unique to each person.
I appreciate your sound advice! I have done alot of research, and I think I know more about it than my last neurologist did. I am taking all advice in a very calm fashion! It is obvious to me that all cases are different just from what I have researched. Just to give a little more background on mine- my symptoms started last September- I was mainly having just the headaches. My PCD ordered an MRI and nothing was detected. (sinus infection- I think) Then this April- my symptoms started worsening- it wasn't just the headaches anymore. My PCD ordered another MRI and that was when the 5mm hernitation was detected and that got me sent to the neurologist. Since then- my symptoms have intensified- all day my head hurts, slight movement gives me that extreme brain pain radiating from my neck to behind my eyes, extreme fatigue, dizziness, difficulty finding words, loss balance, lossing my hair, heavy head, difficulty swallowing, etc, etc..... Right now- by 4:30 or 5:00 every night I am in tears! I take a pain pill every night that helps a little- So- my symptoms in the last six months have gotten worse. I am still functioning. My fear is that before I find a knowledgable doctor- I might really be in bad shape! Hope not- but that is the fear! Thanks for your insights! If anyone has any new insights after hearing my symptoms- I would appreciate it!
Yes, Doctor Hampf is knowledgable about Chiari. He performed my surgery April 23rd and I'm doing pretty well.
You're symptoms sound similar to mine. I also started having problems last Autumn. I had headaches, dizziness/loss of balance, pain in my back and shoulders, vision problems, etc. I'm a lot better now than I was. I'm still having some dizzy issues, but hopefully that will get better with time.
As far as what to expect; that's kind of hard to say, people react differently. But for me; the first couple of nights were bad. I don't mean to scare you, but we are talking about them cutting into your back muscles, head and skull. It hurts for a while. It gets progressively better, but even the first month was pretty bad, mainly because I ended up catching a bug of some sort and the second week I was vomiting quite a bit and that hurts the head a lot! Hopefully that wouldn't happen with you.
I came home after four days and was able to walk unassisted up a flight of stairs. My wife spent the next two days off work with me, then my sister hung out for a couple of days after that to just help out during the middle of the day. I would probably have been able to more or less watch over myself after the first week, but I got sick, so that was a little of a setback, but by the third week, I was feeling a lot better. Sure, I was still weak and drained of energy, but every week I could see an improvement - on a day to day basis I didn't really notice it, but each Saturday I'd take stock and compare how I was the last Saturday.
I kept a blog of my experiance - not a lot, but it should give you a basic idea of what I went through.
Thank you so much for telling me that Doctor Hampf is good- also thank you for telling me your symptoms are similar to mine! That gives me hope that when I go see him I will be taken seriously! Do you mind me asking what your herniation was? Because from what I have read the smaller it is- the harder it is to be taken seriously! Mine is only 5mm, but my symptoms are making me miserable! I don't want to be brushed aside just because my herniation is considered a small one.
Thanks so much!
Well, generally I do believe that the larger it is the more concerned the medical staff gets, but I've also heard of people having surgeries at even 1 or 2mm, maybe even smaller.
I really don't remember what mine was, I'm wanting to think it was 9mm. When they noticed it, everything happened so fast that I was. I was told how big it was and actually looked at my MRI and saw it clearly and I have the size written down somewhere, but I can't find my paperwork right now. If I can find it, I'll let you know.
But I wouldn't worry, he'll take you seriously.
Have you been to see him or scheduled an appointment yet?
Well- my eye doctor tried to call Friday and set up an appointment. But, they said that he wouldn't even schedule an appointment until he has reviewed all of my information. So- we are working to get all of my things sent to them so Dr. Hampf can review it all. My fear- he will review my information and not even allow me to have an appointment with him. I just hate to think my appointment with him is conditional! Since my herniation is small- I worry that I won't get the chance to see him! Any suggestions?
I really don't have any advice to give on how to get in to see him. I did mention to his nurse the other day that there was someone on a forum I visited wanting to see him and she suggested to just make sure you get all your information from any and all of your doctors sent to him. I put in a good word for you :) It's kinda fuuny, yet sad at the same time, that we have to approach seeing a doctor to help us feel/get better in the same way we might try and get a good job.
I asked her to check the charts and see how much mine was herniated. Turns out mine was 20 mm! I guess that kind of explains why mine was so done so quickly. I wonder where I got eh idea it was 9mm? Who knows. I think I would have remembered someone saying 20 mm before.
Good luck, I hope everything works out for you. Keep us in the loop.
Thanks so much for putting in that good word for me! :) Any little bit helps! Wow- your herniation was huge! Yeah- no wonder they moved so fast on yours! I really appreciate all of your input and words of advice! I will keep everyone posted! Thanks again!
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