Arnold Chiari Malformation

Side by side comparison of normal brain placement and Chiari brain placement
Side by side comparison of normal brain placement and Chiari brain placement | Source

Arnold-Chiari Malformation


First I will give a clinical overview of Arnold-Chiari Malformation, then I will add my own personal experience with Chiari and describe the disease in user-friendly terms.


Wonderful Video Explaining Chiari

The Clinical Explanation

Arnold Chiari malformation was first described in the 1890’s by Hans Chiari and later officially defined by Hans Chiari’s colleague Dr. Julius Arnold, thus the name. Arnold-Chiari Malformation, also simply called Chiari, is a disease of the hindbrain and is characterized by a downward placement of the cerebellar tonsils through the foramen magnum. The crowded space at the base of the skull may hinder the Cerebro Spinal fluid (CSF) flow causing several different symptoms that could change over time. Symptoms will depend on the stage of Chiari an individual has. There are 4 different severities of the disease and they are labeled I – IV, with Arnold-Chiari Malformation IV being the most severe and often fatal form.

Type I is a congenital malformation, meaning it is present as birth. But it does not normally present symptoms until later in life as the brain grows and the base of the skull does not. Type I is the most common form of Chiari and manifests with headaches, cerebellar symptoms and herniation of cerebellar tonsils. (Note: Some Chiari I can be acquired through severe head trauma or due to hereditary disorders of connective tissue.)

Type II is cerebellar herniation accompanied by a lumbar myelomeningocele, an unfused portion of the spinal column that allows the spinal cord to protrude through an opening. With Type II the cerebella herniation is typically more severe.

Type III is more rare than Types I and II and it causes severe neurological defects and is associated with an occipital encephalocele, a sac-like protrusion of the brain and its membranes through an opening in the back of the skull.

Type IV is the rarest form of Arnold-Chiari malformation and is characterized by a lack of cerebellar development.

The most complained about symptom of Chiari is a headache.
The most complained about symptom of Chiari is a headache. | Source

Symptoms of Chiari

Symptoms of Chiari are typically neurological in nature and can include:

Headache – especially after straining such as laughing really hard, sneezing or coughing

Tinnitus – ringing of the ears

Dizziness and vertigo

Nausea

Nystagmus – irregular eye movements

Facial Pain

Muscle weakness

Impaired gag reflex

Restless Leg Syndrome (RLS)

Sleep Apnea

Dysphagia – difficulty swallowing

Poor coordination

Intercranial pressure

Pupillary dilation

Dysautonomia – rapid heart rate, fainting, extreme thirst, chronic fatigue

If the blockage of Cerebro-Spinal Fluid (CSF) flow causes a syrinx to form the patient may develop syringomyelia, a fluid filled sac on the spinal column. Central cord symptoms can then present such as hand weakness, dissociated memory loss, and even paralysis.


Diagnosis

Arnold-Chiari I is usually diagnosed when the patient presents to the doctor with complaints of neurological symptoms or headaches. The doctor then orders an MRI or a 3-D CT. The herniation can be seen with either of these imaging devices. Then the doctor will likely follow up with a neurological exam. Arnold Chiari II, III, and IV can be detected prenatally by ultrasound.

Depending on the symptoms the patient may need decompression surgery to relieve some of the pressure in the back of the skull. This is done by a neurosurgeon who removes part of the skull and sometimes the first, second and third cervical vertebrae.

This is my MRI showing my Chiari
This is my MRI showing my Chiari

My Personal Experience

I’m afraid to sneeze. Yes, you heard me right. I’m afraid to sneeze – I might push my brain out of its normal resting place. I was diagnosed with Arnold-Chiari Malformation I a few years back. Due to my OCD tendencies I researched as much as I could about the congenital abnormality. Sadly, at the time of my diagnosis not much information was available. Heck, there wasn’t even an entry for Chiari on WebMD! Today, there is information all over the internet and the disease is gaining more attention.

Chiari is an abnormality of the hind brain where the bottom of my brain hangs out of the back of my skull and into my spinal column. No biggie, right? At least that’s what the neurosurgeon said when I went to visit him for a consultation several years back. He said the 7mm herniation from both sides of my brain was no big deal but he would perform the decompression surgery for me if I wanted. What? Did I hear that right? I got to decide if I wanted…wanted….brain surgery?! Let’s go with “no thank you”. So at least now I have a diagnosis for all the symptoms I’ve dealt with for a very long time. What next? Well, nobody seemed to know at that time so I took the denial approach. Yup, I pretended nothing was wrong, hoped for the best, and prayed more research would be completed while I was waiting for something to happen. I spent months depressed, I spent months acting like I’d never heard of Chiari, I spent months afraid to hold my head in the wrong position. Now for the most part I’m aware and somewhat cautious. I listen to my body and don’t push the limits with exercise, I don’t lift heavy items, and I don’t hit my head. I treat the pain with a heating pad, Motrin and extra rest. I try not to complain too much. This is one of those invisible diseases where friends and family say “You look just like the rest of us” and doctors say “No big deal” because they don’t know what else to say yet. My favorite is when people make jokes that the symptoms must all be in my head. Really? Well, not in it per se, but definitely FROM it!

My symptoms vary from day to day depending on what I do. And heaven forbid I get a cold. The coughing fits and fevers bring on excruciating headaches that leave me crying. The consistent symptoms include headache, general achiness, tinnitus and exhaustion. Sometimes I get dizziness that could also be from sinuses and allergies. Sometimes I get blurry vision but I also have astigmatism. Sometimes I have trouble swallowing but I also have GERD so it could just be from years of acid reflux. So I have decided I will pass on surgery until more research is done and until I can no longer justify the way I live. For now, I’m managing just fine the way I am. Plus I still get to make jokes that I’m too smart for my own good. I’m so smart I can’t even keep my brain in my head.

JB Holmes
JB Holmes | Source

Public Figures with Chiari

Roseanne Cash

George Clooney

Joanna David (English actress)

Marissa Irwin (fashion model)

Chris Dugan (former NFL kicker for Buffalo Bills)

Patti Duke

JB Holmes (Pro golfer)

More by this Author


Comments 21 comments

Bardie 5 years ago

I have so much sympathy for you! Your diagnosis reminds me of the late the 70's when several doctors told me it was my imagination, I had no medical reasons for my symptoms/feelings around the time of my cycle. Ever hear of PMS? Or the early 90's when it took lots of tests to diagnosis my fibromyalgia, and I still have doctors and lay people saying it's all in my head. You think? Please keep writing on your diagnosis, it will help other people.


Ardie profile image

Ardie 5 years ago from Neverland Author

Thanks Bardie - You're the BEST! And now nobody would dare tell a woman PMS isn't real. AND fibromyalgia is in all the health magazines as a truly painful disease that's difficult to live with. Thanks for reading :)


Drjacki profile image

Drjacki 5 years ago from North Carolina

That was a very good description of this problem, and you really laid it out well - I know a few women with this (not sure, but it might be more common for women), and their neurologists told them the same thing. It really isn't a big deal for MOST people but that doesn't mean you are not suffering a bit more than some. The question of surgery is very personal...if the doc says it has a poor track record of really changing your particular symptoms yet carries a fair degree of risk of a complication, then it isn't worth it. BUT..if your symptoms compared with the risks DO make it worth it for you, then go for it. That's my basic approach to any surgical issue unless it's cancer...then there really isn't always much choice. Be well!


Ardie profile image

Ardie 5 years ago from Neverland Author

Hi Drjacki

Thanks for the compliment! Chiari is more common in women for some reason. The main reason I shy away from surgery is that I subscribe to the "If its not broken don't try to fix it" theory. Im not broken enough to require fixing of that magnitude :) Brain surgery is a HUGE deal and not something I want unless it is 100% the only way to go. And I thank God almost daily its not cancer - no choices there.


2413life profile image

2413life 5 years ago from Still figuring that out......

Hi Ardie, thank you for this very informative piece. I am afraid I have never heard of this and I have spent a lot of time researching "brain" stuff with regard to my daughter's MS, which seems to strike mostly women too. I had no idea about this disorder. You obviously do not let it get the upper hand based on all of your great work here. I will wish for you pain free days and a strong immune system to keep colds, sneezes and anything else that will bring you discomfort, far, far away.


Ardie profile image

Ardie 5 years ago from Neverland Author

Hi 2413 :)

Im so sorry to hear about your daughter. I hope she is well even with her diagnosis. MS is such a confusing/difficult disease. My doctor actually sent me for my MRI because she was sure I had MS. We were both very glad when we found out that was not the case. I truly appreciate your kind words more than you could know. This is the first I've been able to talk or write about the Chiari. Im slowly realizing IM still the one in charge here :)


poshcoffeeco profile image

poshcoffeeco 4 years ago from Cambridgeshire

Hi Ardie.Wow... That came like a bolt from the blue. You are so brave and so positive...I admire you for that. Let me know If I can ever help, even just to talk about it. I am here my friend.

The hub will be comfort to many as they may well be thinking that they are the only ones with the condition.

Now I really want to know more about you.


Ardie profile image

Ardie 4 years ago from Neverland Author

I appreciate the read Posh. Its a topic very near and dear to me. It caused me to crash and burn and then it forced me to find my own strength to deal with it. I appreciate the comforting words and anything you want to know - just ask!


RealHousewife profile image

RealHousewife 4 years ago from St. Louis, MO

Hey Ardie - sorry to hear about this. I would NOT want to have brain surgery either. That is trippy. I had never heard of this condition either...amazing they figured it out! It sounds pretty rare. You are amazing!


Ardie profile image

Ardie 4 years ago from Neverland Author

Hi RHW, thanks for reading this hub - it was THE most difficult for me to write. I'd played around with the idea for a year or two :)


Desiree 4 years ago

Hey there I was just diagnosed myself, and I am not going to end until a see a specialist in Chiari there is a neurosurgeon only an hour from where I live that does it every day. He does three different types of surgery and two are not as invasive per say as straight back/neck and brain surgery as the major one does to a person. I have similar symptoms but I am dizzy everyday and have black out spells at least once a week that only last a minute or two, never lose totally control am very aware of surroundings. Mine is not as bad mine is in between a 5 and 6mm but I can not live like this I have three small children and I am a teacher on my feet everyday. the pain that shots up I can deal with just the lightheadedness and the feeling as though my head is constantly floating has got to stop! I understand what you are going through but I have a question do you have Ehlers Danlos I have that as well!


Ardie profile image

Ardie 4 years ago from Neverland Author

Hello Desiree, Im so sorry for your diagnosis {{HUG}} and please please please don't compare your mm to mine. In all my reading I discovered that some people can have a 0 mm herniation (meaning its crowded but not hanging) and the amount of herniation doesn't matter - its more about how the CSF moves around and where its blocked and what-not. I am SO so sorry you are experiencing the black-outs and dizziness. I have decided THOSE are the two symptoms that will force me to look into the decompression surgery more - God forbid it ever comes to that. I would be very happy if you'd use the Contact Ardie function and keep me posted as to your progress or find me on Facebook, the link is in my profile here. But you'll have to mention HubPages or I wont accept...too many spammers :P

Take care of yourself.


Ardie profile image

Ardie 4 years ago from Neverland Author

Sorry, forgot to answer - no Ehlers Danos. Contact me with ANY other questions.


Desiree 4 years ago

Thank you so much for responding. I have got the ball rolling on the specialist but in the mean time, another doctor, an ENT, read my MRI/MRA and they have now found a lose in circulation to my brain. Yipeee yet another finding when all other tests are coming back normal and they are telling me it has nothing to do with my Chiari or that I need to see another doctor. I hope the specialist will work and I am truly starting to feel depressed and not wanting to do anything!


Ardie profile image

Ardie 4 years ago from Neverland Author

Hello Desiree,

Loss in circ to the brain?! Oh my...I hope the specialist can get you in real soon. I know sometimes it can take a month or more to get into an appointment. My docs all told me everything came back normal too - and the things I experienced had nothing to do with my chiari - it SUCKED. Then I was told everything stemmed from my depression. I wanted to scream "What depression?! I wasn't depressed until you all told me I was crazy and making up symptoms!"

Please stay strong girl. I am here to talk ANY time. If you send me a message on FB my phone will blink to let me know it's there. Im here for you. We chiarians have to support one another until more support becomes available.


Teresa Coppens profile image

Teresa Coppens 4 years ago from Ontario, Canada

I don't know what to say Ardie except I am so very sorry you have such a cross to bare. I applaud your commitment to Hubpages and your family. We can only hope a cure or more reliable treatment is available in the near future. I found this article extremely powerful with the inclusion of your own personal story. Thanks so much for having the courage to share with all!


Ardie profile image

Ardie 4 years ago from Neverland Author

Hi Teresa :) I'm ok with this now...for the most part. I've learned over the years how to listen to my body so I don't push myself. Now I just want to connect with and support others who are thrown into this diagnosis with minimal information. A lot of the info out there is terrifying. But I'm here to say you CAN live with chiari without surgery (for now), without being on disability, and without abandoning a life :) I thank you so very much for taking the time to read this. I know its not easy to take in (hug) you're a good friend.


Rolly A Chabot profile image

Rolly A Chabot 4 years ago from Alberta Canada

Hi Ardie... Thanks for the explanation here of this. You have shown incredible strength in sharing this. So please that you are willing. I so appreciate your approach "If it is not broke don't fix it."

To me that shows great strength and courage... keep writing Ardie you have a gift to be shared wit many.

Hugs from Canada


Ardie profile image

Ardie 4 years ago from Neverland Author

Hiya Rolly :) Thanks for reading my Hub on chiari - its not an easy thing to understand or to hear about. Most times I don't tell anyone about it because I get all the crazy stares and the questioning looks. Its not well known and its one of those invisible disorders. One day I hope to be rich enough to spend money making chiari more known.


jenntyl99 profile image

jenntyl99 4 years ago from Pennsylvania

Hi Ardie, It's comforting to know there are others like me. A few years ago I suffered the worst, concurrent migraines I had ever experienced. No medicine worked and I was given shots to stop the pain, which only lasted for a short time. Along with that, I was having my leg give out, visual problems, numbness and tingling and much much more. I underwent multiple MRI's, cine MRI's, spinal and full body MRI's all indictaing I had "borderline" chiari (with between a 4 and 6mm herniation). A few years earlier I had suffered a head injury, requiring stitches and also had to learn how to regain use of my left hand. Anyway, dr.s continued to prescribe depression meds and I refused to take them. I knew this was not the problem and was getting frustrated. After almost a year of tests and dr. visits I gave up and decided I would try to manage my pain and symptoms without dr.'s After all, I am sure there are others who have it much worse than i do. Thanks for sharing this story; It helps me feel like my decision is the right one for now.


nicole 4 years ago

:-) I have been dealing with migraines, weakness in my arms, swallowing difficulties, my leg give out on me and so on and so on. I have been in and out of ER with neck pain to back pain,etc. Given IV meds till comfortable and then sent home. Finally I got the referral to neurology and she said the same about my 5mm herniation too, "its not large enough to be causing all my symptoms. Yet I read through these stories and they're the same. I have days that my head feels like its floating and then other days I feel as though the pressure is going to pop my eye balls out. I have severe neck pain and shooting pains down both arms and am wondering if anybody else has this and if so what causes it and what is your experience till now? I have had MRIs for other things and that's when I decided to get copies and have my own to view. I asked questions and researched it on line and figured id better ask what the 5mm thins means? I recently went out on disability cause I just can't work with the migraines. I haven't had insurance so the last MRI I had was last year. But I feel like its getting sooo much worse but I wouldn't know what it means without info from you all. Hopefully there is someone out there that shares my story and will respond? By the way I also have been diagnosed with fibromyalgia, arthritis, and have an enhanced pituitary gland that hasn't been looked at in about 8 or 9 months. Thanks to all . Nicole

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