September is here and so begins fall, and Chiari Malformation Awareness Month. Now if you don’t know anyone with Chiari chances are you’ve never heard of it. But Chiari isn’t rare, not by a long shot. Chiari affects 1/110 people but it’s most commonly found on autopsies.
Here are some commonly unknown things about Chiari Malformation.
What is Chiari Malformation? In simple terms the base of the skull is malformed or flattened not leaving enough room to properly hold the brain therefore the brain is smashed like play-doh between the skull, the spinal canal and the brain stem.
It was first discovered on an autopsy in 1891 by Hans Chiari who noted the cerebellar tonsils were crammed into the bottom of the skull pressing into the brain stem and spinal canal. Later, it was also seen by students of Dr. Julius Arnold in 1917 who noted in cases of Spina Bifida the brain stem and cerebrellum were herniated. This led to the name Arnold Chiari Malformation.
There are six types of Chiari Malformations. The name Arnold Chiari Malformation seems to be most used with type 2, which is the type I have and the type that is closely associated with Spina Bifida, which I also have. The different types of Chiari Malformations have very little to do with symptoms. Someone with type 0 may be more symptomatic than someone with type 2 and vice versa. Symptoms seem to be dependent on what parts of the brain are affected and what co-morbid conditions the person has along with Chiari. More on that later.
Type 0 Chiari Malformation means that someone does not have a herniation of their brain, but does have the malformed skull and compression of the brain along with a syrinx. A syrinx is a fluid filled sac on the spine that forms when the spinal fluid is blocked from flowing normally.
Type 1 is the most common type and it means that a person’s brain is herniated, but not their brain stem.
Type 1.5 means that a person’s brain and at least part of their brain stem is herniated.
Type 2 is the same as type 1.5 except the person also has Spina Bifida. You must have Spina Bifida along with Chiari to have type 2.
Type 3 is extremely rare and happens when the skull does not entirely form during pregnancy.
Type 4 is the most rare form of Chiari and means the cerebellum did not completely form during pregnancy.
Now you’re probably saying wow that sounds painful. Oh it is. The number one symptom of a Chiari Malformation is a headache at the base of the skull where the malformation is. However, there are over 200 known Chiari Malformation symptoms. Some of the more common ones are headache, dizziness when laughing, straining, coughing or sneezing, numb arms/legs, short term memory loss, pulsating headaches anywhere on the head that seem to match your heartbeat, trouble concentrating either due to pain or pressure on the brain, neck pain, back pain etc. I’ve also seen it cause strokes in people.
Honestly each person with Chiari Malformation has a unique experience because Chiari Malformations affect our brains which means it can literally affect anything within your body at any time.
Chiari research is very underfunded, mainly because most people do not know it exists. One of the top Chiari research organizations has spent 3.8 million dollars researching Chiari since 2003. When you compare that to the amount of money that goes into researching things like cancer it’s basically nothing. The money just isn’t donated because the knowledge isn’t there. Before I was diagnosed if you had told me my brain was falling out of my skull I probably would have laughed and said that’s impossible. But it isn’t.
There’s a lot of controversy in the Chiari community over how it should be handled and I won’t get into that here because frankly I don’t want the drama. However if you ask me my opinion I will give it to you.
I will say this: Your brain controls every single thing your body does from breathing to talking to your heartbeat. You only have one brain. Once you lose it, it’s gone, you’re dead. Chiari has already damaged your brain to some extent just by you having it. Don’t be an idiot. Sound scary? Good! You understand the situation.
Doctors are not taught much about Chiari Malformations in medical school. I know that thought is terrifying but it’s true. Neurosurgeons are given barely a few paragraphs about it, told they will likely never encounter it and move on with their coursework. Yes, that’s right, a condition that every 1/110 people has they are told they will never encounter. Only those who have decided to study it further on their own really learn the ends and outs of the condition and everything that goes along with it. This is why even though I had every single risk factor for Chiari, I was not diagnosed until I was in my late twenties.
There is no treatment for Chiari other than surgery that removes a piece of the lower part of the skull to give the brain more room. Chiari has no cure and even with surgery there is no guarantee there will be any change in symptoms. I’ve learned just by surrounding myself with people who have Chiari what eases our pain and most of us agree caffiene is the only thing that eases a Chiari headache.
As I said before, the top place Chiari is discovered is on autopsy. Often times people die unexpectedly and it isn’t known they have Chiari until after they pass. Now with that said, getting a Chiari diagnosis is not a death sentence. Many of us lead full, long lives with a few modifications. And Chiari is often not the thing that kills someone that has it, often times it’s one of the co-morbids that hasn’t been diagnosed before, like sleep apnea, that actually kills a person.
Chiari is not a disease. It is a birth defect in the same way Spina Bifida is.
Chiari headaches are not migraines. In my opinion they are worse and I experience both. I’d rather have a multi day migraine than a Chiari headache for just an hour.
Chiari is rarely diagnosed by itself. Elhers Danlos Syndrome, Spina Bifida, Syringomyelia (Syrinx as mentioned above), Postural Orthostatic Tachycardia Syndrome, Neuropathy, Occipital Neuropathy, Scoliosis, Sleep Apnea and Disk Degenerative Disease are just a few of the MANY conditions known to also be found in Chiari patients.
Some FAQ I get from family and friends concerning Chiari:
How does Chiari affect my day to day life is a question I get a lot. Here’s the answer.
I never know how I’m going to feel for the day until I wake up in the morning and often times how I feel can change throughout the day. Today for example, I woke up feeling okay, by noon my sight was doing weird things and by this afternoon a Chiari headache hit me hard. Even as I write this now, I’m hurting and drinking espresso to try and curb the pain. My partner, who also has Chiari, is asleep on the floor trying to sleep off his pain.
I have days when I don’t feel pain at all. They are rare, but they happen and I cherish each and every one of them.
Sometimes my biggest symptom in a day will be nausea or I’ll be dizzy. It’s not always painful, but Chiari is always there.
How do I know the difference between a migraine and a Chiari headache?
Simple. A migraine will make me nauseated, sensitive to lights and sounds and has the usual migraine symptoms. Chiari headaches are pure pain.
The barometer can greatly affect my Chiari symptoms as well. If the barometer is too high OR too low my symptoms will spike. The barometer measures the pressure in the air and the pressure in the air definitely affects the pressure in my head.
What symptoms do I personally have?
Earlier in this post I listed a few general Chiari symptoms, with over 200 symptoms it’s almost impossible to remember them all, but here are the ones I experience regularly.
- Nausea (the difference between this and a migraine is I don’t have to have a headache to be nauseous, I just am)
- Dizziness when moving too fast or straining/laughing/coughing
- Memory Loss
- Forgetting words
- Neck/Back pain
- Sleep Apnea
- Unable to concentrate (I will literally forget what I’m doing WHILE I am doing it)
- Dropping things
- Depth perception loss
- Vision issues (cannot be corrected with glasses as there is nothing physically wrong with my eyes or optic nerve)
- Bladder issues (Spina Bifida also causes this but I can tell the difference in the two)
- Hearing loss
- Unable to form words (different than forgetting them, I remember them, I just can’t verbalize them correctly)
- Tinnitus (ringing in the ears)
I also have the following co-morbid conditions:
- Spina Bifida
- EDS (Ehlers Danlos Syndrome)
- POTS (Postural Orthostatic Tachycardia Syndrome)
- Sleep Apnea (this is by FAR the scariest of the symptoms. I personally have had friends die from this)
- Disc Degenerative Disease
Why is Chiari not diagnosed more often?
Besides a lack of knowledge on doctors parts, most people with Chiari do not realize that anything is wrong with their brains. Most of us have had symptoms our entire lives and symptom does not always equal pain. Most of us grew up thinking the things that are considered symptoms were normal and that everyone experienced them. Most of us are shocked to discover we are not as normal as we thought we were.
How is Chiari diagnosed?
MRI is the only way to properly diagnose Chiari. Although most of the time it is not an accurate way to determine if someone’s spinal fluid is blocked or how big exactly their herniation is.
What doctor treats Chiari?
Neurosurgeons treat Chiari, although there are less than five specialists in the US that I would ever let touch me.
Is Chiari progressive?
Yes it is. The longer the pressure remains on the brain the more damage is done.
Is Chiari genetic?
Studies have been done that indicate it does run in families.
Have you had the Chiari surgery?
Yes and it was one of the most painful things I have ever done, but with that said it was worth it.
Did your symptoms change after surgery?
Yes! Absolutely! My recovery was long and painful but about six months after surgery I felt better. The symptoms I have now are very much a muted version of the ones I had before surgery.
What is done during Chiari surgery? Can you give us more detail about it?
The surgery performed for Chiari is called a decompression surgery and what that means is part of the skull, and sometimes parts of the backs of the top vertebrae in the spine, are removed to give the brain more space and to take the pressure off of it that is created by the malformation itself. The surgery is performed in hopes of relieving symptoms in the patient and isn’t always successful. Often times the damage done to the brain is too extensive to repair itself, but some patients have experienced a reduction in symptoms after surgery.
Would you recommend surgery for everyone with Chiari?
Depending on symptoms and if they were able to get to one of the few specialists yes I would. I did NOT get to one of the specialists and I do regret that, I think my surgery would have gone a lot better if I had, but with the things that were going on in my life at the time I believe the surgeon I saw saved my life and I know I am one of the lucky ones. A lot of people that go to regular neurosurgeons for surgery have major complications or die. It is not a surgery to take lightly and if anyone would like to discuss my experiences further feel free to drop me a comment. Also if there’s anything you want to know about Chiari that I have not discussed here drop me a comment and let me know. I’m always open to helping people learn about Chiari as much as I can.
Some good links to learn more about the conditions I talk about here: