What Is Arnold Chiari Malformation?
Meet My Friend, Arnold
It all began with a headache. Not just any headache, a H-E-A-D-A-C-H-E. A headache so unbelievably painful that it woke me from a dead sleep because the intensity of the pain made it hurt to breathe. Now that's a HEADACHE! And when the same excruciating pain awoke me four nights in a row, I knew that I had a problem.
So began my membership in the world of neurological diseases and my journey down the path of coping with an Arnold Chiari Malformation.
Please, grab something to drink and maybe a snack, plop down on a comfortable chair, and join me on my journey down the Zipperhead Road.
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Living with a Brain Fart
"I am a sick man. ... I believe my liver is diseased. However, I know nothing at all about my disease, and I do not know for certain what ails me." -- Notes from the Underground, Fyodor Dostoyevsky
Unlike Dostoyevsky's Underground Man, I do know what ails me, and it isn't my liver; it's my brain. I have a progressively degenerative condition called Arnold Chiari (key-AH-ree) Malformation I, or ACM I for short. The image above shows and describes the problem quite well. In medical terminology, ACM I is a herniation of the brain, but I prefer to call it a brain fart. What it means for those of us who suffer from it (and I do mean suffer!) is that a portion of the brain has poked through (herniated) the dura mater, the protective membrane, and migrated south into the spinal canal where it competes for space with the spinal cord.
Probably because of its importance, the brain gets three layers of protection. Of the three layers, the dura mater is the outermost and toughest, so just imagine the pressure required to push the brain out of its triple-strength cover! It's like squeezing a piece of meat through the cellophane wrapping on the package. Most people who have ACM don't know when their brain breached the walls of its fortress because it's not until the brain actually begins its vacation in the south that they become aware of a problem.
The spinal canal is just wide enough for the spinal cord, so when a hunk of brain takes up residence there, all sorts of unpleasant things begin to happen: headaches, dizziness, vertigo, balance problems, disequilibrium, coughing, sneezing, loss of fine motor control, neck pain, muscle weakness, double vision, hearing difficulties, tingling sensations in the limbs, and headaches. Did I mention headaches? It was after a series of paralyzing headaches so painful it hurt to breathe that I decided something must be wrong and went to see my doctor, who ordered an MRI.
"Carleen," my physician said, "I've got good news and bad news. Which do you want first?"
"Let's start with the good news because it will soften the bad," I replied.
"The good news," he said as he looked the radiologist's report, "is that the MRI is proof that you have a brain. The bad news is that it has a problem." Thus began my entry into the world of neurological diseases.
Thanks to Google, I was able to learn a lot about the condition and to find others who have it. Even better for me, the information that I had gathered about Chiari allowed me to begin linking symptoms that had gone on for years, but were misdiagnosed as often happens, to the disorder. It also enabled me to prepare a series of questions for the neurologist. After a whole new battery of tests, the neurologist recommended surgery to ease the symptoms and slow the progression of the brain seepage.
ACM will worsen over time, and since the contest for space between my brain and spinal cord was already causing misery, decompression surgery was necessary. Did I mention that if left untreated, ACM can cause paralysis or death? Since neither of those options took much consideration on my part, I had lots more tests and had surgery in February 2006.
I am now an official "zipper head," the nickname that Chiari patients call themselves. The three part surgery -- craniectomy (opening the skull and removing a piece of the bone), duraplasty (opening and repairing or replacing a piece of the dura with a patch -- mine is made from bovine pericardium), and laminectomy (removing bones in the neck -- I had the C1 and C2 taken out)-- has improved my symptoms considerably. The headaches aren't gone, but they don't happen nearly as often and seem now to be triggered by stress more than anything else.
Most Chairi patients experience symptoms on one side of the body almost exclusively and for me, they happen on the right side. I still have vision issues with the right eye despite having corrective surgery. I can go up a flight of stairs but my balance and vision are so bad that I can't walk down them without falling. Even stepping off a curb can cause me to fall! One symptom that worsened instead of improving was an almost constant tingling sensation in the right arm and a feeling of spacing out.
A follow-up visit to the neurosurgeon prompted an EEG which revealed scar tissue and seizure activity in the right parietal lobe. Evidently I had been having partial-complex seizures for quite a while without realizing it! The politically correct term for this problem is "seizure disorder," but it is nothing more than epilepsy. Although I lost my driver's license for 18 months, I did have a chauffeur who took me to and from work. Husbands come in really handy sometimes!
My life has changed dramatically since I first got the Chiari diagnosis, but it hasn't been all bad. I choose not to sit on a pity pot and let life pass me by in the process. Instead, I do what I can when I can and go on from there. Yes, I get funny looks from people when I get into the elevator on the second floor to go down to the first, but I don't care what anyone thinks. It's kind of hard to avoid curbs, so I just step down sideways to avoid falling. Doing anything that requires me to stand on one leg at a time is just about impossible thanks to the balance problems, but I compensate by leaning on something or sitting down whenever possible. Nope, no pity parties for me! I am lucky enough to have received a diagnosis and treatment before the brain fart paralyzed or killed me, so why complain about tripping over curbs?
ACM I is classified as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that ACM I affects less than 200,000 people in the US population.
Common ACM Symptoms
The cerebellum, called "the brain with a brain" because of the functions it performs, is about the size of a peach and is located at the base of the brain. Its responsibilities include controlling things like sensory perception, coordinating movement, planning, on, and motor skills. It sends information to the body about maintenance of equilibrium, balance, regulation of muscle tension, and coordination of limb control.
In Chiari patients, the mid-brain, pons, medulla and cerebellum are crowded together, often with the cerebellum pushing down into the spinal cord column. With these areas of the brain being squished and with a displaced cerebellum, the normal functions that these sections of the brain perform become impaired or compromised. Additionally, the protrusion of the cerebellum into the spinal canal can block the even flow of cerebrospinal fluid which creates increased pressure in the brain and around the spinal cord.
So, what happens when the brain gets squished? Well, we get things like:
- neck pain
- difficulty swallowing
- poor balance
- muscle weakness in the head and face
- involuntary rapid eye movements
- double vision
- ringing in the ears
- inability to coordinate movements
- sudden pain around the eyes
- leg weakness
- weakness in arms and hands
- shortness of breath
- word finding problems
- loss of peripheral vision
- hoarse voice
Roseanne Cash, daughter of the late Johnny Cash, had decompression surgery in December 2007 for ACM I. Read all about it.
Types of ACM
As if one isn't bad enough, there are four different types of ACM and the higher the number, the worse the condition.
Characterized by protrusion of the brain tissue below the opening of the base of the skull. Patients may also have an abnormal fluid "blister" called a syringomyelia and/or a greater accumulation of CSF (cerebrospinal fluid) in the skull, also called hydrocephalus.
This type is diagnosed when both the cerebellum and the brain stem protrude down into the spinal column. These patients may also have syringomyelia and hydrocephalus. ACM II is most often associated with spina bifida, a congenital disorder in which the vertebrae fail to completely encircle the spinal cord.
Includes all of the signs of the other two types PLUS a bulge in the spinal cord caused by a sac (myelomeningocele) that contains CSF. This type causes severe neurological defects.
The last type of ACM involves an incomplete or underdeveloped cerebellum, also called cerebellar hypoplasia. In this rarest form of ACM, the cerebral tonsils are located further down in the spinal canal, some parts of the cerebellum are missing, and portions of the skull and spinal cord are visible.
Chiari Support & Education
Discovery Health Channel's "Mystery Diagnosis"
Because the myriad symptoms make it difficult to diagnose ACM, many patients suffer a long time without treatment. See one patient's journey to diagnosis on an episode of Mystery Diagnosis by clicking here:
Discovery Health Channel.
You don't need to download anything; the show is available online.
My husband says that I sleep like a cat. You know how cats nap for an hour or two and then get up to play or eat? That's been my sleeping pattern for a few years now. It is frustrating, debilitating, and most of all, exhausting! I count a good night's sleep as one where I've managed to stay asleep continually for at least 3 hours. They don't happen very often. Is it any wonder that my neurologist has classified me as someone with chronic sleep deprivation?
Because I don't get enough sleep, I go through life looking and feeling exhausted all the time. In order to function, I have to nap during the day. And sometimes, when my body decides that it has had enough and needs some sleep, it will knock me on my fanny for a day or two while it plays catch up.
Of the myriad symptoms associated with ACM, sleep deprivation is the one that bothers me the most. In addition to developing the habit of snoring, I struggle with heart palpitations and seizures during the night -- none of which is conducive to sleep. I'm not alone in my misery, though.
A study published in the December 2003 issue of the Journal of Neurosurgery indicates that sleep apnea is a common cause of ACM patients' persistent tiredness. Since the respiratory control centers are located in the portions of the brain under assault by ACM, it would make sense that Chiari patients would have a higher incidence of sleep disruption than the control group. In fact, we beat the control group by a significant margin: 64% of the patients in the Chiari group had, on average, 18 episodes of sleep disruption in a single night as compared to 12% of the control group whose sleep was disrupted an average of 3 times in the same time frame.
It's no wonder I sleep like a cat and can count chronic sleep deprivation among the numerous diagnoses that accompany Chiari!
Boy, aged 3, sleeps for first time in his life!
Rhett Lamb's ACM I has kept him from sleeping all his young life. Following an experimental surgery, the little guy slept for the first time ever! Read his story here and here.
Chiari Patients' Blogs - Enter Our World in Our Words
There's nothing better than getting information about something straight from the horse's mouth, so I've collected blogs written by Zipperheads about ACM and their struggles with it.
After reading some of these entries, I was immediately reminded of how very grateful I am that my own trial with ACM isn't any worse than it is. There really are people in the world whose situations are far worse than mine.
- Life, Liberty, and Good Coffee
This is my blog and because I am so much more than the limitations that ACM places on me, I write about Chiari, education (I teach at a state university), politics, family, and anything else that strikes my fancy.
- Live Love Laugh
Lacie is a young mom with young children. She blogs about dealing with her fairly recently diagnosed ACM, her family, and coping with it all.
- Creative Disaster
Nicole's upbeat take on Chiari and the surgeries she has had to help treat it make for some fun reading. Her blog isn't only about Chiari, and that makes it even more fun to read.
- Shauna's Life in Pain
If you want to read a truly inspirational blog from someone who deals with chronic pain, read Shauna's! It is filled with lots of practical information (Shauna is a nurse) -- like preparing medications for an emergency evacuation or some other type o
- Carolyn's Chiari
Carolyn has a myriad of issues that she has to deal with, including ACM. She posts regularly and gives plenty of information about the conditions for which she is currently receiving treatment.
- Too Much Brain to Contain
Although this blogger is currently on hiatus, her blog is filled with insightful information on dealing with ACM. She's got a great sense of humor, too!
- Droopybrain's Weblog
This patient vents her frustrations over those in the medical profession who refuse to listen to their patients' list of ACM I symptoms because they have a fixed set of symptoms in their heads which they feel confident is the only set of symptoms ass
- Sheila's Chiari and SM Journey
In addition to some great links for information about Chiari, Sheila's blog is filled with personal posts about how she copes with the day to day pain associated with the disorder.
Food for Thought
Women have a higher incidence of ACM I than men, although nobody in the medical field knows why. I think I have the answer: women naturally have bigger brains than men because they are smarter! ;)
Learn More about ACM
- National Institute of Neurological Disorders and Stroke Chiari Malformation Information Page
This informative page covers everything from symptoms to diagnosis to treatment to current research. It's a government website, so the information is fairly current.
- The World Arnold Chiari Malformation Association
Here you will find information from others, like me, who have ACM. The Yahoo Group is quite active and helpful to the newly diagnosed as well as those who just want to understand the disease.
The American Association of Neurological Surgeons has a very informative website that includes a brief article on ACM and a link to help you find a neurologist in your area.
- Large Study Reveals Wide Range of Chiari Symptoms
A study published in the March, 2004 issue of the Journal of the American Academy of Nurse Practitioners has verified what Chiari patients have known for years; namely, that while some symptoms are common to many Chiari sufferers, the malformation a
Partial Complex Seizures
As I mentioned, decompression surgery didn't eliminate or significantly reduce an almost constant tingling sensation in my right arm or a feeling of spacing out. An EEG revealed seizure activity in the right parietal lobe of my brain, along with plenty of scar tissue indicating that I had been having seizures for quite a while without ever realizing that's what they were.
A partial-complex seizure, which is the type that I have, isn't quite as scary as the grand-mal type that most people associate with Epilepsy. I don't black out or lose consciousness, I just kind of daydream for a while. I am fully aware of what is going on around me, but I am completely powerless to respond or react to it until the seizure is over.
Most people who have partial-complex seizures have an aura, or a warning sensation that lets them know in advance that the seizure is coming. Lucky for me, I have two: the smell of something burning and an immediate, overwhelming feeling of nausea. Because I live in an area that has a fire season and wildfires occur every year, the first aura can sometimes cause confusion for me. Am I going to have a seizure or is there really a fire somewhere? In any case, when that aura happens, I have no idea when the seizure will hit me -- just that it will. With the nausea one, though, I know that it's just a matter of seconds before I'm right in the middle of the event.
Please exercise caution around people who have seizures. Don't try to restrain the person unless they are behaving violently or are in immediate danger of hurting themselves. Jumping in to "help" someone in an active seizure could cause them to accidentally hurt you.
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If you learned something about ACM or found this lens informative, please let me know. If you already know about ACM and have suggestions for improving this lens, please let me know that, too!