What Is Arnold Chiari Malformation?

"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?

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.