My Experience.
The Science.
Useful Web Sites. 

My Experience.
Before I get into the whole science part of my condition, let me give you some background as to how this all was thrown into my lap and forced me to pay attention to a whole bunch of boring stuff that I would have fallen asleep listening to before.

On December 20-something, 1999, a few hours after I took my last exam ever as an undergraduate in college, I was walking out of the Brower Commons dining hall on the Rutgers College campus, near where I was living that last semester. I guess I was thinking of the last-minute things I had to do before I had to start packing up my things so that I could move out of my dorm room the next day. I remembered that I had to check my mailbox one last time, so I took a quick 180° turn so fast that I lost my footing and had the worst fall of my life. Alright, I'll admit that I have had a lot of experiences in falling. And to be honest, none of them were ever graceful. But unlike the others, this fall knocked my head onto the pavement so hard that I was like gone for a few seconds and I wasn't able to move for some time. Hell, I couldn't even talk for a while. Luckily there were these two girls walking by with a cell phone and they called 911 and stayed with me while I waited for the ambulance. I'm not going to go into specifics of what happened at the hospital, but just that I couldn't get my balance back. Everyone knows I didn't exactly walk like I was all that steady to begin with, but this time I felt how shaky my legs were.

Now this fall wouldn't have been such a big deal if it hadn't started a chain reaction that would eventually result in another changing of my whole life. College was over. This was supposed to be one of the happiest times of my life. Or at least it should be a cause for celebration and pride for my having crossed into a new phase. Well anyways, a friend helped me get back from the hospital so I could pack up and be ready to move out of the dorm the next morning. When Mom got there to pick me up and saw my face, she was so worried for me. But those of you who really know my Mom can probably picture how she reacted the moment she saw me. The whole right side of my face looked like a poster visualizing domestic violence. I had a black eye and a swollen lip. That along with the cane caused Mom to become almost hysterical for a few minutes. She was like "what happened? What happened? Oh my God. What happened to my little girl?" I had to calm her down and tell her the whole story. She took me home, but the whole time she kept saying how much it probably hurt. She can be so cute sometimes.

I looked through Mom's big book of doctors and I ended up finding Dr. Elliott Grossman, a neurologist that has an office in Union that he only comes to on Wednesdays. The first time that I went to see him, I went with my Mom and Dhaval. After giving me a superficial neurological checkup and checking through my medical background, he gave me a prescription for an MRI of my head. He of course told me that I had nothing to worry about and that this was all just normal. I really had no problem with having to get an MRI done since I've gotten a few done before. I just wanted to know what was happening to me. I hated the feeling of not knowing whether or not I would fall the next time I took a step. I didn't really think there could be anything major, so I wasn't scared or anxious. Mom was worried, but I told her that the doctor was just following procedure. A few days after the MRI, I got a call from Dr. Grossman and the first thing he told me was that there was nothing to be worried about. But that is always what they say before giving you bad news. He tried to explain to me that I had something called a syrinx in my spinal cord and that I would need surgery. He said the syrinx was the building up of fluid in the spinal cord. And of course he vowed to me again that I had nothing to be worried about because this was not such a big problem. I don't know why, but I wasn't worried at all and I thought that I was going to be just fine. It wasn't because Dr. Grossman was keeping me calm, but I just don't think I really absorbed what he was saying at the time. All I wanted to know was what would happen next.

Dr. Grossman referred me to Dr. Arno Fried. He assured me that he was a great neurosurgeon who also happens to be a friend and former classmate of his. I found it interesting how he kept trying to convince me of Dr. Fried's capabilities. I never doubted them. Why do people see doctors as infallible beings and abandon all their senses and blindly take their word as that of God without so much as a simple question? And it's funny once you notice how no doctor would admit to referring you to a doctor who was nothing less than the best. I had so many questions that I should have asked of both doctors, but at the time all I could see was the word "surgery" flashing before my eyes. I needed to be calm and collected for myself and for my mother. She always had a habit of imagining the worst possible scenario of a situation and then getting all riled up about it. If you know her, you know what I mean.

Dr. Fried seemed like a distinguished doctor. I went to his office at the Hackensack University Medical Center with my brother. Dr. Fried looked through the MRIs and showed us the problem areas and tried to explain them to us. From what I understood, it was some sort of a birth defect. In Mom's uterus my brain apparently failed to develop properly. Cerebrospinal fluid that is constantly produced by the body to cushion the brain is normally diverted out from around the brain to wherever it's supposed to go and then it's broken down by the body, but in my case the fluid was being diverted into my spinal cord. This happens because the brain forms like this thing that looks like an extra flap of tissue at the base of my cerebellum. So this extra flap thing is called the Arnold Chiari Malformation after the guy who discovered it. A lot of the other stuff that he went on talking about didn't really make much sense to me at the time. Dr. Fried even suggested that the hydrocephalus that I had developed at the age of 10 was actually a result of this defect. I was so amazed and happy that I was finally getting an explanation for what happened to me when I was 10, even though it wasn't really making all that much sense to me.

So I thought to myself that this was all fine, but what would happen next. He explained that the weakness that I had been feeling and the problems with my balance were all due to the syrinx, or fluid building up in the spinal cord. He had me have another MRI done, this time of my spinal cord, to see the extent of the damage. Dr. Fried would have to go into the back of my neck and make some space because of the malformation and also put a shunt in to drain out the fluid that had accumulated in the spinal cord. The operation would be a necessity because without it, the fluid would keep on building up, increasing the size of the syrinx and eventually resulting in my death subsequent with the loss of my bodily functions. He couldn't have painted a more gruesome picture. Dr. Fried showed me in my own MRI the things that he was talking about. A normal spinal cord is like a thin string, but when you look at it from the top, it looks like a pipe or the top of a doughnut, where the doughnut hole is very small. But in my case, the fluid had caused the sides of the spinal cord to be pushed out to the point where the doughnut was like a thin ring. The fluid was causing massive pressure on the spinal cord, which in turn was affecting everything controlled by the spinal cord.

The Science

Now I give you some of the basics of the medical explanation of Arnold-Chiari Type II Malformation with Syringomyelia. It's important to mention that I have two separate conditions, but also that one usually results in the other. It's hard to talk about one without talking about the other because you can't necessarily differentiate where one begins and the other ends.

Syringomyelia.

The word syringomyelia refers to the collection of different conditions characterized by damage to the spinal cord that is caused by a formation of abnormal fluid filled cavities (syrinx) within the cord. In 1827, French physician Charles-Prosper Ollivier d'Angers suggested that term syringomyelia after the Greek syrinx, meaning pipe or tube, and myelos, meaning marrow. Later, the term hydromyelia was used to indicate a dilatation of the central canal, and syringomyelia referred to cystic cavities separate from the central spinal canal.

Generally, there are two forms of syringomyelia. The first major form consists of most cases, including mine, whereby the disorder is related to an abnormality of the brain called an Arnold-Chiari malformation, named after the physician who first characterized it. This abnormality causes the lower part of the cerebellum to protrude from its original location in the back of the head into the cervical or neck portion of the spinal canal. The syrinx may then develop in the cervical region of the spinal cord. Because of the relationship that was once thought to exist between the brain and spinal cord in this type of syringomyelia, it is usually referred to as communicating syringomyelia. Here, symptoms usually beginning between the ages of 25 and 40 and may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate suddenly. Some patients, however, may have long periods of stability.

The fluid filled cavity, or syrinx, expands slowly and elongates over time, causing progressive damage to the nerve centers of the spinal cord due to the pressure exerted by the fluid. This damage results in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms may include a loss of the ability to feel extremes of hot or cold, especially in the hands. People with syringomyelia experience different combinations of symptoms.

About 21,000 American men and women have syringomyelia, with symptoms usually beginning in young adulthood. Syringomyelia occurs in approximately eight of every 100,000 individuals. The onset is most commonly observed between ages 25 to 40 and those who have acquired syringomyelia due to some form of trauma. Rarely, syringomyelia may develop in childhood or late adulthood. Males are affected more often than females and there is no geographic difference in the prevalence of syringomyelia.

Most people with syringomyelia experience headaches, along with intermittent pain in the arms and legs, usually more severe on one side of the body. Numbness and tingling in the arms, chest, or back is often reported. Syringomyelia can also adversely affect sweating, sexual function, and, later, bladder and bowel control. The inability to feel the ground under the feet is frequently experienced. Weakness of an extremity, leading to clumsiness in grasping objects or difficulty walking may also occur in individuals with syringomyelia. Eventually, functional use of the limbs may be lost.

A watery, protective substance known as cerebrospinal fluid normally flows around the spinal cord and brain, transporting nutrients and waste products. It also serves to cushion the brain. A number of medical conditions can cause an obstruction in the normal flow of cerebrospinal fluid, redirecting it into the spinal cord itself. For reasons that only now are becoming clear, this results in syrinx formation. Cerebrospinal fluid fills the syrinx. Pressure differences along the spine cause the fluid to move within the syrinx. Physicians believe that it is this continual movement of fluid that results in cyst growth and further damage to the spinal cord.

Examination by a neurologist may reveal loss of sensation or movement caused by compression of the spinal cord. Diagnosis is usually reached by magnetic resonance imaging (MRI) of the spine, which can confirm syringomyelia and determine the exact location and extent of damage to the spinal cord. The most common place, as it was with me, for a syrinx to develop is in the cervical spine (neck). MRI of the head can be useful to determine the presence of any additional lesions present, as well as the presence of hydrocephalus (excess cerebrospinal fluid in the ventricles of the brain). I had hydrocephalus when I was 10 years old and because of the lack of MRI technology, the syringomyelia went undiagnosed.

Treatment, usually surgery, is aimed at stopping the progression of spinal cord damage and maximizing functioning. Surgical procedures are often performed if there is an identifiable mass compressing the spinal cord (in my case the Chiari malformation). The main goal of surgery is to provide more space for the cerebellum at the base of the skull and upper cervical spine without entering the brain or spinal cord. This often results in flattening or disappearance of the primary syrinx or cavity as the normal flow of cerebrospinal fluid is restored. Surgery results in stabilization or modest improvement in symptoms for most patients. Many physicians advocate surgical treatment only for patients with progressive neurological deterioration or pain. Delay in treatment when the condition is progressive may results in irreversible spinal cord injury, and post-traumatic syringomyelia remains difficult to manage. Recurrence of syringomyelia after surgery may make additional operations necessary; these may not be completely successful over the long term.

In some patients (like me) it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. The system is also known as a shunt. The physician locates the syrinx, and then places a shunt into it with the other end draining cerebrospinal fluid into a cavity. By draining syrinx fluid, a shunt can stop the progression of symptoms and relieve pain, headache, and tightness. Without correction, symptoms generally continue.

Since the natural history of syringomyelia is poorly understood, a conservative approach is usually recommended. When surgery is not yet advised, patients should be carefully monitored by a neurologist and periodic MRI's and evaluations should be scheduled. Whether treated or not, many patients will be told to avoid activities that involve straining.

Arnold-Chiari Malformation.

The Arnold-Chiari malformation, specifically, is a developmental anomaly at the base of the brain which results in the downward displacement of some of the brain's structures into the spinal canal. The excess cerebral matter extends downward towards the medulla oblongata, crowding the outlet to the spinal canal. There were four different types of Chiari malformation classified. They're subdivided to reflect the degree of displacement.
Type I-only the cerebellum tonsils have been displaced.
Type II-the tonsils plus the inferior part of the vermis and the inferior part of the IV ventricle are displaced. It is always associated with open bifid spine and hydrocephalus. It usually appears in children.
Type III-all the cerebellum has been displaced and there exists occipital encephalitis.
Type IV-there is hypoplasia of the cerebellum.

At the present time, only two types are distinguished:
Chiari type I-it appears clinically in the adult age.
Chiari type II-it appears in children with bifid spine and hydrocephalus.

I was told by my doctor that I had the type II variety of Chiari, so I'll focus mostly on that form's background and research.

The exact cause for Chiari Malformation is unknown but it has been suggested that malformations happen during the early development of the embryo. It is not unusual for the Chiari Malformations to have an associated accumulation of fluid within the interior of the spinal cord or brainstem (syringomyelia).

I don't want to bore you with all the information on symptoms, frequency, and such about Chiari II because they are all available at any of the web sites I've listed below.

Failure to diagnose a Chiari II malformation and its associated anomalies in a neonate or a child can cause serious brain and spinal complications. Failure to identify early cranial and spinal cord lesions may result in delayed intervention and permanent neurological deficit.

The urgent decompression of the children with Chiari Type II and symptoms of main trunk function offers very good results. 10 of 13 children treated with this methodology presented a clear and fast improvement of the symptoms after the surgery. 68% obtain a total or nearly total resolution of the symptoms, 12% percent as after effects some moderate deficit and a 20% does not present improvement. Respiratory failures are the first cause of death, followed by meningitis/ventriculitis and broncoaspiration. According to some studies with patients aged between seven months to six years post-operating mortality was of the 37.8%. The preoperating state and the fast deterioration of the symptoms are the most important predictive factors.

The benefits of surgery should always be weighed carefully against its risks. Although some patients experience a reduction in the symptoms, there's 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.

Arnold-Chiari type II, which is what I have, is a bit rarer and more difficult to treat than the type I version of the Chiari malformation. So obviously for that reason, research money and grants are more likely to be spent on the former rather than the latter.

There is a study currently being conducted to establish protocols for developing a larger-national multicenter study to evaluate surgical approaches and outcomes in surgery for patients with Chiari type I malformation and syringomyelia. That's a bummer for me, but this may just be the first step needed to start the process going.

While browsing over the Internet, I was surprised when I saw that in 2001 a place called the Chiari Institute opened up in Great Neck, New York by the North Shore-Long Island Jewish Health System. It was founded by Dr. Thomas H. Milhorat. The Chiari Institute is dedicated to the treatment of patients suffering from Chiari malformation, syringomyelia, and related disorders. Their mission is to educate patients in the neuroscience community, and advancing our understanding of the diagnosis and treatment of those conditions.