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In March of 2000 doctors at Cardinal Glennon Children’s Hospital implanted a new device in Chelsea’s chest, a battery-powered “vagal nerve stimulator” designed to calm the brain activity that causes seizures.

“A guide wire from the stimulator is placed along the left vagus nerve, which wanders around many internal organs in the body,” said Thomas P. Geller, M.D., a neurologist at Glennon and associate professor of neurology at Saint Louis University. “It turns out that stimulation of the input to that nerve can inhibit seizures by its effect on the lower brain stem centers.”

Tiny electrical pulses from the device are sent to the vagal nerve at regular intervals.

“The stimulator was first tested on humans in the early 1990s. It is becoming a part of the management of the intractable epilepsy patient. It usually doesn’t cure the problem but it can substantially reduce the frequency of seizures and allow us to reduce the amount of medicine these patients are taking.”

Chelsea, now a tall, smiling seven-year-old, was one of the first dozen children to receive the stimulator at Glennon. The results have been better than expected. “It has decreased her seizure activity down to two or three a week,” Mrs. McCormick said. “Her attention span has improved, and the biggest change we’ve seen is in her verbalization.

“Before the vagal nerve stimulator she said maybe five to ten words and they were very sporadic. She’d say a few words and then you wouldn’t hear any for months. Since she got the implant, she has been making leaps and bounds. She still is not talking like a seven-year-old, but she learns a new word almost every day. Dr. Geller thought she might have missed her window for speech development, so he is really surprised.

Her teachers are amazed at what she has done in the last year.” No structure on earth may be as complex, wonderful or mysterious as the human brain. The brain and the rest of the nervous system play a role in the work of all other organs of the body.

“The nervous system is the tissue of the body that is sensitive and conveys information,” Geller said. “Its cells transmit information by electrical excitability. The neuron is the one cell type within the body that is capable of responding to stimulation by what’s called an action potential, which is an electrical spark.

“In a sense, the neuron can excite the cells around it. It can make the cells of the muscles move, and the interaction of these cells is what constitutes the ability to think.” Glennon’s five neurologists care for about 3,000 patients each year. There are many diseases, congenital conditions and injuries that can impair the nervous system.

“We see premature infants to college students, and everything from epilepsy, migraine headaches, cerebral palsy, mental retardation and developmental delays to less common things like tumors of the nervous system or brain and metabolic diseases,” Geller said. “For the most part, neurologic diseases are a consequence of a genetic disorder that affects the sensitivity of these cells or the way they transmit information, or a problem with the cells’ supply of glucose and oxygen and their elimination of waste materials.”

“The cells need a system that has the right temperature, nutrients and oxygen content in order to function.”

Glen Fenton, M.D., assistant professor of neurology at Saint Louis University and another member of Glennon’s neurology division, helps patients and parents understand the nervous system by describing it as an electrical communication system.

“I talk about the nerves being like telephone lines that the brain cells use to communicate with the muscles and other organs of the body,” Fenton said. “There is an electrical jumping of impulses that comes down the cable of a neuron, which then communicates with the adjacent cell by a chemical reaction.

“There is insulation on the cables to help the nerves work better. There are certain diseases that cause the insulation to come off, and other diseases that impair the cable itself.”

The brain and nervous system share countless connections that enable the brain to constantly receive information from all parts of the body, analyze that information in various portions of the brain and respond with commands sent back out on the nerves.

“It is pretty complex. There are a number of feedback loops from one component of the nervous system to another. Information is constantly going back and forth,” Geller said. The nervous system, however, lacks the ability to regenerate cells as most other parts of the body can. Once your nervous system has formed, you will live with it — and any subsequent injuries to it — for the rest of your life.

“The same cells you generated before you were 20 weeks old as an embryo are still there,” said Glennon neurologist and Saint Louis University assistant professor of neurology, Alma Bicknese, M.D. “Once the nervous system is locked down, it’s held there forever.”

As a result of the system’s complexity, there are many opportunities for its operations to go awry. “There are probably hundreds of conditions we see,” Fenton said. Epileptic seizures, like Chelsea’s, are one of the most common conditions seen by neurologists at Glennon.

“Epilepsy is a tendency to have repeated, unprovoked seizures. These are brain storms that result in a clinical manifestation, usually a movement like chronic jerking, an impairment in consciousness or both,” Geller said.

“Epilepsy is usually caused by some kind of scarring in the brain, either during the patient’s development or resulting from an injury or infection.”

Neurologists have vastly more tools to use in the treatment of epilepsy and other conditions, even compared to a few years ago.

“When I started practicing neurology ten years ago, there were five or six anti-seizure medications. Now there are 15 to 20,” Fenton said.

Some patients are put on a special high-fat protocol, called a ketogenic diet, which somehow discourages the brain from having seizures. Surgical procedures may be helpful to other patients. The vagal nerve stimulator is one of many new treatments that are expected to be available to neurology patients in the near future.

“The decade of 1990 to 2000 was declared ‘The Decade of the Brain’ by Congress, and a lot of new information on the brain was developed during that time frame. Our understanding of the brain is exploding,” Geller said.

A lengthy history of the patient’s symptoms and family health is the first step taken in diagnosing a neurological condition.

“The nervous system is so vast and abstract, so our diagnostic process is a little less concrete than in other fields of medicine,” Geller said. “We start by testing the nervous system using simple techniques that have been established for many years. These basic tools help us localize the problem in the nervous system.”

After the simple investigative techniques have pinpointed a problem, a number of sophisticated tools can be applied to identify specific abnormalities. Computer tomography and magnetic resonance imaging can provide pictures of the brain’s structure and even its metabolic processes. Nerve conduction tests and electromyograms analyze the abilities of the nerves and muscles to conduct and respond to electrical impulses. Barbara Tournour, R.N., C.P.N.P., has been a nurse at Glennon for 40 years, 21 of them in the neurology department.

“We can find some pretty incredible information. There are so many ways that we can diagnose things now that we couldn’t do when I started,” she said. “Back then, things were so different.”

The advancement of neurological patient care has even extended to the understanding of how a proper wheelchair fit can improve a patient’s quality of life. “There are a thousand ways things have improved, just in the past five years,” she said.

Parents can be relieved just to learn why a patient has a neurological condition, Tournour said, but that is not always possible.

“When we read about things as common as mental retardation and developmental delays, the best studies have shown that almost 40 percent have an unknown etiology,” said Bicknese.

Many nurses and doctors are attracted to neurology just because so much remains to be learned about the nervous system.

“Every patient is like a puzzle that you have to figure out,” Bicknese said.

“The brain is the most important organ we have, and the most intriguing,” said Fenton. “It is the least-known organ, therefore it holds out the most advancements that can be made in the future.”

Sleep disorders are a special interest of Fenton and Geller. Fenton oversees Glennon’s sleep disorders laboratory, in which children spend a night under observation while wearing instrumentation that measures brain, muscle and breathing activities.

“The most common sleep problem in children is insomnia. It is very common to have that along with attention deficit hyperactivity disorder. About six percent of the population of six- to- eight-year-olds have ADHD, and a good percentage of those have sleep problems along with it.”

Most insomnia is caused by emotional disturbances that can be treated with psychological counseling and sometimes medication. Children also may have airway problems that can be alleviated through surgery or the use of a device that increases airway pressure during the night.

Geller is studying sleep disorders that seem to appear after children have been treated for leukemia and brain tumors. Many children come to Glennon for evaluation of learning and behavioral difficulties. “Some of these kids have underlying neurological problems,” Fenton said.

“Mental retardation and attention deficit disorder are frequently associated with behavioral disturbances, impulse control problems and aggressive behaviors.” Ruthmary K. Deuel, M.D., Saint Louis University professor of neurology, is a Glennon neurologist who has a special interest in what she calls “disorders of thinking,” which include learning disabilities and autism. She is writing a book about autistic disorders, which cause patients to be extremely inward-focused with limited recognition of the world around them.

Statistics indicate that developmental problems affect five to seven percent of school children who have not had brain injuries, she said.

“It is much more carefully recognized today. There is much more categorization and better ways of determining specific behaviors and testing cognitive abilities,” she said.

Many of the children she sees are referred to her for attention deficit/hyperactivity disorder, which she views as a catch-all description that includes many neurological and behavioral problems that are characterized by distractibility, impulsivity and inability to normally focus and refocus attention.

“Behavior is what we see. But then we have to understand what causes that behavior,” she said. “Does the child have a problem with thinking or understanding? Is the child reacting emotionally? We have to analyze what the child does on several different levels.

“About 10 percent of children who are considered to have attention deficit disorder have a primary problem with the ability to focus their attention, and that probably is due the way the brain is formed.”

Medication is currently a leading treatment for attention and hyperactivity disorders. Deuel believes new forms of cognitive and behavioral therapy will soon help patients with these disorders learn how to compensate for their conditions.

The rapidly expanding knowledge of human genetics also should boost the treatment of neurological diseases, Fenton said.

“The most enigmatic patients are those who have an enzyme deficiency. We can treat their symptoms but we can’t really cure their disorder,” he said. “Sorting out where the genetic defect is may help us fix the defect so we can reverse it before damage is done.”

Bicknese is pursuing research into the development of neurons and possible means of helping the body grow new nerve cells. She is working with the St. Louis Cord Blood Bank at Cardinal Glennon to see if any of the stem cells found in umbilical cord blood can be managed so that they become nervous system cells.

Stem cells are embryonic cells that are capable of differentiating into any of the hundreds of types of cells in the body. Cells from the Cord Blood Bank have been used to replace the blood systems in hundreds of patients, in St. Louis and elsewhere, after chemotherapy. Those patients have been treated for cancers and other blood-related diseases.

“We have some cells in umbilical cord blood that look like they can at least express proteins that are only expressed in the brain,” Bicknese said. “I’m hoping to find cells that could be used to treat neurodegenerative diseases.”

It will be many years before such treatments are likely to be available, she said. “So little is known about how the brain develops, the possibilities of doing a therapy are very remote. I’m just building a basement, or part of a basement, so eventually someone else can build a house.” While many neurology patients cannot be cured, much can be done to improve the quality of their lives.

“There are many disorders that are still mysteries to us, but there are many treatments we can provide our patients to make their lives better,” Geller said. “We try our best to make their lives comfortable and provide them with educational accessories and specific occupational or physical therapies.”

The treatment of neurology patients involves many of the medical divisions and services of Glennon. “We couldn’t give the patients the care we give them without the team we have here,” he said. Tournour works to be a resource for parents as well as patients.

“Most of our parents are pretty incredible with what they have to deal with. Taking care of some of these children is a 24-hour-a-day job,” she said. “I teach the families on a one-on-one basis and try to give them the tools they are going to need. Parents are more comfortable asking a nurse about their concerns. Sometimes they call just because they need to talk to someone.”

Tournour tries to help families with seriously-ill children anticipate the stresses that will arise in their lives. “I tell them to take time out for your marriage, and get baby-sitters so you can get out and take care of yourself.

Mothers with babies who are really complicated think nobody else can take care of their child. I convince them they need to do things to keep themselves healthy.”

Mrs. McCormick, herself a pediatric nurse, said she doesn’t know how she could have made it through Chelsea’s struggles without the emotional support of Glennon’s doctors, Tournour and Tracy Moore, R.N., the neurology pediatric nurse practitioner. “It definitely made a difference.”

About half the neurology patients who are evaluated at Glennon remain patients until they reach adulthood.

“When I see a baby in the nursery today and see the problems he has, I know he will be here for the next 18 years,” Tournour said. “It is really hard to let them go when they reach 18. After getting to know us all their lives, many families are just devastated when it’s time to move their babies to an adult environment. Some of them continue to call for years.”

Since Chelsea Tucker received the vagal nerve stimulator, Dr. Fenton and Dr. Geller have been able to reduce her medications. The teachers at her Edwardsville, Illinois, school are thinking of gradually transferring her from a special class to a mainstream kindergarten or first-grade class.

“She loves school and can’t wait to get on the bus in the morning. She gets up and says ‘bus-bus-bus.’ She writes her name. She likes to play basketball and swim, and she likes to draw and read books,” her mother said. “She always has loved books, but at first she just liked to look at the pictures. Now she will look at the pictures and say the name of the object. She thinks it’s great.”

It seemed that Chelsea used to notice little of the world around her.

“She has a sparkle in her eyes that she didn’t have before. Dr. Fenton noticed that the last time we saw him. He said, ‘Wow, look at that smile!’”

And, Mrs. McCormick said, “She is aware of her environment. One day we were walking and she looked up and pointed at the sky and said ‘airplane.’ We didn’t know she ever noticed anything five feet in front of her. Everything has just come together.

She wasn’t supposed to do a lot of the things she is doing. She’s like a whole new child!”