CCE Seizure Disorders: Barrow Neurological Institute

August 7, 2009

Although BNI conducts research and clinical trials focusing on a wide variety of pediatric- and adult-onset epilepsies, one of its marquee programs is within the Barrow Hypothalamic Hamartoma Center.

Barrow Neurological Institute
Phoenix, Arizona

There can be no middle ground when it comes to epilepsy. "The goal of epilepsy management is to make patients completely seizure-free without side effects, so they can live normal lives and realize their full potential," says David M. Treiman, MD, director of the Epilepsy Center at St. Joseph's Hospital's Barrow Neurological Institute in Phoenix. Patients referred to BNI usually experience seizures on a daily, weekly, or monthly basis. "One seizure a month means you cannot legally drive," Treiman points out. "It compromises your ability to live your life."

The philosophy of a seizure-free patient is what drives the Epilepsy Center at BNI in its research, education, and patient care.

"Our intent is to provide state-of-the-art, comprehensive care for our patients, advance knowledge of epilepsy and how to treat epilepsy through both laboratory studies and at the bedside, and to teach the art and science of epilepsy to other physicians," Treiman says.

Focus on hypothalamic hamartoma

Although BNI conducts research and clinical trials focusing on a wide variety of pediatric- and adult-onset epilepsies, one of its marquee programs is within the Barrow Hypothalamic Hamartoma Center. "Hypothalamic hamartoma is a rare tumor that occurs in the third ventricle of the brain, but it gives rise to epilepsy," Treiman says, "which is very difficult to control unless treated surgically." The tumor develops in infants and children.

The center is one of the few worldwide equipped to surgically treat HH, and it has performed about 150 of the procedures since opening in 2003. Patients have come from 36 states and 10 countries; the youngest patient was 8 months old and the oldest was 58. Barrow neurosurgeons learned the technique from specialists in Melbourne, Australia. The center provides comprehensive HH care encompassing numerous disciplines that focus on evaluating, diagnosing, treating, and counseling patients and their families.

For example, BNI researchers have analyzed HH tissue to determine how brain cells interact to produce an epileptic seizure. "It's been useful," Treiman notes. "The research has gone from the bedside to the laboratory. Hopefully, and eventually, it will go back to the bedside."

Comprehensive management is key

BNI provides diagnostic testing, treatment of epilepsy through medical management and surgery, counseling and stress reduction programs for children and adults with epilepsy, and referrals to community resources. The facility has two epilepsy monitoring units: a 10-bed unit for adults and a 5-bed unit for children. Its staff includes 13 epileptologists-five adult, five pediatric, and three research-and four neurosurgeons-two adult and two pediatric. There is also a full-time neuropsychologist assigned to the Epilepsy Monitoring Unit.

Care begins by ascertaining the patient's history. "What differentiates an epileptologist from another neurologist is our ability to talk to and learn from the patient and the "patient unit" (the patient plus whoever else accompanies the patient)," Treiman says. "Sometimes patients are referred for better management of known epilepsy; sometimes they are referred because of a fainting spell or some other cause of episodic impairment of consciousness. We take a history and sort out what is likely going on. We use a neurologic examination as part of the process."

Patients undergo an electroencephalograph if none had previously been conducted and a high-resolution magnetic resonance imaging. Physicians will also use a video EEG to simultaneously observe the patient's behavior and EEG readings.

Most epileptics are treated with one or more antiepileptic agents. About two-thirds of those treated have excellent control and few side effects, Treiman says. Patients whose epilepsy is not controlled after a trial of medication are eligible for alternative treatments, or surgery, depending on age and gender.

About 80 percent of patients acquire epilepsy as a result of some form of brain insult, while the remaining 20 percent are genetically predisposed to the disorder (most genetic epilepsy is childhood onset), Treiman says. "The good news [for the 20 percent] is that these types of epilepsy are usually easy to treat," he points out.

Genetics also plays a role in the other 80 percent. "There is a genetic susceptibility that makes one person get epilepsy after a brain insult, while the next person with the same insult does not get epilepsy," Treiman notes.

One-half of patients that acquire epilepsy after a brain insult are classified as having cryptogenic epilepsy, because the insult is never identified, Treiman says.

Research directions

BNI conducts basic and clinical research in seven research laboratories, where researchers focuse on better treatments and studying the fundamental mechanisms underlying seizures. At any given time, BNI is engaged in 15 adult and 5 pediatric ongoing clinical studies that mostly focus on antiepileptic drugs.

Treiman describes most breakthroughs from BNI's research as incremental.

For years, Treiman has been interested in the treatment of status epilepticus, recurrent epileptic seizures without recovery between them; for example, a patient is rendered unconscious by a seizure and has another seizure before awakening. "That's bad news, because there is a cumulative effect on the brain from each seizure, and it makes it harder to stop further seizures," Treiman says.

Treiman has identified a series of EEG changes that are markers of the severity of an episode of status eplilepticus. Most of the research has been conducted using animal models but there is some data from human studies, he says. "There is a sequence of five predictable EEG changes during the course of an episode of status epilepticus," Treiman points out. "This is a better marker of the severity of status epilepticus, rather than duration. It is an important contribution to our understanding."Jong Rho, MD, associate director of the Division of Child Neurology and director of Pediatric Epilepsy Research, is conducting a study into the underlying biochemistry of the ketogenic diet on catastrophic epilepsies-designated as "catastrophic" because the seizures are difficult to control-of childhood. The high-fat, no carbohydrate diet has been effective for some of these children. "It's a hard diet to do, because one cookie and you blow it," Treiman says. "It's useful with small children."

Treiman says most patients with epilepsy can live normal lives. BNI has numerous patients who are now completely seizure-free, either through skilled use of antiepileptic drugs, or by surgically removing the seizure focus from the brain. "At the BNI Epilepsy Program having a large number of clinicians and also a large amount of lab activity provides a critical mass that allows us to do both excellent patient care and high level research," Treiman says.