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William Gaillard

Putting childhood epilepsy in the spotlight at American Epilepsy Society Meeting

William Gaillard

“We aim to build the evidence base for treatments that are effective specifically for children with epilepsy,” says William D. Gaillard, M.D., chief of Child Neurology, Epilepsy and Neurophysiology, and director of the Comprehensive Pediatric Epilepsy Program.

While epilepsy affects people of all ages, the unique way it manifests in infants, children and adolescents can be attributed in part to the complexities of the growing and developing brain. Researchers from the Children’s National Comprehensive Pediatric Epilepsy Program brought their expertise on the challenges of understanding and treating epilepsy in children to the recent American Epilepsy Society Annual Meeting, the largest professional gathering on epilepsy in the world.

“We aim to build the evidence base for treatments that are effective specifically for children with epilepsy,” says William D. Gaillard, M.D., chief of Child Neurology, Epilepsy and Neurophysiology, and director of the Comprehensive Pediatric Epilepsy Program. “We have learned much from studies in adult populations but technologies like functional MRI allow us to get in-depth understanding, often in non-invasive ways, of precisely how epilepsy is impacting a child.”

Dr. Gaillard was also recently elected to serve as the Second Vice President of the American Epilepsy Society. “The AES is the largest multidisciplinary professional and scientific society dedicated to the understanding, treatment and eradication of epilepsy and associated disorders, and I am honored to serve as the new Second Vice President,” he said.

The team’s presentations and poster sessions focused on several key areas in pediatric epilepsy:

Better ways to see, measure and quantify activity and changes in the brain for children with epilepsy before, during and after surgery

  • Novel applications of fMRI for children with epilepsy
    • Evaluation of an fMRI tool that tracks verbal and visual memory in children with epilepsy – one of the first to capture memory functions in this population of children using noninvasive fMRI;
    • Early study of the use of “resting-state” fMRI to map language skills before epilepsy surgery – an important first step toward noninvasively evaluating children who are too young or neurologically impaired to follow tasks in traditional MRI studies;
  • A study of whether intraoperative MRI, i.e. imaging during neurosurgery, allows for more complete removal of abnormal brain tissue associated with focal cortical dysplasia in children, which is a common cause of intractable epilepsy;
  • A preliminary case review of existing data to see if arterial spin labeling MRI, which measures blood flow to the brain, has potential to identify blood flow changes in specific locations of the brain where seizures occur;
  • An analysis of language laterality – the dominant side of the brain controlling language –  questioning the true reasons that the brains of children with epilepsy have differences in the hemisphere that predominantly controls language;
  • A review of some common assessments of language and working memory that are used pre- and post-operatively to gauge the impacts of pediatric epilepsy surgery. The study found that using multiple assessments, and studying results individually rather than as a group average, resulted in a more complete picture of the outcomes of surgery on these areas of brain function;
  • A preliminary study examining whether continuous EEG monitoring of neonates with hypoxic ischemic encephalopathy, or lack of oxygen to the brain, can be a reliable predictor of neurodevelopmental outcomes while the infant is undergoing therapeutic hypothermia.

“In order to expand our understanding of causes, impacts and outcomes, the range of research is broad given the complexity of epilepsym,” says Madison M. Berl, Ph.D. “This is the only way we can contribute to the goal of providing our colleagues and the families they serve with better resources to make informed decisions about how best to assess and treat pediatric epilepsy.”

The molecular, genetic and biological factors that contribute to onset and severity of pediatric epilepsy

  • A retrospective study of young patients with malformations in cortical development that are important causes of childhood epilepsy;
  • Investigation of a simple saliva test to effectively identify the presence of two common viral infections, human herpesvirus-6B and Epstein-Barr virus, that may be contributors to onset of epilepsy in otherwise normally functioning brains;
  • A preliminary review of the possible relationship between febrile infection-related epilepsy syndrome and the co-occurrence of another neuro-inflammatory condition – hemophagocytic lymphohistiocytosis.

Madison Berl, Ph.D., director of research in the Division of Pediatric Neuropsychology, and a pediatric neuropsychologist in the Comprehensive Pediatric Epilepsy Program, adds, “In order to expand our understanding of causes, impacts and outcomes, the range of research is broad given the complexity of epilepsy. This is the only way we can contribute to the goal of providing our colleagues and the families they serve with better resources to make informed decisions about how best to assess and treat pediatric epilepsy.”

Expanding awareness of SUDEP

Madison Berl

Madison M. Berl, Ph.D., is helping to expand awareness of SUDEP among patients, families and caregivers.

When 4-year-old Henry Lapham died in his sleep just weeks after being diagnosed with epilepsy in 2009, it was a shock to everyone — even his pediatrician and neurologist. Henry’s cause of death was sudden unexpected (or unexplained) death in epilepsy persons (SUDEP), a condition that causes sudden death in about 1 of every 1,000 otherwise healthy patients with epilepsy. Neither health care professional had mentioned this as a possibility, as remote as it was.

“I was desperate to make sense out of our tragedy,” writes Henry’s mother, Gardiner Lapham, R.N., M.P.H., in “Increasing awareness of sudden death in pediatric epilepsy together,” an article published in the February 2017 issue of Pediatrics. After her son’s death, by working with a group called Citizens United for Epilepsy Research, Lapham connected with other families affected by the same heartbreak. “I have met many bereaved family members,” she adds, “and the most consistent thing I hear is that they wish they had known about SUDEP.”

Now, a new collaboration with Children’s National Health System, where Henry received care, University of Virginia Medical Center (UVA) and other academic medical centers is helping to expand awareness of SUDEP among patients, families and caregivers alike. Known as Childhood Epilepsy Risks and Impact on Outcomes (CHERIO), the multiyear effort aims to develop approaches to increase knowledge about SUDEP and other conditions that can accompany epilepsy, such as attention deficit hyperactivity disorder, autism, anxiety, depression and sleep issues, according to co-authors of the Pediatrics article.

CHERIO got its start in 2014 at the American Epilepsy Society annual meeting. There, Lapham met Madison M. Berl, Ph.D., director of research, Division of Pediatric Neuropsychology at Children’s National, who studies epilepsy comorbidities. When Lapham asked what she could do to help raise awareness of SUDEP at Children’s National, she and Berl, along with William Davis Gaillard, M.D., Henry’s neurologist, hatched a plan.

Working with multiple disciplines and stakeholders, including neuropsychologists, psychiatrists, neurologists, epidemiologists, basic scientists, nurses and parent advocates at both Children’s National and UVA, CHERIO plans to assess the level of knowledge about SUDEP and other epilepsy comorbidities among medical providers and parents and to implement ways to increase knowledge. The first item on the agenda, Berl explains, was to conduct a survey to see just how much doctors knew about SUDEP.

“Although many neurologists are aware of this condition, ours was the first to survey pediatricians, and the majority was not aware of SUDEP – despite having children with epilepsy in their practice,” Dr. Gaillard says. “We know that many neurologists do not discuss SUDEP with patients and the reasons for not talking about SUDEP are varied. Thus, CHERIO felt that in addition to educating neurologists about the need to discuss the risk of death associated with epilepsy, increasing pediatricians’ awareness of SUDEP is one approach that could open more opportunities for families to have this discussion.”

To help make it easier to talk about this risk, the CHERIO team is developing strategies for doctors to start the conversation with patients and their families by framing SUDEP in the context of more common epilepsy comorbidities.

“Clinicians walk a fine line in giving information at the right time to make people more aware,” Berl adds, “but also being realistic and giving information that fits with what’s going on in a particular child’s case. By discussing SUDEP along with other, more common epilepsy risks, it brings context to a family so that they’re not unduly concerned about death – which also can paralyze a family and create unnecessary alarm.” The risk of death in most children with epilepsy is very low, slightly higher than the risks faced by healthy children. But parents of children with complicated epilepsy who have more risk factors for sudden death should be especially aware , she says.

Another way to help facilitate discussion may be through a simple tweak in the medical record, Berl adds. The team is currently developing a checklist that pops up annually in a patient’s medical record to remind clinicians of important points to discuss with patients and their families, including SUDEP.

Additionally, they are working on ways that can help families become more empowered to start the discussion themselves. Materials for the waiting room or questionnaires to fill out before appointments could trigger conversations with care providers, Berl says.

Last, the team also is collaborating with a medical device company that is working on a nighttime monitoring system that could provide an alert if patients with epilepsy experience nighttime seizures, a risk factor for SUDEP. Such technologies have not been proven to prevent SUDEP. Yet, it may help caregivers get help more quickly than if they did not receive the alert.

For each of these efforts, Berl notes, having Lapham as a partner has been key. “She’s part of our meetings and has input into the direction of each project,” Berl explains. “When you have a partner who is so close to the daily work you’re doing, it just heightens those efforts and brings to the forefront the simple message of why this is important.”