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Epilepsy Infographic

By the Numbers: Comprehensive Pediatric Epilepsy Program

Children’s National Health System’s Comprehensive Pediatric Epilepsy Program is one of the largest and most experienced multidisciplinary epilepsy programs in the country. With a range of programs specializing in new onset epilepsy, the Ketogenic diet, intractable epilepsy, neuroinflammation, neurogenetics, epilepsy surgery and more. The epilepsy program at Children’s National is continuously working to improve care for patients through clinical innovation, active studies and utilizing the most advanced technologies in epilepsy surgery. Children’s National has one of the best surgical outcomes in the county, aided by advanced structural and functional imaging, minimally invasive techniques, deep brain stimulation, neuronavigation, neurorobotics using the ROSA stereotactic neurosurgical robot and intraoperative MRI.

Epilepsy Infographic

neuron

Children’s National to host 29th Annual Pediatric Neurology Update

neuron

The Children’s National Health System Center for Neuroscience and Behavioral Medicine is proud to host the 29th Annual Pediatric Neurology Update course.

This year’s course will focus on three critical areas in pediatric neuroscience and neurodevelopment: epilepsy with focuses on innovations in epilepsy surgery and new therapeutics; tuberous sclerosis including neurosurgical advances and transition to adulthood; and autism spectrum disorder with emphasis on new understandings and pre-requisites for an “Autism Friendly Hospital.”

We invite you to join us for presentations from renowned experts in the field in this full-day, CME accredited event on April 11, 2019 at the Bethesda North Marriott Hotel & Conference Center in Rockville, MD.

For more information and to register, visit ChildrensNational.org/NeurologyUpdate.

Chima Oluigbo examines a patient

Eradicating epilepsy with Visualase

Chima Oluigbo examines a patient

Chima Oluigbo, M.D., and his team are using Visualase to identify and eliminate seizure foci and provide patients with a minimally invasive procedure for treating epilepsy.

About one in 26 people will be diagnosed with epilepsy in their lifetime. That adds up to about 3.4 million people in the U.S., or about 1 percent of the population nationwide. This condition can have huge consequences on quality of life, affecting whether children will learn well in school, eventually drive a car, hold down a job or even survive into adulthood.

For most of those that develop epilepsy, medications can keep seizures in check. However, for about a third of patients, this strategy doesn’t work, says Chima Oluigbo, M.D., an attending neurosurgeon at Children’s National Health System. That’s when he and his team offer a surgical fix.

Epilepsy surgery has come a long way, Dr. Oluigbo explains. When he first began practicing in the early 2000s, most surgeries were open, he says – they involved making a long incision in the scalp that can span half a foot or more. After drilling out a window of skull that can be as long as five inches, surgeons had to dig through healthy brain to find the abnormal tissue and remove it.

Each part of this “maximally invasive” procedure can be traumatic on a patient, Dr. Oluigbo says. That leads to significant pain after the procedure, extended hospital stays of at least a week followed by a long recovery. There are also significant risks for neurological complications including stroke, weakness, paralysis, speech problems and more.

However, open surgery isn’t the only option for epilepsy surgery anymore. Several new minimally invasive alternatives are now available to patients and the most promising, Dr. Oluigbo says, is called Visualase. He and his team are the only surgeons in the region who perform this procedure.

In Visualase surgeries, Dr. Oluigbo and his colleagues start by making a tiny incision, about 5 millimeters, on the scalp. Through this opening, they bore an even tinier hole into the skull and thread a needle inside that’s about 1.6 millimeters wide. “The brain barely notices that it’s there,” he says.

The tip of this wire holds a laser. Once this tip is placed directly at the seizure foci – the cluster of nerve cells responsible for generating a seizure – the patient is placed in an intraoperative magnetic resonance imaging (MRI) device. There, after checking the tip’s precise placement, the surgeons turn the laser on. Heat from the laser eradicates the foci, which the surgeons can see in real time using MRI thermography technology. The margins of the destroyed tissue are well-defined, largely sparing healthy tissue.

After the wire is removed, the incision is closed with a single stitch, and patients go home the next day. The majority of patients are seizure free, with rates as high as 90 percent for some types of epilepsy, Dr. Oluigbo says. Although seizure-free rates are also high for open procedures, he adds, Visualase spares them many of open surgeries’ painful and difficult consequences.

“Having done both open surgeries and Visualase,” Dr. Oluigbo says, “I can tell you the difference is night and day.”

Although open procedures will still be necessary for some patients with particularly large foci that are close to the surface, Dr. Oluigbo says that Visualase is ideal for treating medication-resistant cases in which the foci are buried deep within the brain. A typical example is a condition called hypothalamic hamartoma, in which tumors on the hypothalamus lead to gelastic seizures, an unusual seizure type characterized by uncontrollable laughing. He also uses Visualase for another condition called tuberous sclerosis, in which waxy growths called tubers develop in the brain, and for cancerous and benign brain tumors.

It’s gratifying to be able to help these children become seizure-free for the rest of their lives, says Dr. Oluigbo – even more so with the numerous updates he receives from families telling him how much this procedure has improved their children’s lifestyle.

“Visualase has completely changed the way that we approach these patients,” Dr. Oluigbo says. “It’s extraordinary to see the effects that this one procedure can have on the quality of life for patients here at Children’s National.”

NeuroPace RNS x-ray

New brain “pacemaker” offers new hope for refractory epilepsy

NeuroPace RNS x-ray

Example of NeuroPace RNS System placement.

If a child’s refractory seizures – seizures that don’t respond to medication – are originating in a part of the brain that is central to function (for example, impacting memory or verbal skills) the standard next step – surgical resection – is not an option for seizure reduction or relief. In most cases, these children are followed, more medications are tried, and other strategies attempted, but few viable options exist to ease their symptoms.

It’s possible that the next generation of implantable neurostimulators, which act as a type of pacemaker for the brain, might make a difference for some children previously left with no answers. Children’s National neurosurgeon Chima Oluigbo, M.D., in collaboration with the Comprehensive Pediatric Epilepsy Program at Children’s National, is looking at how these devices might be used to reduce or eliminate refractory seizures in pediatric patients. One example of this type of device is the RNS System.

“The RNS has been FDA approved for adults since 2013,” says Dr. Oluigbo, who recently implanted a NeuroPace RNS in the first pediatric patient at Children’s National, and one of the first young patients in the country. “The safety and efficacy data in the adult population, now gathered from a cohort of more than 800 adults, is showing positive outcomes so far. That allows pediatric neurosurgeons to consider an off-label use of this device for patients under the approved usage age of 18, when no other treatments exist.”

The RNS operates differently from previous neuro pacemaker-style devices. It is a “closed-loop” system that doesn’t require external activation once a seizure has started. Instead, the precise location of seizure origination is identified via functional magnetic resonance imaging (fMRI). Leads are then placed at the seizure site via surgery, and once activated, the RNS monitors and self-activates when pre-seizure electrical impulses are detected. The device responds by emitting a series of its own electrical impulses to interrupt and reset the brain’s seizure activity. The RNS system’s ability to continuously monitor the patient also allows physicians to get an inside look at the ongoing brain function of these young patients.

“Children’s National is one of the first places to apply the use of this device in children, because we are one of the few locations on the East coast with the multi-disciplinary expertise to implement it safely and effectively,” says Dr. Oluigbo. “Our clinical epilepsy team has been imaging and treating children with epilepsy for almost 30 years. With one of the oldest neurosurgical programs in country and our technological capabilities, Children’s National becomes the perfect location to explore how technology like this can improve the quality of life for our patients, many of whom have previously been told there is nothing more we can do to help.”

De-personalized data from patients who receive the NeuroPace RNS will be shared with the company in the hopes that the data will assist the FDA in assessing the appropriateness of extending the age range of approval from 18 and above to 12 and above.

“Our hope is to contribute to the body of data about this device and determine if it will improve the lives of our younger patients the way it has already been done for adults,” Dr. Oluigbo concludes. “Kids’ brains may respond differently, however, sharing our patients’ experiences and outcomes will help us identify whether or not this is a viable and promising option for more children with refractory epilepsy.”

Presidnet's Award for Innovation in Research

President’s Award highlights innovative work by early-career researchers

Presidnet's Award for Innovation in Research

As part of Research and Education Week 2018, two Presidential awardees were recognized for their research contributions, Catherine “Katie” Forster, M.D., M.S., and Nathan Anthony Smith, Ph.D.

Catherine “Katie” Forster, M.D., M.S., and Nathan Anthony Smith, Ph.D., received the President’s Award for Innovation in Research honoring their respective research efforts to explore an understudied part of the microbiome and to shed light on an underappreciated player in nerve cell communication.

Drs. Forster and Smith received their awards April 19, 2018, the penultimate day of Research and Education Week 2018, an annual celebration of the excellence in research, education, innovation and scholarship that takes place at Children’s National Health System. This year marks the fifth time the President’s Award honor has been bestowed to Children’s faculty.

Dr. Forster’s work focuses on preventing pediatric urinary tract infections (UTIs). Frequently, children diagnosed with illnesses like spina bifida have difficulty urinating on their own, and they often develop UTIs. These repeated infections are frequently treated with antibiotics which, in turn, can lead to the child developing antibiotic-resistant organisms.

“The majority of the time if you culture these children, you’ll grow something. In a healthy child, that culture would indicate a UTI,” Dr. Forster says. “Children with neurogenic bladder, however, may test positive for bacteria that simply look suspect but are not causing infection. Ultimately, we’re looking for better ways to diagnose UTI at the point of care to better personalize antibiotic treatment and limit prescriptions for children who do not truly need them.”

Powered by new sequencing techniques, a research group that includes Dr. Forster discovered that the human bladder hosts a significant microbiome, a diverse bacterial community unique to the bladder. Dr. Forster’s research will continue to characterize that microbiome to determine how that bacterial community evolves over time and whether those changes are predictable enough to intervene and prevent UTIs.

“Which genes are upregulated in Escherichia coli and the epithelium, and which genes are upregulated by both in response to each other? That can help us understand whether genes being upregulated are pathogenic,” she adds. “It’s a novel and exciting research area with significant public health implications.”

Smith’s work focuses on the role of astrocytes, specialized star-shaped glial cells, in modulating synaptic plasticity via norepinephrine. Conventional thinking describes astrocytes as support cells but, according to Smith, astrocytes are turning out to be more instrumental.

Norepinephrine, a neurotransmitter that plays an essential role in attention and focus, is released by a process known as volume transmission, which is a widespread release of a neurotransmitter at once, says Smith, a principal investigator in Children’s Center for Neuroscience Research. Astrocytes, which outnumber neurons in the brain, are strategically and anatomically located to receive this diffuse input and translate it into action to modulate neural networks.

“We hypothesize that astrocytes are integral, functional partners with norepinephrine in modulating cortical networks,” Smith adds. “Since astrocytes and norepinephrine have been implicated in many central nervous system functions, including learning and attention, it is critical to define mechanistically how astrocytes and norepinephrine work together to influence neural networks. This knowledge also will be important for the development of novel therapeutics to treat diseases such as attention deficit hyperactivity disorder and epilepsy.”

William Gaillard

William D. Gaillard, M.D., elected Second Vice President of the American Epilepsy Society

William Gaillard

William Davis Gaillard, M.D., has been elected second vice president of the American Epilepsy Society (AES), a medical and scientific society with 4,000 members. Dr. Gaillard’s term started at the end of the society’s annual meeting, December 1-5, in Washington, D.C.

“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.” Dr. Gaillard said.

Dr. Gaillard, an internationally recognized expert in pediatric epilepsy and imaging, is chief of Neurology, Epilepsy and Neurophysiology at Children’s National. He is also the associate director of the DC-IDDC and director of the of the Intellectual and Developmental Disabilities Research Center (DC-IDDRC) imaging core and associate director of the Center for Neuroscience Research, Children’s Research Institute. His academic appointments include professor of Pediatrics and Neurology at George Washington University and professor of Neurology at Georgetown University.

As division chief of Child Neurology, Epilepsy and Neurophysiology, Dr. Gaillard directs a team of pediatric specialists who see thousands of patients each year. Dr. Gaillard has worked throughout his career to care for children and young adults with epilepsy from the onset of seizures through novel therapeutic interventions, medication trials and, when appropriate, surgery. Treatment at Children’s National addresses the full range of the condition, including problems of difficult-to-control epilepsy. Additionally, treatment includes the concurrent social, educational and emotional issues faced by children with the condition and their families.

An active participant in AES activities, Dr. Gaillard has served as treasurer and as chair of the Clinical Investigator Workshop and Pediatric Content Committees. He also serves as an associate editor for the journal Epilepsy Research, and as a regular reviewer on AES and Epilepsy Foundation study sections. Dr. Gaillard will service as first vice president in 2019 and accede to the presidency of AES in 2020.

Surprising consensus on pediatric anti-epilepsy meds

A study that includes William D. Gaillard, M.D., among its authors indicates that U.S. doctors appear to have reached an unexpected consensus about which anti-seizure medicine to prescribe to their pediatric patients.

The number of available anti-seizure medications has exploded in the past two decades, going from just a handful of medicines available in the 1990s to more than 20 now. Once the Food and Drug Administration (FDA) approves each new medicine based on trials in adults, it’s available for clinicians to prescribe off-label to all age groups. However, says William D. Gaillard, M.D., division chief of Child Neurology and Epilepsy, Neurophysiology and Critical Care Neurology at Children’s National Health System, trials that lead to FDA approval for adults do not provide any information about which medications are best for children.

“With so many medications and so little data,” Dr. Gaillard says, “one might think doctors would choose a wider variety of medicines when they prescribe to children with epilepsy.”

However, the results from a recent study that included Dr. Gaillard and colleagues, published online in Pediatric Neurology on June 27, 2017, show otherwise. The study indicates that doctors in the United States appear to have reached an unexpected consensus about which medication to prescribe for their pediatric patients.

The study is part of a broader effort to collect data on the youngest epilepsy patients – those younger than 3 years old, the age at which epilepsy most often becomes evident. As part of this endeavor, researchers from 17 U.S. pediatric epilepsy centers enrolled in the study 495 children younger than 36 months old who had been newly diagnosed with non-syndromic epilepsy (a condition not linked to any of the commonly recognized genetic epilepsy syndromes).

The researchers mined these patients’ electronic medical records for information about their demographics, disease and treatments. About half of the study participants were younger than 1 year old when they were diagnosed with epilepsy. About half had disease marked by focal features, meaning that their epilepsy appeared to originate from a particular place in the brain. Nearly all were treated with a single medication, as opposed to a cocktail of multiple medicines.

William Gaillard

“This study identifies current practices, but whether those practices are correct is a separate question,” explains Dr. Gaillard. “Just because a medication is used commonly doesn’t mean it is the best medication we should be using.”

Of those treated with a single medication, nearly all were treated with one of five medicines: Levetiracetam, oxcarbazepine, phenobarbital, topiramate and zonisamide. However, the data showed a clear prescribing preference. About 63 percent of the patients were prescribed levetiracetam as a first choice. By contrast, oxcarbazepine and phenobarbital, the next most frequently prescribed medicines, were taken by patients as a first choice by a mere 14 percent and 13 percent respectively.

Even more striking, of the children who were not prescribed levetiracetam initially but required a second medication due to inadequate efficacy or unacceptable side effects, 62 percent also received this medication. That made levetiracetam the first or second choice for about 74 percent of all the children in the study, despite the availability of more than 20 anti-seizure medications.

It’s not clear why levetiracetam is such a frequent choice in the United States, says Dr. Gaillard. However, in its favor, the drug is available in a liquid formulation, causes no ill effects medically and can be started intravenously if necessary. Studies have shown that it appears to be effective in controlling seizures in about 40 percent of infants.

Yet, levetiracetam’s market dominance appears to be a North American phenomenon, the study authors write. A recent international survey that Dr. Gaillard also participated in suggests that outside of this continent, carbazepine and oxcarbazepine were the most frequently prescribed medications to treat focal seizures.

What’s really necessary, Dr. Gaillard says, is real data on efficacy for each of the medications commonly prescribed to pediatric epilepsy patients – a marked vacuum in research that prevents doctors from using evidence-based reasoning when making medication choices.

“This study identifies current practices, but whether those practices are correct is a separate question,” he explains. “Just because a medication is used commonly doesn’t mean it is the best medication we should be using.”

To answer that question, he says, researchers will need to perform a head-to-head clinical trial comparing the top available epilepsy medications in children. This study sets the stage for such a trial by identifying which medications should be included.

“Uncontrolled pediatric epilepsy can have serious consequences, from potential problems in development to a higher risk of death,” Dr. Gaillard says. “You want to use the optimal medicine to treat the disease.”

Oluigbo and Myseros neurosurgery

Working miracles to control seizures and preserve brain power in newborns

Oluigbo and Myseros neurosurgery

In the spring of 2017, a multidisciplinary team applied an innovative approach to help preserve function in the working right hemisphere of a baby who experienced her first seizure hours after birth.

When orderly early fetal brain development is disturbed in one half of the brain, infants can be born with hemimegalencephaly—a rare occurrence—that results in one of the brain’s two hemispheres being oversized, heavy and malformed. This brain malformation arises early in the fetal period of life, is not inherited and is associated with seizures early in life.

Children with hemimegalencephaly can develop horrible seizures within the first hours or days of life. According to published research, every month these infants experience uncontrolled seizures correlates to a steep decline in IQ.

Because these types of seizures do not respond to multiple anti-seizure medications—medicines which may also cause worrisome side effects of their own in neonates—care teams attempt to schedule surgery as soon as feasible to remove or disconnect the hemisphere triggering the damaging seizures. “The ‘bad’ brain does not sustain any function and it interferes with the ‘good’ brain doing what it needs to do,” says William D. Gaillard, M.D., chief of Children’s division of Epilepsy and Neurophysiology and chief of Neurology.

Hemispherectomy is intricate surgery on an organ that is softer than normal and crisscrossed with a tangle of blood vessels that supply the damaged hemisphere with blood. Because of the risks of life-threatening blood loss in very young infants, the dramatic surgery is usually not performed until babies are at least 3 months old and weigh at least 10 pounds.

The challenge: The vulnerable babies who most need relief, infants who have been seizing since early life, are too young for the operation.

Neurosurgeons have clamped the carotid artery that supplies blood to the brain to minimize blood loss when the hemisphere is surgically removed. Dr. Gaillard says knowledge of that approach led the team to think: What if we use embolization—blocking blood supply to targeted locations in the brain—to achieve the same effect?  The plan effectively destroys the malformed brain from within, neutralizing its ability to cause the seizures.

“It was eye-opening for us to think about actually inflicting brain injury as a way of treating something in the brain that was causing seizures. That is really novel in itself: We’re thinking out of the box in applying existing techniques in a different age group. The conventional thinking with newborns is to let them be; their seizures don’t look that bad,” says Taeun Chang, M.D., director of Children’s Neonatal Neurology and Neonatal Neurocritical Care Program.

“We have evidence to suggest this is a safe and effective way of avoiding recurrent seizures and minimizing the need to give these infants potentially toxic medications so early in life. Ultimately, this helps a select group of babies who need the surgery to get to the point of being old enough to have it—all the while, sparing the healthy part of their brain,” Dr. Gaillard adds.

Darcy hemimegalencephaly

Once the embolization ended Darcy’s most severe seizures, the little girl could make eye contact, started smiling, and then graduated from smiling to full laughs. In weekly physical therapy, the infant works on tummy time, head control and ensuring her eyes track.

In the spring of 2017, the multidisciplinary team applied the innovative approach to help preserve function in the working right hemisphere of a baby named Darcy Murphy. Darcy experienced her first seizure hours after she was born, and when she arrived at Children’s National had been in and out of two different emergency rooms in another state for the first few weeks of her life.

The team explained to the Murphy family that Darcy was on multiple medications, but her seizures continued unabated. The options included inducing a coma, sending Darcy home despite ongoing seizures or minimally invasive embolization.

“We would not have even posed this if we were not confident in our ability to do the procedure and deal with potential complications,” Dr. Chang says.

“Oh my gosh, as a parent you know what you’re doing is permanent,” says Rachel Murphy, 29, Darcy’s mom said of the decisions that she and husband Ryan, 33, faced for the youngest of their three children. “What if it’s not the right decision? What if in a week they come out with a new procedure you could have done? We were horrified all the time. The nice part with this procedure is the reward is apparent very quickly, and it just gets better. You don’t have to wait two years to know you made the right decision. You can see half a brain is better than the whole thing for this specific child.”

Once the embolization ended Darcy’s most severe seizures, the little girl could initiate and maintain eye contact with family members, started smiling and then graduated from smiling to full laughs. In weekly physical therapy, the infant works on tummy time, head control and ensuring her eyes track.

Children’s multidisciplinary care team includes experts in newborn intensive care (neonatologists) to aggressively manage seizures in the traditional fashion as they occur and to monitor vital signs; a neonatal neurologist/neurointensivist at the bedside and in the Angio suite monitoring Darcy’s brain activity; a neonatal epileptologist; a surgical epilepsy team; an interventional neuroradiologist; neurosurgeons to perform the delicate functional hemispherectomy to remove any residual brain tissue from the bad hemisphere; and physical therapists working to help Darcy achieve maximum function after surgery.

“We were just like one unit in the sense of being able to provide coherent, comprehensive care. It’s about blood pressure management, breathing, electrolytes, making sure everything is right for going to the operating room,” Dr. Chang explains. “Darcy’s case highlights the ways in which Children’s National is different and offers personalized care that is superior to other centers.”

The team, which recently published a case report of two previous serial embolizations followed by hemispherectomy, plans follow-up papers describing EEG manifestations during an acute stroke in a newborn, advice to the field on best practices for the embolization and using cooling to control the planned brain injury during embolization hemispherectomy.

Revised Nov. 7, 2017

Related resources

Chima Oluigbo

A novel way to treat intractable epilepsy caused by hemimegalencephaly

Chima Oluigbo

A multidisciplinary team led by Chima Oluigbo, M.D., F.R.C.S.C., pioneered a novel technique to preserve newborns’ healthy brain tissue, buying time until the infants became old enough to undergo a hemispherectomy.

What’s known

Hemimegalencephaly is an extremely rare birth defect in which one side of the brain grows larger than the other. This anomaly typically leads to severe, recurrent seizures that can be difficult to control solely with medications. While the seizures themselves are detrimental to the developing brain, the amount of medications used to reduce seizure frequency often come with significant side effects and have the potential to hamper brain growth. Hemispherectomy, a radical surgery in which one half of the brain is removed, is often the most successful way to treat severe and intractable epilepsy. However, this surgery can be challenging to perform successfully in very young babies.

What’s new

In this case report, the Children’s National Health System Epilepsy Team led by Chima Oluigbo, M.D., F.R.C.S.C., a pediatric neurosurgeon; Tammy N. Tsuchida, M.D., PhD., a pediatric surgical epileptologist; Monica Pearl, M.D., a pediatric interventional neuroradiologist; Taeun Chang, M.D., a neonatal neurointensivist; and the neonatal intensive care team explored the possibility of using minimally invasive surgery to cut off the blood supply to the brain hemisphere responsible for generating seizures in newborns with hemimegalencephaly. This procedure, they reasoned, could buy time for babies to mature and become more resilient to withstand the future hemispherectomy while also lessening the damage caused by uncontrolled, recurrent seizures. The case report focused on the first two patients with hemimegalencephaly who had sequential procedures to gradually restrict blood flow to the affected brain hemisphere within their first few weeks of life, followed by hemispherectomies at a few months of age. This novel approach significantly lessened their seizures until hemispherectomy, allowing these children to continue to grow and develop seizure-free.

Questions for future research

Q: Which patients are best suited for this surgical procedure?
Q: How can surgeons reduce the risk of excessive blood loss during hemispherectomy caused by the growth of additional blood vessels after flow through the brain’s major vessels has been blocked?
Q: What are the long-term outcomes for infants who undergo these procedures?

Source: “ ‘Endovascular embolic hemispherectomy’: A strategy for the initial management of catastrophic holohemispheric epilepsy in the neonate.” Oluigbo, C., M.S. Pearl, T.N. Tsuchida, T. Chang, C.-Y. Ho and W. D. Gaillard. Published by Child’s Nervous System October 29, 2016.

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.”

Chima Oluigbo

The benefits of deep brain stimulation for pediatric patients

Chima Oluigbo

There was no effective treatment for uncontrolled, difficult, and sometime painful movements associated with movement disorders. That is, before the development of deep brain stimulation (DBS) techniques.

Children’s National Health System is one of only two children’s hospitals with fully integrated DBS programs. Chima Oluigbo, M.D., who leads the pioneering Deep Brain Stimulation Program within Children’s Division of Neurosurgery, is one of few pediatric deep brain stimulation experts in North America and cross-trained in pediatric and functional neurosurgery.

Dr. Oluigbo says the effects of DBS are often dramatic: 90 percent of children with primary dystonia show up to 90 percent symptom improvement.

A 6-year-old boy with dystonia so severe that his body curved like a “C” was one of the first patients to undergo the procedure at Children’s National. Six weeks later, he gained the ability to sit straight and to control his hands and legs. He also was able to smile, an improvement that brought particular joy to his parents.

Inside the brain with movement disorders

Patients with movement disorders experience difficulties due to neurological dysfunction that impact the speed, fluency, quality, and ease in which they move. In these cases, neurons in the brain’s motor circuits misfire. Through the use of DBS, neurosurgeons can synchronize neuronal firing and accomplish the previously impossible: restoring muscle control to patients with these disorders.

Movement disorders are common in children. “It’s not just numbers, it’s also about impact. Think about the potential of a child who is very intelligent and can contribute to society. When that child is not able to contribute because he or she is disabled by a movement disorder, the lost potential is very significant. It has an impact,” Dr. Oluigbo says.

What is deep brain stimulation?

DBS uses an implantable device to send continuous, low-level electrical impulses to areas deep within the brain. The impulses prevent the brain from firing abnormal signals that are linked to movement disorders and seizures. When a child is considered to be a candidate for the technique, here’s what happens next:

  1. Imaging: Magnetic resonance imaging (MRI) helps pinpoint the area of brain tissue responsible for movement disorders and informs the treatment plans.
  2. Neurotransmitter implant procedure: Using minimally invasive neurosurgery techniques, doctors access the brain through a tiny incision in the child’s skull and place thin, insulated wires (leads) in the area of brain tissue responsible for the condition.
  3. Pulse generator implant procedure: The pulse generator (neurostimulator) is a battery-operated device that sends low-level electrical impulses to the leads. During a separate procedure, the pulse generator is implanted near the child’s collarbone. Leads are threaded under the child’s skin to connect with the pulse generator.
  4. Stimulation treatments: Once the leads and pulse generator are connected, the child receives a continuous stream of electrical impulses. Impulses are generated by the neurostimulator, travel through the leads, and end up in the deep tissue of the brain. Here, they block abnormal signals that are linked to the child’s movement disorder.
  5. Follow-up care: The child will likely need deep brain stimulation throughout his or her lifetime to make sure the device is working correctly and to adjust the neurotransmitter settings to meet his or her changing needs.

Deep brain stimulation at Children’s National

Children’s National is currently conducting clinical trials seeking to expand the use of this procedure to patients with cerebral palsy, one of the most common dystonias. The effective use of deep brain stimulation requires ongoing attention from a multidisciplinary team (from neuropsychology to rehabilitation medicine), giving seamless care under one roof.

There is evidence to suggest that this technique could be used to aid people with memory disorders, patients in minimally conscious states, and patients with incurable epilepsies.