Dear colleagues:
Welcome to UT Health Austin Pediatric Neurosciences at Dell Children’s. In this issue, we highlight our epilepsy chief Dave Clarke, MD, recipient of the American Epilepsy Society’s J. Kiffin Penry Award; a useful Pediatrics in Review
article on vitamin deficiency co-authored by our clinical neurophysiology fellow; new faculty and staff; and several recent accomplishments and events.
As our program continues expanding its impact in Central Texas and beyond, we remain dedicated to offering outstanding educational opportunities, promoting research and scholarly activity, and delivering exceptional multidisciplinary clinical care for all children with neurological disorders.
Chief of Pediatric Neurosciences
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CLARKE RECEIVES PENRY AWARD FROM AMERICAN EPILEPSY SOCIETY
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Dave F. Clarke, MD, professor of neurology and pediatrics at The University of Texas at Austin’s Dell Medical School and holder of the Kozmetsky Family Endowed Chair in Pediatric Epilepsy, received the J. Kiffin Penry Award for Excellence in Epilepsy Care by the American Epilepsy Society (AES) at its recent annual meeting in Orlando, Florida. Clarke leads the Comprehensive Pediatric Epilepsy Center in UT Health Austin Pediatric Neurosciences at Dell Children’s.
Clarke
emphasizes medical and surgical management of drug-resistant epilepsy. Additionally, he has long championed efforts to broadly improve epilepsy treatment and reduce disparities in epilepsy care regionally and internationally via outreach programs in Texas, Africa, and the Caribbean island countries. He is a member of the North American Commission of the International League Against Epilepsy, the Epilepsy Leadership Council Steering Committee, the International Affairs Committee of the Child Neurology Society, and the American Epilepsy Society Advocacy Task Force, as well as secretary-treasurer of the National Association of Epilepsy Centers Board. In 2021, Clarke received the Ambassador for Epilepsy Award jointly from the International League Against Epilepsy and the International Bureau for Epilepsy in recognition of his outstanding contributions to furthering the cause of epilepsy
internationally.
The award is given each year to honor the late Dr. Penry’s lifelong commitment to improving the care of individuals with epilepsy, promoting epilepsy research, and encouraging scholarly and educational activities and mentorship. Penry was chief of the clinical epilepsy section of the National Institute of Neurological Disorders and Stroke and later associate dean for research at Wake Forest University School of Medicine. He also began the still-running, highly regarded Penry Epilepsy MiniFellowship, an immersion program that, in the last four decades, has boosted epilepsy management skills for thousands of practitioners.
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Clarke teaching electroencephalography interpretation
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A SECOND OPINION
This nine-year-old girl was evaluated following a probable seizure. The episode occurred just after awakening and consisted of bilateral twitching of the eyes, shoulders, arms, and hands followed by transient lethargy. She was unable to speak during the episode but could accurately describe conversations that took place. Two days later, she was diagnosed with influenza but recovered without complications.
During the previous year, she had experienced several morning episodes of bilateral “quivering” of her lips associated with a sense of tongue numbness. She remained fully conscious during these episodes, and no one had witnessed other periods of unresponsiveness. A neurologist suggested a diagnosis of benign rolandic epilepsy (now termed “benign epilepsy with centrotemporal spikes,” or BECTS) based on her electroencephalogram (EEG) findings. Since the episodes were brief and did not occur at school, the family opted not to begin medication.
She was an excellent student until age eight, when she began to experience lack of focus, inattentiveness, memory issues, and difficulty completing assignments. Her overall school performance deteriorated, although her day-to-day performance varied from nearly normal to very poor. Her struggles were initially attributed to attention-deficit/hyperactivity disorder, and she was treated unsuccessfully with methylphenidate. She was subsequently diagnosed with a learning disability but continued to perform poorly despite various classroom accommodations.
See below for additional discussion.
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FEATURED ARTICLE: VITAMIN-DEPENDENT GENETIC DISORDERS
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Not to be confused with simple vitamin deficiencies, vitamin-dependent disorders are genetic conditions that can be effectively treated with pharmacological doses of a vitamin. Vitamins play a critical role in neurologic, endocrinologic, psychiatric, developmental, and hematologic processes. Vitamins serve as cofactors for one or more enzymes, and for selected genetic disorders, supplementation with a key vitamin cofactor can dramatically alleviate the clinical manifestations of these conditions. Several vitamin-responsive conditions exist, and lifelong vitamin supplementation is generally the only effective therapy.
Clinical neurophysiology fellow Emily L. Ramirez, DO, mentored by geneticist James Gibson and neurogenetics program chief Kristina Julich, MD,
recently published a thorough review of the vitamin-responsive genetic disorders of childhood. Most neurologists are familiar with well-known disorders such as pyridoxine dependency and biotinidase deficiency, but Ramirez and colleagues also discuss many less well-known conditions, providing detailed information about each disorder’s clinical presentation, diagnosis, and optimal treatment.
This practical guide to a challenging but highly treatable group of genetic disorders is conveniently synthesized in a single article. Read “Vitamin-Dependent Genetic Disorders of Childhood” in Pediatrics in Review, available at https://doi.org/10.1542/pir.2022-005637.
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PEDIATRIC NEUROSCIENCES WELCOMES NEW COLLEAGUES
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UT Health Austin Pediatric Neurosciences at Dell Children’s has grown dramatically since it was founded in 2019. The multidisciplinary program now features 20 child neurologists, 20 advanced practice providers, two pediatric neurosurgeons, two pediatric physical medicine and rehabilitation specialists, eight pediatric neuropsychologists, and a pediatric neuro-ophthalmologist. We recently welcomed new colleagues to the program.
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Kylene Willsey, MD, an assistant professor in the departments of Neurology and Pediatrics, joins our team from Green Tree Pediatrics in Ann Arbor, Michigan, where she practiced as a general pediatrician. She works primarily with the Dell Children’s Traumatic Brain Injury/Concussion Clinic.
Willsey received her medical degree from Baylor College of Medicine in Houston and completed her pediatrics residency at the University of Michigan in Ann Arbor. She has been involved in the community through various sports-related initiatives, including coaching, providing sports coverage for high school football games, and performing baseline concussion testing. She is a member of the American Academy of Pediatrics, the Council on Sports Medicine and Fitness, and the American Medical Society for Sports Medicine.
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Rachel Bridges, PhD,
joins our team as a pediatric neuropsychologist and an assistant professor of neurology. She specializes in neuropsychological evaluations for patients with complex medical and neurological disorders, including brain and spine cancer, leukemia, sickle cell disease, and rare genetic/degenerative disorders. She also has expertise in the assessment of oncology/hematology patients with stroke, posterior fossa syndrome, and other neurological injuries post-neurosurgical intervention.
Bridges earned her master’s degree and her doctorate in school psychology with a concentration in neuropsychology from the University of South Carolina. She completed a predoctoral internship in pediatric neuropsychology at Mt. Washington Pediatric Hospital of Johns Hopkins Medicine and the University of Maryland Medical System, followed by a two-year postdoctoral fellowship at St. Jude Children’s Research Hospital.
Bridges’ research focuses on outcomes following childhood cancer treatment, neurocognitive monitoring during CNS-directed therapy, and assessment measures for the neurocognitive screening of patients with cancer. She is a member of the American Academy of Clinical Neuropsychology, the International Neuropsychological Society oncology special interest group, and the National Academy of Neuropsychology.
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Molly McVey, MSN, FNP-C, is a licensed family nurse practitioner in the pediatric rehabilitation medicine program. She received her Bachelor of Science in nursing from The Ohio State University and a Master of Science in nursing, family nurse practitioner, from The University of Texas at Austin. Previously, she was a neonatal intensive care unit nurse at Dell Children’s Medical Center, Nicklaus Children’s Hospital, and Ann & Robert H. Lurie Children’s Hospital. She also has experience as a NICU travel nurse. She is a member of the National Academy of Dermatology Nurse Practitioners, the Dermatology Nurses’ Association, Texas Nurse Practitioners, and Austin Advanced Practice Nurses.
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We’re also excited to welcome Tanner Britton, our digital documentarian and academic content creator. Tanner previously worked as the multimedia producer for the Texas Comptroller of Public Accounts in Austin. He received a Bachelor of Music in business and performance from McNally Smith College of Music and has experience as a filmmaker, photographer, and videographer for commercials, documentaries, music videos, and events throughout the country.
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Hardy Represents Medical Community in Olympic Trials
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Congratulations to Duriel Hardy, MD, pediatric neuroimmunologist and assistant professor of neurology, for qualifying for the 2024 U.S. Olympic Team Trials in the marathon category!
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Duriel Hardy, MD, training for the Olympic trials
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Freedman Appointed Vice Chair of American Epilepsy Society Committee
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Daniel Freedman, DO, assistant professor of neurology and director of the Psychogenic Nonepileptic Events Clinic,
was named vice chair of the Guidelines and Assessment Committee for the American Epilepsy Society. He will serve a three-year term as vice chair and a three-year term as chair.
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Austin Appointed Chief of Pediatric Neuropsychology
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Cynthia Austin, PhD, ABPP,
assistant professor of neurology and neuropsychologist in the Pediatric Neuropsychology Clinic and the Dell Children’s Traumatic Brain Injury/Concussion Clinic, was appointed chief of the nine-member division of pediatric neuropsychology in October. Austin earned her master’s in program evaluation and her doctorate in school psychology from The University of Texas at Austin. She completed an internship in pediatric neuropsychology at the University of Minnesota Medical School and a fellowship in pediatric neuropsychology from Kennedy Krieger Institute/Johns Hopkins School of Medicine. Her research interests include parent experience and satisfaction with the neuropsychological assessment process as well as predictors and outcomes of traumatic brain injury. Congratulations!
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Clarke Honored as Kozmetsky Family Endowed Chair
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Dave F. Clarke, MD, is the inaugural recipient of the Kozmetsky Family Endowed Chair in Pediatric Epilepsy in recognition of his years of leadership, service, and research in the field of epilepsy. The position is funded by the Kozmetsky Family Foundation, a philanthropic investor whose trustees established it after their son benefited directly from Clarke’s care. This is the first endowed chair within our program.
Read the official announcement.
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Edmond Begins Term as President of the American Academy of Ophthalmology
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Pediatric neuro-ophthalmologist Jane Edmond, MD, is president
of the American Academy of Ophthalmology in 2024. Thank you for your leadership!
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Pediatric Psychology Internship Program Awarded Highest Level of APA Reaccreditation
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The Dell Medical School/Dell Children’s Medical Center Psychology Internship program was awarded reaccreditation for 10 years by the American Psychological Association. The internship is housed in the Department of Psychiatry and Behavioral Sciences and is co-directed by Sasha Jaquez, PhD, Ankita Krishnan, PhD, and pediatric neuropsychologist
Amanda Winter-Greenberg, PhD, ABPP, an assistant professor of neurology who supervises the internship program’s neuropsychology rotation. The accreditation is the highest level awarded by the APA and signifies that the program adheres to the Standards of Accreditation for Health Service Psychology.
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Roach Inducted into Shine Academy of Health Science Education
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E. Steve Roach, MD, has been elected to membership in The University of Texas System Kenneth I. Shine, M.D., Academy of Health Science Education.
Roach was nominated for Shine Academy membership by Dell Medical School Dean Claudia Lucchinetti, MD, who cited his innovative biomedical writing skills workshop, numerous medical textbooks, clinical teaching awards, resident curriculum development projects, and years of service as a medical journal editor.
“Dr. Roach has inspired generations of students, trainees, and faculty through his rigorous teaching and mentorship,” said David Paydarfar, MD, professor and chair of the Department of Neurology at Dell Medical School. “He is a role model in medical education, teaching others as a master clinician and medical scholar, skillfully translating knowledge to clinical practice.”
Named for former Institute of Medicine President Kenneth I. Shine, MD, the academy promotes excellence in health science education by recognizing outstanding educators and advancing knowledge and innovation in medical education through project grants. Each year the academy inducts a limited number of outstanding health science educators and leaders from the eight UT System health care institutions. Nominations for membership may come from a university president, dean, or faculty senate at any of the system’s health care facilities.
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Four Faculty Members Named Castle Connolly 2024 Top Doctors
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Elizabeth Tyler-Kabara, MD, PhD
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We are proud to announce that four of our pediatric neuroscience providers were named Castle Connolly Top Doctors: Jane Edmond, MD; Elizabeth Tyler-Kabara, MD, PhD;
Louisa Keith, MD; and E. Steve Roach, MD.
Keith was also named a Castle Connolly Rising Star. Castle Connolly Top Doctors represent the top 7% of all U.S. practicing physicians and are peer nominated through surveys sent out to tens of thousands of doctors. Those results are rigorously vetted by a research team of Castle Connolly physicians based on several factors.
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Faculty Members Receive Dell Med Educational & Research Awards
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Several pediatric neurosciences faculty members were recently presented with awards at the annual Dell Med Educational Innovation, Research and Awards Symposium: Rosalia Costello, PsyD (Dell Med Teaching Health Equity Award) and
Kristen Arredondo, MD; Glendaliz Bosques, MD;
Audrey Brumback, MD, PhD; Daniel Freedman, DO;
Duriel Hardy, MD; Sara Pavitt, MD; and
Veda Vedanarayanan, MD (GME Clinical Learning Environment Awards).
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PEDIATRIC NEUROSCIENCES CO-SPONSORS SECOND ANNUAL NF FAMILY DAY
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NF Family Day presenters (L to R) Sasha Jaquez, PhD; Kendra Koch, PhD; Virginia Harrod, MD, PhD; Sage T. Green, DO; and Manikum Moodley, MD
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The second annual Neurofibromatosis Family Day Meeting was held October 28 at Dell Children’s. Co-sponsored by the Dell Children’s Neurofibromatosis Center of Excellence, the pediatric neurosciences program, and the Children’s Tumor Foundation, this meeting allowed patient families affected by NF to learn the latest developments in clinical care and research through presentations and breakout sessions. Pediatric neuroscience presenters included Manikum Moodley, MD,
professor of neurology and co-director of the Neurofibromatosis Center of Excellence; clinical research administrator Kendra Koch, PhD; and neurofibromatosis center coordinator Karla Robles-Lopez, MD, PhD.
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CLARKE ADVOCATES AT NATIONAL EPILEPSY CONGRESSIONAL BRIEFING
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Clarke presenting on disparities in epilepsy care and access
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Clarke was also recently named a Dell Med Visionary. Nominated and selected by their peer leaders, Dell Med Visionaries recognize faculty leaders who are bringing Austin into focus as a premier destination for integrated, patient-centered health care.
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WELLONS PRESENTS AT SECOND ANNUAL TIMOTHY M. GEORGE MEMORIAL SYMPOSIUM
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Jay Wellons, MD, MSPH, presenting keynote lecture
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John (Jay) Wellons III, MD, MSPH,
professor of neurological surgery and pediatrics and chief of pediatric neurological surgery at Vanderbilt University Medical Center, presented at the second annual Timothy M. George, MD, Memorial Lecture Research Symposium: Outcomes in Pediatric Neuroscience at Dell Children’s on November 10. Wellons, a past pediatric neurosurgery mentee of George, discussed the state of fetal surgery for spina bifida after the Management of Myelomeningocele Study. This year’s lectureship was expanded to create a mini symposium with other speakers, including pediatric neuroscience faculty members M. Omar Iqbal, MD;
Elizabeth Tyler-Kabara, MD, PhD; and Dave F. Clarke, MD.
Timothy M. George, MD, was a professor of neurosurgery at Dell Medical School and a beloved pediatric neurosurgeon at Dell Children’s when he died unexpectedly at age 59 in 2019. His quiet confidence and unfailing empathy and compassion endeared him to colleagues and patients alike.
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A SECOND OPINION: EPILOGUE
The
child’s second EEG revealed sleep-activated right central-temporal spikes and slow waves, findings characteristic of BECTS. These seizures typically occur during sleep, during transition to wakefulness, or during wakefulness following sleep loss. The facial muscles are most often affected, but secondary generalization can occur. Many wakeful patients have difficulty speaking during the seizures despite preservation of awareness. Children with BECTS usually have a favorable long-term prognosis, with resolution of seizures by mid-adolescence. However, a substantial number of the children exhibit cognitive and behavioral changes, leading some physicians to shift the characterization of BECTS from “benign” to “self-limited” (thus the term “self-limited epilepsy with centrotemporal spikes”). Medication is typically started if the seizures become frequent, undergo generalization, or lead to
encephalopathy.
The deterioration of this child’s school performance was alarming. Although the formal diagnosis of attention-deficit/hyperactivity disorder and learning disability is often delayed, neither is likely to appear de novo in a previously high-performing eight-year-old. Acquired learning difficulty is common in children with poorly controlled epilepsy, particularly with the epilepsy syndromes with sleep activation. Up to 20% of children with BECTS develop epileptic encephalopathy. Deteriorating school performance could also stem from genetic metabolic disorders, toxic exposures, depression, or psychosocial dysfunction. Fortunately, this child’s cognitive dysfunction quickly resolved after she began taking an antiseizure drug.
Final Diagnosis
1. BECTS
2. Epileptic encephalopathy associated with BECTS, resolved on medication
Additional Reading
Porat RA, Kramer U, Hausman KM, et al. Early prediction of encephalopathic transformation in children with benign epilepsy with centro-temporal spikes. Brain Dev. 2021;43(2):268-279.
Van Bogaert P. Epilepsy syndromes of childhood with sleep activation: insights from functional imaging. Eur J Paediatr Neurol. 2020;24:58-60.
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IMPRES Pediatric Epilepsy Surgery Conference
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The Comprehensive Pediatric Epilepsy Center is hosting its first Innovative Minds: Pediatric Research in Epilepsy Surgery (IMPRES) Conference on Friday, February 16, to Sunday, February 18, at Hotel Van Zandt in downtown Austin. CME and agenda details are on the registration site. Email
dcmcepilepsy@ascension.org for student, nurse, and trainee discounts.
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The Fifth Annual Practical
Pediatric Neuroscience Symposium
The fifth annual Practical Pediatric Neuroscience Symposium will be held on Saturday, May 18, at Dell Children’s with virtual access. The target audience is general pediatric practitioners who care for children with a wide range of neurological disorders. This free event will feature several interactive presentations by faculty members of the pediatric neurosciences program with topics ranging from concussion to emerging gene therapies for neuromuscular disease. Each presentation will provide specific techniques and evidence-based recommendations for diagnosis and management. CME credits will be offered. Registration details to come.
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The Neurotransmitter, 2024, Issue 1
UT Health Austin Pediatric Neurosciences at Dell Children’s is a clinical partnership between
Dell Children’s Medical Center and UT Health Austin, the clinical practice of
Dell Medical School at The University of Texas at Austin.
For additional program information:
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