Neurology Commons^™

Childhood Absence With Polyspike Ictal-Onset, Sherouk A. Abdelmoity

Research Days

No abstract provided.

Stroke As The Initial Presentation Of Takayasu's Arteritis: A Case Report, Paran Davari, Paige Sutton, Kimberly S. Jones

Neurology Faculty Publications

In this study, we report the case of a pediatric neurology stroke patient who was ultimately diagnosed with Takayasu's Arteritis. Our case describes a 14-year-old Hispanic female with no significant past medical history who presented to an outside hospital for acute onset of confusion and right sided weakness. She was given tissue plasminogen activator (TPA) at the outside hospital and transferred as a stroke alert. Initial The NIH stroke scale (NIHSS) was 12 with primarily right sided symptoms. Physical exam was also significant for asymmetric pulses and blood pressures. Imaging was significant for multifocal stenosis. She was ultimately diagnosed with …

Transcutaneous Auricular Vagus Nerve Stimulation-Paired Rehabilitation For Oromotor Feeding Problems In Newborns: An Open-Label Pilot Study, Bashar W. Badran, Dorothea D. Jenkins, Daniel Cook, Sean Thompson, Morgan Darcy, William H. Devries, Georgia Mappin, Philipp Summers, Marom Bikson, Mark S. George

Publications and Research

Neonates born premature or who suffer brain injury at birth often have oral feeding dysfunction and do not meet oral intake requirements needed for discharge. Low oral intake volumes result in extended stays in the hospital (>2 months) and can lead to surgical implant and explant of a gastrostomy tube (G-tube). Prior work suggests pairing vagus nerve stimulation (VNS) with motor activity accelerates functional improvements after stroke, and transcutaneous auricular VNS (taVNS) has emerged as promising noninvasive form of VNS. Pairing taVNS with bottle-feeding rehabilitation may improve oromotor coordination and lead to improved oral intake volumes, ultimately avoiding the …

Management Of Childhood Migraine By Headache Specialist Versus Non-Headache Specialists, Kelly Valentini, Radhika Gutta, Gunjanpreet Kaur, Ahmad Farooqi, Lalitha Sivaswamy

Medical Student Research Symposium

This study aims to compare the management practices of a headache specialist with non-headache specialists in the treatment of pediatric migraine. The use of appropriate rescue medications and prophylactic agents, application of neuro-imaging, and short-term outcomes are compared in children treated by the two groups of physicians. A retrospective cohort study was conducted utilizing the electronic medical records of children 3-18 years of age with migraine, who were evaluated at a tertiary care children’s hospital from 2016-2018. Of the 849 patients that met the study criteria, 469 were classified as having chronic migraine or high-frequency episodic migraine and were followed …

Musculoskeletal Pain Outcomes Pre- And Post Intrathecal Baclofen Pump Implant In Children With Cerebral Palsy: A Prospective Cohort Study., Chantel C. Barney, Alyssa M. Merbler, Jean Stansbury, Linda E. Krach, Michael D. Partington, Patrick Graupman, Peter D. Kim, Debbie Song, Frank J. Symons

Manuscripts, Articles, Book Chapters and Other Papers

Objective: To characterize musculoskeletal pain intensity, duration, frequency, and interference with activities of daily living in children with cerebral palsy (CP) before and after intrathecal baclofen pump placement.

Design: Prospective cohort study.

Setting: Children's tertiary hospital.

Participants: Participants were children with CP (N=32; 53% male; mean age, 9.9y; age range, 4-17y). The majority of participants had a CP diagnosis of quadriplegia (76%) and relied on wheeled mobility (91%).

Interventions: Assessments were completed pre- and post intrathecal baclofen pump implant.

Main outcome measures: Because of considerable patient heterogeneity, both pain measures (Brief Pain Inventory, Dalhousie Pain Interview) were completed by proxy …

Comparing Open And Fetoscopic Fetal Surgical Repairs Of Myelomeningocele, Kyra Isaacs

Capstone Showcase

Introduction: There have been great advances in the treatment of myelomeningocele (MMC) spina bifida in the past 20 years. An open surgical repair in-utero has been shown to reduce mortality and morbidity, specifically decreasing the rate of shunt placement and hindbrain herniation compared to a postnatal surgical approach. However, this surgery introduced risks to the mother that were never a consideration in previous surgical approaches.

Methods: This paper compares research on new minimally invasive fetoscopic surgical techniques to open fetal surgery in the prenatal repair of MMC. Searches in PubMed and Clinical Key were conducted to produce papers published on …

Brain Development: Why The Young Sleep Longer, Budhaditya Chowdhury, Orie T. Shafer

Advanced Science Research Center

From absorbing new languages to mastering musical instruments, young children are wired to learn in ways that adults are not (Johnson and Newport, 1989). This ability coincides with periods of intense brain plasticity during which neurons can easily remodel their connections (Hubel and Wiesel, 1970). Many children are also scandalously good sleepers, typically getting several more hours of sleep per night than their parents (Jenni and Carskadon, 2007). As sleep deprivation has negative effects on learning and memory, learning like a child likely requires sleeping like one (Diekelmann and Born, 2010). Yet, how the ability to sleep for longer is …

Burden Of Rare Deleterious Variants In Wnt Signaling Genes Among 511 Myelomeningocele Patients, Luke Hebert, Paul Hillman, Craig Baker, Michael Brown, Allison Ashley-Koch, James E Hixson, Alanna C Morrison, Hope Northrup, Kit Sing Au

Faculty, Staff and Student Publications

Genes in the noncanonical WNT signaling pathway controlling planar cell polarity have been linked to the neural tube defect myelomeningocele. We hypothesized that some genes in the WNT signaling network have a higher mutational burden in myelomeningocele subjects than in reference subjects in gnomAD. Exome sequencing data from 511 myelomeningocele subjects was obtained in-house and data from 29,940 ethnically matched subjects was provided by version 2 of the publicly available Genome Aggregation Database. To compare mutational burden, we collapsed rare deleterious variants across each of 523 human WNT signaling genes in case and reference populations. Ten WNT signaling genes were …

Utilization Of A Continuous Pericapsular Nerve Group (Peng) Block With An Opioid-Sparing Repair Of A Femoral Neck Fracture In A Pediatric Patient, Karla Wyatt, Moustafa Zidane, Chyong-Jy Joyce Liu

Faculty, Staff and Students Publications

In the pediatric population, femoral neck fractures are usually associated with high-impact trauma and often present with pain in the groin area. Regional anesthesia can offer adjunctive therapy for acute pain management. Various techniques have been employed to circumvent pain related to hip fractures and resultant hip surgery. Neuraxial, lumbar plexus, caudal, epidural, fascia iliaca, and femoral continuous nerve block techniques are advantageous in mitigating hip pain. However, these approaches require patient repositioning during placement and carry the potential for motor blockade with resultant weakness. A newly described method, the Pericapsular Nerve Group (PENG) block, allows for analgesia of the …

Dr. Ahmed Abdelmoity To Receive Endowed Chair, Children's Mercy Hospital

Our Story Continues

Former Children’s Mercy CEO Rand O’Donnell, PhD, and his wife, Melva, are establishing an endowed chair position that will be awarded to Ahmed Abdelmoity, MD, FAAP, Division Director of Child Neurology, Section Chief of Epilepsy and Neurophysiology. Dr. Abdelmoity will become our 31st endowed chair at Children’s Mercy.

Muscle Activation Patterns During Movement Attempts In Children With Acquired Spinal Cord Injury: Neurophysiological Assessment Of Residual Motor Function Below The Level Of Lesion., Darryn A Atkinson, Laura Mendez, Natalie Goodrich, Sevda C Aslan, Beatrice Ugiliweneza, Andrea L Behrman

Physical Therapy Collection

Introduction: Characterization of residual neuromotor capacity after spinal cord injury (SCI) is challenging. The current gold standard for measurement of sensorimotor function after SCI, the International Society for Neurological Classification of Spinal Cord Injury (ISNCSCI) exam, seeks to determine isolated intentional muscle activation, however many individuals with SCI exhibit intentional movements and muscle activation patterns which are not confined to specific joint or muscle. Further, isolated muscle activation is a feature of the neuromuscular system that emerges during development, and thus may not be an appropriate measurement standard for children younger than 6.

Methods: We utilized neurophysiological assessment …

Examining Pediatric Tourette Syndrome, Alyssa Orlando

Honors Projects

Pediatric Tourette Syndrome is a complex neurological disorder. Many people associate it with swearing, however in reality it is very rarely associated with that. This paper entails my own personal experiene with Tourette Syndrome and examines potential treatment options in the pediatric population.

Pediatric Brainstem Gliomas: An Institutional Experience, Altaf Ali Laghari, Mirza Zain Baig, Muhammad Ehsan Bari, Aneela Darbar, Naureen Mushtaq, Umme E Hani Abdullah, Daniya Aziz Khan

Section of Neurosurgery

Objective: The aim of this study was to analyze the clinical profiles and outcomes of pediatric brainstem gliomas treated at our institute.
Methodology: We reviewed the files of 18 pediatric age group patients diagnosed with brainstem glioma at our institution. The following variables were recorded: age, sex, duration of symptoms, date of diagnosis, main clinical symptoms, Karnofsky performance status score, magnetic resonance imaging findings, histopathology findings, details of the treatment given, disease progression, and date of mortality/last follow-up. This data were then transferred to SPSS version 23 which was used for further analysis.
Results: The mean age of our cohort …

Treatment Of Attention-Deficit Hyperactivity Disorder In Children And Adolescents: Benefits And Challenges, Kaila Bollinger, Albert Bui, Adam Smith, Kelsey Fink, Manoranjan D'Souza

Pharmacy and Wellness Review

Attention-deficit hyperactivity disorder (ADHD) is a neurobehavioral disorder affecting approximately 11 percent of the country's children and adolescents, between the ages four to seventeen years. Stimulant medications such as methylphenidate and amphetamines are the first-line of treatment for ADHD. The increasing use of these stimulant medications has resulted in increased media attention and raised questions about their efficacy and safety. This review focuses on the history of stimulant use in ADHD, the disease's pathophysiology, the long-term benefits of pharmacotherapy, and the possible subsequent adverse effects associated with prolonged stimulant use in children and adolescents suffering from ADHD. Furthermore, we will …

Burden And Risk Of Neurological And Cognitive Impairment In Pediatric Sickle Cell Anemia In Uganda (Brain Safe): Final Results Of The Cross-Sectional Analysis, Nancy Green, Deogratias Munube, Paul Bangirana, Linda Buluma, Bridget Kebirungi, Robert Opoka, Ezekiel Mupere, Philip Kasirye, Sarah Kiguli, Annet Birabwa

Paediatrics and Child Health, East Africa

Background: Children with sickle cell anemia (SCA) are highly susceptible to stroke and other manifestations of pediatric cerebral vasculopathy. Detailed evaluations in sub-Saharan Africa are limited.

Methods: We aimed to establish the frequency and types of pediatric brain injury in a cross-sectional study at a large SCA clinic in Kampala, Uganda in a randomly selected sample of 265 patients with HbSS ages 1–12 years. Brain injury was defined as one or more abnormality on standardized testing: neurocognitive impairment using an age-appropriate test battery, prior stroke by examination or transcranial Doppler (TCD) velocities associated with stroke risk in children with …

Scurvy Presenting With Limp And Weakness: A Case Report., Robin M. Lund, Mara L. Becker, Steven Shapiro, Tyler Allison, Julia G. Harris

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Scurvy is one of the oldest diseases known to mankind. Although presently rare in the developed world, scurvy was a common potentially fatal disease. In recent times, the most common risk factors for scurvy include alcoholism, low socioeconomic status, and severely poor nutrition or dietary restriction secondary to psychiatric illness or developmental disorders. Our case demonstrates the importance of having a high index of clinical suspicion of an uncommon disease in developed countries and emphasizes the necessity of a dietary screening that could potentially reduce extensive work-up in patients with nonspecific complaints.

CASE PRESENTATION: We report a case of …

Initiating The Ketogenic Diet In Infants With Treatment Refractory Epilepsy While Maintaining A Breast Milk Diet, Jean-Baptist Lepichon, Lindsey M. Thompson, Megan Gustafson, Ahmed Abdelmoity

Manuscripts, Articles, Book Chapters and Other Papers

© 2019 The Authors

Purpose: The ketogenic diet has been found to be safe and effective in the treatment of drug resistant epilepsy in childhood. The age range of children undergoing this treatment has steadily been going down. There is strong evidence that it is a safe alternative in infants with drug resistant seizures. The American Academy of Pediatrics strongly supports continuing a breast milk diet until infants are at least six months of age. The purpose of this study is to evaluate the safety and efficacy of the ketogenic diet in infants while maintaining a breast milk diet.

Method: …

Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard

Department of Neurology Faculty Papers

BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available.

METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease.

RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of …

Recommendations For The Design Of Therapeutic Trials For Neonatal Seizures., Janet S. Soul, Ronit Pressler, Marilee Allen, Geraldine Boylan, Heike Rabe, Ron Portman, Pollyanna Hardy, Sarah Zohar, Klaus Romero, Brian Tseng, Varsha Bhatt-Mehta, Cecil Hahn, Scott Denne, Stephane Auvin, Alexander Vinks, John Lantos, Neil Marlow, Jonathan M. Davis, International Neonatal Consortium

Manuscripts, Articles, Book Chapters and Other Papers

Although seizures have a higher incidence in neonates than any other age group and are associated with significant mortality and neurodevelopmental disability, treatment is largely guided by physician preference and tradition, due to a lack of data from well-designed clinical trials. There is increasing interest in conducting trials of novel drugs to treat neonatal seizures, but the unique characteristics of this disorder and patient population require special consideration with regard to trial design. The Critical Path Institute formed a global working group of experts and key stakeholders from academia, the pharmaceutical industry, regulatory agencies, neonatal nurse associations, and patient advocacy …

Asymmetric Crying Facies Syndrome, Kai-Yu Ho Pt, Phd, Jing Nong Liang Pt, Phd

Physical Therapy Faculty Research

A female infant, born vaginally at 40 weeks of gestation with a birthweight of 3260 g had an Apgar score of 10 at 1 and 5 minutes. She presented with an asymmetric facial expression when crying, with the right side of her mouth deviating inferior-laterally (Figure, A and Video[available at www.jpeds.com]). At rest, her facial expression was symmetric and a thinner left lower lip was noted (Figure, B). A pediatricneurologist noted intact motor, reflex, and cranial nerve functions, and she was diagnosed with asymmetric crying facies, likely because of hypoplasia of the left depressor angularis oris muscle. Asymmetric crying facies …

Total And Free Plasma Bilirubin And Clinical Outcomes In Severe Hyperbilirubinemia, Sean M. Riordan, Jean-Baptiste Lepichon, Steven Shapiro, Tina Slusher, Fatima Abdullahi, Hafsat M. Suleiman, Victor C. Pam, Mamu B. Samuel, Christopher S. Yilgwan, Christian Isichei, Idris Y. Mohammed

Posters

Acute bilirubin encephalopathy (ABE) and kernicterus spectrum disorder (KSD) have become relatively uncommon in high income countries but remain a major cause of morbidity and mortality in low- and middle-income countries. To better understand the relationship between free (Bf) and total (TB) bilirubin levels and the development of ABE and KSD we followed infants born in three large tertiary centers in northern and central Nigeria (Jos, Kano and Zaria).

Weighted Pathway Genetic Load Analysis Of Hyperbilirubinemic Infants Indicates A Potential Genetic Component For Susceptibility To Bilirubin Neurotoxicity, Sean M. Riordan, Jean-Baptiste Lepichon, Steven Shapiro, John Cowden, Monica Villagullen, Laurence Thielemans, Dina Villanueva Garcia, Jesus Aguirre-Hernandez

Posters

Severe kernicterus spectrum disorder (KSD) is described as motor and auditory deficits resulting from brain damage caused by hyperbilirubinemia. The severity of HB does not always predict the severity of injury. The lack of a strong monogenetic link to susceptibility suggests bilirubin-induced brain damage may be due to impaired bilirubin response pathways. This poster describes work to use a modified pathway genetic load (mPGL) score method to perform a targeted genetic analysis of whole exome data from patients with various degrees of neonatal HB, with an ultimate goal of developing a neonatal screen to susceptibiltiy to bilirubin neurotoxicity.

A Pilot Study Identifying Brain-Targeting Adaptive Immunity In Pediatric Extracorporeal Membrane Oxygenation Patients With Acquired Brain Injury, Sterling B. Ortega, Poornima Pandiyan, Jana Windsor, Vanessa O. Torres, Uma M. Selvaraj, Amy Lee, Michael Morriss, Fenghua Tian, Lakshmi Raman, Ann M. Stowe

Neurology Faculty Publications

OBJECTIVES: Extracorporeal membrane oxygenation provides short-term cardiopulmonary life support, but is associated with peripheral innate inflammation, disruptions in cerebral autoregulation, and acquired brain injury. We tested the hypothesis that extracorporeal membrane oxygenation also induces CNS-directed adaptive immune responses which may exacerbate extracorporeal membrane oxygenation-associated brain injury.

DESIGN: A single center prospective observational study.

SETTING: Pediatric and cardiac ICUs at a single tertiary care, academic center.

PATIENTS: Twenty pediatric extracorporeal membrane oxygenation patients (0-14 yr; 13 females, 7 males) and five nonextracorporeal membrane oxygenation Pediatric Logistic Organ Dysfunction score matched patients.

INTERVENTIONS: None.

MEASUREMENTS AND MAIN RESULTS: Venous blood samples were …

A Closer Look At Arsa Activity In A Patient With Metachromatic Leukodystrophy., Kathleen Doherty, S. Barron Frazier, Matthew Clark, Anna Childers, Sumit Pruthi, David A. Wenger, Jessica Duis

Department of Neurology Faculty Papers

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in …

Early Progression Of Krabbe Disease In Patients With Symptom Onset Between 0 And 5 Months., Maria L. Beltran-Quintero, Nicholas A. Bascou, Michele D. Poe, David A. Wenger, Carlos A. Saavedra-Matiz, Matthew J. Nichols, Maria L. Escolar

Department of Neurology Faculty Papers

BACKGROUND: Krabbe disease is a rare neurological disorder caused by a deficiency in the lysosomal enzyme, β-galactocerebrosidase, resulting in demyelination of the central and peripheral nervous systems. If left without treatment, Krabbe disease results in progressive neurodegeneration with reduced quality of life and early death. The purpose of this prospective study was to describe the natural progression of early onset Krabbe disease in a large cohort of patients.

METHODS: Patients with early onset Krabbe disease were prospectively evaluated between 1999 and 2018. Data sources included diagnostic testing, parent questionnaires, standardized multidisciplinary neurodevelopmental assessments, and neuroradiological and neurophysiological tests.

RESULTS: We …

Difficult Conversations., Alison Christy

Articles, Abstracts, and Reports

No abstract provided.

Early Identification Of Depression In Patients With Pediatric Epilepsy, Erin Fecske, Paul Glasier, Lines Vargas Collado, Elizabeth Rende

Posters

Describes the use of the Neurological Disorders Depression Inventory-Epilepsy-Youth (NDDI-E-Y) in adolescent patients in a comprehensive epilepsy center to identify patients who need referral for mental health care.

Pediatric Cardioembolic Stroke In Midaortic Syndrome, Ana C. Albuja, Mauricio F. Villamar, Alejandra M. Stewart, Donita D. Lightner

Neurology Faculty Publications

No abstract provided.

Manifestations Of Hypomelanosis Of Ito, Ana C. Albuja, Arpan Shrivastava, Gulam Q. Khan

Neurology Faculty Publications

No abstract provided.

Review Of Karyotypic Data From Low Grade Glial Brain Tumors, Specifically Pilocytic Astrocytomas, And Correlation Of Genetic Aberrations With Tumor Recurrence., Linda D. Cooley, Scott C. Smith, Lisa Warren, Melissa Gener, Kevin Ginn, John Herriges

Posters

Abstract: Brain tumors are the most common solid tumor of childhood. Approximately 50% of pediatric CNS tumors are low grade gliomas (WHO grade I or II) and Pilocytic astrocytoma (PA) is the most common accounting for 33% of all gliomas in children 0-14 years and ~18% of all childhood brain tumors. Prognosis with this slow-growing tumor is excellent; 10 year overall survival of ~95%. However, event free survival averages ~50%. Patient age and extent of tumor resection are key prognostic factors; tumor location and size impact resection and outcome. Histopathological features indicate PA is a benign tumor and rarely are …