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Journal of Child Neurology

Courtney A Haney, Anita Charpentier, Zahava Turner, Stacey K Bessone, Sarah C Doerrer, Eric H Kossoff
INTRODUCTION: Since its creation, patients on ketogenic diet are told to avoid liquid medications due to theoretical concerns of "hidden" carbohydrates. However, switching from liquid to tablet formulations can be problematic, especially for infants and young children. We theorized that increasing the daily ketogenic ratio might compensate for liquid antiseizure drug carbohydrates. METHODS: Two tables were created (for 3:1 and 4:1 ketogenic ratios), with variables including daily volume of antiseizure drugs and calories...
February 20, 2019: Journal of Child Neurology
Senem Ayça, Halil Ural Aksoy, İsmail Taştan, Muzaffer Polat
Levels of melatonin have been reported before in children with epilepsy, but such has not been reported to date in those with continuous spikes and waves during sleep. The aim of the present study was to assess serum melatonin levels and melatonin circadian rhythm in patients with continuous spikes and waves during sleep and epilepsy. Serum melatonin was measured in 39 children stratified into 3 groups. Group 1 included 15 patients with continuous spikes and waves during sleep, group 2 included 12 epilepsy patients, and group 3 included 12 controls, respectively...
February 14, 2019: Journal of Child Neurology
Ang Li, Xuan Kang, Frederick Edelman, Andrew J Waclawik
Stormorken syndrome is a rare genetic disorder (MIM 185070) first reported in 1983 with thrombocytopenia, muscle weakness, asplenia, and miosis caused by a mutation of the stromal interaction molecule 1 ( STIM1) gene. 1 The muscle weakness is caused by a myopathy with tubular aggregate formation. We report a family in which both child and mother presented with proximal muscle weakness and thrombocytopenia. Histologic, histochemical, and electron microscopy studies were performed on the muscle specimen. It documented accumulation of tubular aggregates and chronic myopathic changes with dystrophic features...
February 14, 2019: Journal of Child Neurology
Sarah Grace Buttle, Brigitte Lemyre, Erick Sell, Stephanie Redpath, Srinivas Bulusu, Richard J Webster, Daniela Pohl
BACKGROUND/OBJECTIVE: Seizure monitoring via amplitude-integrated EEG is standard of care in many neonatal intensive care units; however, conventional EEG is the gold standard for seizure detection. We compared the diagnostic yield of amplitude-integrated EEG interpreted at the bedside, amplitude-integrated EEG interpreted by an expert, and conventional EEG. METHODS: Neonates requiring seizure monitoring received amplitude-integrated EEG and conventional EEG in parallel...
February 14, 2019: Journal of Child Neurology
Gustavo H B Maegawa
The leukodystrophies are a group of genetic metabolic diseases characterized by an abnormal development or progressive degeneration of the myelin sheath. The myelin is a complex sheath composed of several macromolecules covering axons as an insulator. Each of the leukodystrophies is caused by mutations in genes encoding enzymes that are involved in myelin production and maintenance. The lysosomal storage diseases are inborn disorders of compartmentalized cellular organelles with broad clinical manifestations secondary to the progressive accumulation of undegraded macromolecules within lysosomes and related organelles...
February 13, 2019: Journal of Child Neurology
Harikrishan S Sachdev, Bhairav Patel, Mark McManis, Mark Lee, Dave F Clarke
Intractable epilepsy may have a more severe effect on children in comparison to adults because the motor, sensorial, and cognitive functions of children are still undergoing development. For this same reason, however, children maintain a greater potential for recovery from intractable epilepsy because of the remaining cerebral plasticity. Thus, after 2 unsuccessful antiepileptic medications, surgical intervention is recommended. Pre-surgical localization of the epileptogenic zone increases seizure-freedom post-surgery by 200% to 300%...
February 12, 2019: Journal of Child Neurology
J Ben Renfroe, Mark Mintz, Ronald Davis, Jose Ferreira, Sharon Dispoto, Jim Ferry, Yuko Umetsu, Bhaskar Rege, Oneeb Majid, Ziad Hussein, Antonio Laurenza
Study 232, an open-label pilot study with an extension phase, evaluated the pharmacokinetics and preliminary safety/tolerability and efficacy of adjunctive perampanel oral suspension (≤0.18 mg/kg/d) in epilepsy patients aged ≥2 to <12 years. Patients were grouped into cohorts 1 (aged ≥7 to <12 years) and 2 (aged ≥2 to <7 years). The Core Study included pretreatment (≤2 weeks) and treatment phases (7-week titration; 4-week maintenance; 4-week follow-up [for those not entering the extension])...
February 10, 2019: Journal of Child Neurology
Selman Kesici, Murat Tanyıldız, Filiz Yetimakman, Benan Bayrakci
OBJECTIVE: Intravenous immunoglobulin and plasma exchange are proven treatments for Guillain-Barré syndrome. Despite these treatments, the prognosis for severe Guillain-Barré syndrome is still not satisfactory. This article seeks for a logical timing for plasma exchange-intravenous immunoglobulin synergy, which may improve outcome in severe Guillain-Barré syndrome requiring mechanical ventilation. STUDY DESIGN: This study is an open-label study. Nine pediatric severe Guillain-Barré syndrome patients requiring mechanical ventilation were treated with novel treatment strategy named as "zipper method...
January 29, 2019: Journal of Child Neurology
Jeremias Antinew, Bruno Pitrosky, Lloyd Knapp, Mary Almas, Verne Pitman, Jing Liu, Dana Craiu, Margaret Modequillo, Douglas Nordli, Viktor Farkas, Mark Kristof Farkas
Efficacy and safety of pregabalin as adjunctive treatment for children (aged 4-16 years) with partial-onset seizures, hereafter termed focal onset seizures for this study, was evaluated. This double-blind, randomized, placebo-controlled, international study had 3 phases: 8-week baseline, 12-week double-blind treatment (2-week dose escalation; 10-week fixed dose), and 1-week taper. Selection criteria included experiencing focal onset seizures and receiving a stable regimen of 1 to 3 antiepileptic drugs. Study treatments were pregabalin 2...
January 27, 2019: Journal of Child Neurology
Naama Yosha-Orpaz, Sharon Aharoni, Malcolm Rabie, Yoram Nevo
Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. During the acute phase, the disorder can be life-threatening by involving the respiratory muscles and the autonomic nervous system. Nevertheless, the prognosis is good, and most children achieve full recovery. The aim of this study was to characterize the clinical and electrophysiologic findings in children with Guillain-Barré syndrome referred to a tertiary center in Israel. A retrospective database review from 2009 to 2015 identified 39 children...
January 23, 2019: Journal of Child Neurology
Marian S McDonagh, Rebecca Holmes, Frances Hsu
Sleep problems are common in children, especially those with neurodevelopmental disorders, and can lead to consequences in behavior, functioning, and quality of life. We systematically reviewed the efficacy and harms of pharmacologic treatments for sleep disorders in children and adolescents. We searched MEDLINE, Cochrane library databases, and PsycINFO through June 2018. We included 22 placebo-controlled randomized controlled trials (1-13 weeks' duration), involving 1758 children (mean age 8.2 years). Single randomized controlled trials of zolpidem and eszopiclone in children with attention-deficit/hyperactivity disorder (ADHD) showed no improvement in sleep or ADHD ratings...
January 23, 2019: Journal of Child Neurology
Sandro J Corti, Natalie M Pizzimenti, Matthew T McCarthy, Kate M Essad, Jeffrey S Kutcher
Despite growing research on concussion, there is minimal evidence comparing the acute presentation of concussion between pediatric and adult patients. This cross-sectional study compares injury characteristics, symptoms, and neurologic examination in sport-related concussion based on age. Patients presenting to an outpatient sports neurology clinic for initial assessment of concussion within 7 days of injury were divided into 2 groups, 18 and older (n = 28) and 17 and younger (n = 107). There were no significant differences between pediatric and adult patients in any score of the Sport Concussion Assessment Tool-3rd Edition symptom scale, neurologic examination category, pertinent elements of past medical history, or characteristics of the concussion...
January 22, 2019: Journal of Child Neurology
David R Howell, Jessie R Oldham, Anna N Brilliant, William P Meehan
Our objective was to identify factors associated with trouble falling asleep postconcussion. Patients seen for care after sport-related concussion were assessed ≤10 days postinjury and were between 6 and 19 years of age. Outcomes included symptoms (Post-Concussion Symptom Scale), postural steadiness, missed school, and medical history. There were 261 patients (49% female; mean age=14.8±2.5 years): 45% reported trouble falling asleep. A greater proportion of patients who reported trouble falling asleep missed ≥2 days of school (64% vs 49%; P = ...
January 22, 2019: Journal of Child Neurology
Carlo M Bertoncelli, Paola Altamura, Edgar Ramos Vieira, Domenico Bertoncelli, Susanne Thummler, Federico Solla
BACKGROUND: Intellectual disability and impaired adaptive functioning are common in children with cerebral palsy, but there is a lack of studies assessing these issues in teenagers with cerebral palsy. Therefore, the aim of this study was to develop and test a predictive machine learning model to identify factors associated with intellectual disability in teenagers with cerebral palsy. METHODS: This was a multicenter controlled cohort study of 91 teenagers with cerebral palsy (53 males, 38 females; mean age ± SD = 17 ± 1 y; range: 12-18 y)...
January 22, 2019: Journal of Child Neurology
Anaita U Hegde, Purva K Karnavat, R Vyas, Melissa L DiBacco, P Ellen Grant, Phillip L Pearl
γ-Aminobutyric acid (GABA)-transaminase deficiency is an ultra-rare disorder of GABA metabolism that was described for decades as an early-onset epileptic encephalopathy plus movement disorder and hypersomnolence with mortality in early childhood. We report 2 affected siblings in adolescence and adulthood, both with profound developmental impairment, intractable epilepsy, movement disorder, and behavioral fluctuations. This considerably expands the phenotype and longevity of this inherited neurotransmitter disease...
January 15, 2019: Journal of Child Neurology
Qi Xu, Vann Chau, Chinnuwat Sanguansermsri, Katherine E Muir, Emily W Y Tam, Steven P Miller, Darren S T Wong, Hao Chen, Peter K H Wong, Jill G Zwicker, Kenneth J Poskitt, Alan Hill, Elke H Roland
OBJECTIVE: To determine if patterns of hypoxic-ischemic brain injury on magnetic resonance imaging (MRI) in term newborns predict subsequent childhood epilepsy. Methods: This retrospective cohort study includes term newborns with encephalopathy (n = 181) born between 2004-2012 and admitted to British Columbia Children's Hospital. MRI was performed between 3 and 5 days of age. The predominant patterns of hypoxic-ischemic injury were classified as Normal, Watershed, Basal Nuclei, Total, and Focal-Multifocal...
January 15, 2019: Journal of Child Neurology
Kim Beernaert, Malin Lövgren, Jørgen Jeppesen, Ulla Werlauff, Jes Rahbek, Thomas Sejersen, Ulrika Kreicbergs
OBJECTIVE: This study aims to assess the experiences and wishes of parents of children with severe spinal muscular atrophy regarding information and decision-making throughout the course of the illness. STUDY DESIGN: A full population survey, conducted in 2015, among parents of children with severe spinal muscular atrophy who were born in Denmark between January 1, 2003, and December 31, 2013. We used a study-specific questionnaire with items about experiences and wishes concerning the provision of information about diagnosis, treatment, and end-of-life care...
January 15, 2019: Journal of Child Neurology
Alysa Almojuela, Mohammed Hasen, Frederick A Zeiler
The Full Outline of UnResponsiveness (FOUR) score is a neurologic assessment score. Its benefit over pre-existing scores is its evaluation of brainstem reflexes and respiratory pattern. Our goal was to perform a scoping systematic review of the literature on the application of the FOUR score within pediatric patients. Six databases were searched and 2 reviewers independently screened the results. The initial search yielded 1709 citations; ultimately, 6 studies composed of 571 pediatric patients were used. Four studies examined interobserver reliability of the FOUR score and found it to be good to excellent...
January 10, 2019: Journal of Child Neurology
Amaran Moodley, Jack Swanson, Charles Grose, Daniel J Bonthius
Varicella vaccination is now virtually universal in North America, as well as in some European and Asian countries. Since varicella vaccine is a live attenuated virus, the virus replicates in the skin after administration and can travel via sensory nerves or viremia to become latent in the dorsal root ganglia. In some immunized children, virus reactivates within a few months to a few years to cause the dermatomal exanthem known as herpes zoster (shingles). Herpes zoster caused by vaccine virus often reactivates within the same dermatome as the site of the original varicella vaccine injection...
January 10, 2019: Journal of Child Neurology
Sara Wofford, Sarah Noblin, Jessica M Davis, Laura S Farach, S Shahrukh Hashmi, Pedro Mancias, Victoria F Wagner
Identifying genetic diagnoses for neurologic conditions with a considerable hereditary component, such as autism spectrum disorder, intellectual disability, and epilepsy, is critical to providing proper medical management for patients and their families. However, many patients with these conditions are not tested appropriately or receive no genetic testing at all. The current study was designed to characterize the genetic testing practices of the providers most likely to evaluate or order genetic testing for these patients: pediatric neurologists, geneticists, and genetic counselors...
January 4, 2019: Journal of Child Neurology
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