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https://www.readbyqxmd.com/read/28926844/atypical-presentation-of-a-pediatric-cerebellar-ganglioglioma
#1
Richard Bram, Roberta J Seidman, David Chesler
BACKGROUND/AIMS: Gangliogliomas (GGs) are rare central nervous system tumors occurring primarily in the supratentorial compartment with infratentorial instances most often involving the brain stem. Infratentorial GGs typically present with signs and symptoms of increased intracranial pressure (ICP), cranial nerve deficits, or focal cerebellar findings; rarely, these tumors have been associated with focal seizures. METHODS: In this report, we describe an atypical presentation of a cerebellar GG in a 20-month-old male who initially presented with syncope and emesis in the absence of electrographic evidence of seizures, radiographic evidence of hydrocephalus, or elevated ICP...
September 20, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28925974/if-you-build-it-they-will-come-initial-experience-with-a-multi-disciplinary-pediatric-neurocritical-care-follow-up-clinic
#2
Cydni N Williams, Aileen Kirby, Juan Piantino
Pediatric Neurocritical Care diagnoses account for a large proportion of intensive care admissions. Critical care survivors suffer high rates of long-term morbidity, including physical disability, cognitive impairment, and psychosocial dysfunction. To address these morbidities in Pediatric Neurocritical Care survivors, collaboration between Pediatric Neurology and Pediatric Critical Care created a multidisciplinary follow-up clinic providing specialized evaluations after discharge. Clinic referrals apply to all Pediatric Neurocritical Care patients regardless of admission severity of illness...
September 19, 2017: Children
https://www.readbyqxmd.com/read/28922350/stem-cells-and-cell-based-therapies-for-cerebral-palsy-a-call-for-rigor
#3
REVIEW
Lauren L Jantzie, Joseph Scafidi, Shenandoah Robinson
Cell-based therapies hold significant promise for infants at risk for cerebral palsy (CP) from perinatal brain injury (PBI). PBI leading to CP results from multi-faceted damage to neural cells. Complex developing neural networks are injured by neural cell damage plus unique perturbations in cell signaling. Given that cell-based therapies can simultaneously repair multiple injured neural components during critical neurodevelopmental windows, these interventions potentially offer efficacy for patients with CP...
September 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28922267/the-1-year-follow-up-clinic-for-neonates-and-children-after-respiratory-extracorporeal-membrane-oxygenation-support-a-10-year-single-institution-experience
#4
Suzan Kakat, Maura O'Callaghan, Liz Smith, Raymond Hreiche, Deborah A Ridout, Jo Wray, Timothy Thiruchelvam, Katherine L Brown, Aparna U Hoskote
OBJECTIVES: To establish the effectiveness of a "1-year extracorporeal membrane oxygenation follow-up clinic" and to characterize any neurodevelopmental concerns identified. DESIGN: Single-center retrospective cohort of respiratory extracorporeal membrane oxygenation survivors over 10 years. SETTING: Nationally commissioned center for neonatal and pediatric (> 28 d of life) respiratory extracorporeal membrane oxygenation. PATIENTS: Children attending the follow-up clinic 1 year after receiving respiratory extracorporeal membrane oxygenation between 2003 and 2013...
September 15, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28921764/acanthamoeba-granulomatous-amoebic-encephalitis-after-pediatric-hematopoietic-stem-cell-transplant
#5
Scott L Coven, Eunkyung Song, Sarah Steward, Christopher R Pierson, Jennifer R Cope, Ibne K Ali, Monica I Ardura, Mark W Hall, Melissa G Chung, Rajinder P S Bajwa
Acanthamoeba encephalitis is a rare, often fatal condition, particularly after HSCT, with 9 reported cases to date in the world literature. Our case was originally diagnosed with ALL at age 3 years, and after several relapses underwent HSCT at age 9 years. At 17 years of age, he was diagnosed with secondary AML for which he underwent a second allogeneic HSCT. He presented with acute-onset worsening neurological deficits on day +226 after the second transplant and a post-mortem diagnosis of Acanthamoeba encephalitis was established, with the aid of the CDC...
September 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28921731/inter-observer-agreement-in-pediatric-cervical-spine-injury-assessment-between-prehospital-and-emergency-department-providers
#6
Lorin R Browne, Hamilton Schwartz, Fahd A Ahmad, Michael Wallendorf, Nathan Kuppermann, E Brooke Lerner, Julie C Leonard
BACKGROUND: Investigators have derived cervical spine injury (CSI) decision support tools from physician observations. There is a need to demonstrate that prehospital emergency medical services (EMS) providers can use these tools to appropriately determine the need for spinal motion restrictions and make field disposition decisions. OBJECTIVE: To determine the inter-observer agreement between EMS and emergency department (ED) providers for CSI risk assessment variables and overall gestalt for CSI in children after blunt trauma...
September 18, 2017: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
https://www.readbyqxmd.com/read/28917084/evaluation-of-neurodevelopment-of-children-with-congenital-hypothyroidism-by-the-denver-developmental-screening-test
#7
Ayşe Derya Buluş, Esra Tiftik
BACKGROUND: Thyroid hormones are essential for growth and brain development in childhood. Although congenital hypothyroidism (CH) is the most common reason for mental retardation, normal neurological development can be achieved through early and effective treatment. The aim of the present study was to evaluate the neurological development of CH patients aged 24-56 months. METHODS: The study included a total of 116 healthy control subjects and 112 patients aged 24-56 months who were diagnosed with CH during the neonatal period and were being followed up at the Pediatric Endocrinology Department, Keçiören Training and Research Hospital, between 2012 and 2015...
September 16, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#8
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28915516/mog-antibodies-in-pediatric-neurology
#9
Peter Huppke
No abstract text is available yet for this article.
September 15, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#10
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28904682/severity-of-motor-dysfunction-in-children-with-cerebral-palsy-seen-in-enugu-nigeria
#11
Christian Chukwukere Ogoke, Sylvester Onabeke Iloeje
INTRODUCTION: Children with cerebral palsy (CP) have gross motor dysfunction (GMD) of varying degrees of severity. The Gross Motor Function Classification System (GMFCS) is widely used internationally to classify children with CP into functional severity levels. There are few reports on the use of GMFCS in Nigeria to determine the severity of motor dysfunction in children with CP. This study aims to classify children with CP in Enugu on the basis of severity of their GMD in order to ascertain their management needs...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28904571/transcranial-magnetic-stimulation-as-an-additional-diagnostic-tool-in-children-with-acute-inflammatory-demyelinating-polyneuropathy
#12
Voitenkov Vladislav Voitenkov, Klimkin Andrey, Skripchenko Natalia, Aksenova Anastasia
CONTEXT: The diagnosis of polyneuropathy may be challenging at the early stages of the disease. Despite electromyography (EMG) efficacy in the establishment of polyneuropathy diagnosis, in some cases, results are dubious and neurophysiologists may implement additional techniques to ensure that conduction is affected. AIMS: The aim of the study was to evaluate motor-evoked potential (MEP) characteristics in children with acute inflammatory demyelinating polyneuropathy (AIDP)...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28904568/pediatric-autoimmune-encephalitis
#13
REVIEW
Massimo Barbagallo, Giovanna Vitaliti, Piero Pavone, Catia Romano, Riccardo Lubrano, Raffaele Falsaperla
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28903905/the-pathogenicity-of-genomic-genetic-variant-of-x-chromosomal-genes-in-males-with-intellectual-disability
#14
Ji-Ping Peng, Fang Liu, Hua Xie, Xiao-Li Chen
Intellectual Disability (ID, previously named mental retardation) is a group of common pediatric neurology disorders characterized by extensive genetic and phenotypic heterogeneity. About 25%-50% of ID was caused by genomic/genetic variants, in which genomic/genetic variants of X-chromosome are one of key pathogenic causation (25%-30%), resulting in X-linked ID (XLID). The epidemiological data showed that the male to female ratio is 1.3: 1 in ID patients. The prevalence of XLID in the whole ID population is 10%-15%, and this prevalence reaches 20%-25% in the male ID population...
June 20, 2017: Yi Chuan, Hereditas
https://www.readbyqxmd.com/read/28901462/role-of-ghrelin-in-small-intestinal-motility-following-pediatric-intracerebral-hemorrhage-in-mice
#15
Jieyu Zan, Lei Song, Jiejie Wang, Rong Zou, Fei Hong, Jinhua Zhao, Yijun Cheng, Ming Xu
Small intestinal motility (SIM) disorder is a common complication following pediatric intracerebral hemorrhage (ICH), leading to a poor prognosis in patients. Previous studies have shown that ghrelin is involved in SIM in various diseases; however, the role of ghrelin in pediatric ICH‑induced SIM disorder remains to be elucidated. The present study was designed to investigate the association between ghrelin and SIM post‑ICH, and to examine the effect of exogenous ghrelin administration on SIM in vivo...
September 12, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28898497/a-pediatric-structural-mri-analysis-of-healthy-brain-development-from-newborns-to-young-adults
#16
Jacob Levman, Patrick MacDonald, Ashley Ruyan Lim, Cynthia Forgeron, Emi Takahashi
Assessment of healthy brain maturation can be useful toward better understanding natural patterns of brain growth and toward the characterization of a variety of neurodevelopmental disorders as deviations from normal growth trajectories. Structural magnetic resonance imaging (MRI) provides excellent soft-tissue contrast, which allows for the assessment of gray and white matter in the developing brain. We performed a large-scale retrospective analysis of 993 pediatric structural brain MRI examinations of healthy subjects (n = 988, aged 0-32 years) imaged clinically at 3 T, and extracted a wide variety of measurements such as white matter volumes, cortical thickness, and gyral curvature localized to subregions of the brain...
September 12, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28894950/autism-spectrum-disorder-an-early-and-frequent-feature-in-cerebrotendinous-xanthomatosis
#17
Bianca M L Stelten, Olivier Bonnot, Hidde H Huidekoper, Francjan J van Spronsen, Peter M van Hasselt, Leo A J Kluijtmans, Ron A Wevers, Aad Verrips
BACKGROUND: Cerebrotendinous xanthomatosis (CTX) is an autosomal recessively inherited inborn error of metabolism (IEM) due to mutations in the CYP27A1 gene. The clinical picture ranges from being nearly asymptomatic in early childhood, up to severe disability at adult age. Infantile-onset diarrhea and juvenile-onset cataract are the earliest symptoms in childhood. In the current study, we evaluated the presence of autism spectrum disorder (ASD) in a large cohort of CTX patients. METHODS: We performed a retrospective patient file study in 77 genetically confirmed Dutch CTX patients to determine the frequency of ASD...
September 11, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28887279/sacral-alar-iliac-fixation-in-children-with-neuromuscular-scoliosis-minimum-5-year-follow-up
#18
Amit Jain, Brian T Sullivan, Anne Kuwabara, Khaled Kebaish, Paul D Sponseller
OBJECTIVE: The aim of our study was to investigate the 5-year outcomes of children with neuromuscular scoliosis treated with sacral-alar-iliac screws. METHODS: We reviewed clinical and radiographic records of patients ≤18 years old treated by 1 pediatric orthopaedic surgeon for neuromuscular scoliosis with spinal fusion using sacral-alar-iliac pelvic anchors. Thirty-eight patients, with a minimum 5-year radiographic follow-up (6.0 ± 1.2 years), were studied. Mean patient age was 13 ± 2...
September 5, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28883872/therapeutic-effects-of-adrenocorticotropic-hormone-acth-in-children-with-severely-intractable-seizure
#19
Jafar Nasiri, Azam Sarajan, Mehri Salari, Maryam Sedghi
OBJECTIVE: Treatment of intractable seizures other than spasms is difficult and controversial. There are few studies on efficacy of adrenocorticotropic hormone (ACTH) in treatment of patients with intractable seizure. MATERIALS & METHODS: Twenty-five patients with intractable seizure other than spasm including 14 boys and 11 girls with median age of 58 months referred to university clinics of Pediatric Neurology in Isfahan, Iran, during 2014-2015 were prospectively investigated...
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28882353/neurophysiological-monitoring-in-radiofrequency-ablation-of-spinal-osteoid-osteoma-with-a-progressive-time-and-temperature-protocol-in-children
#20
Mariano A Nöel, Martin J Segura, Sergio Sierre, Ida A Francheri Wilson, Carlos A Tello, Eduardo Galaretto, Rodrigo G Remondino, María E Talarico, Ernesto S Bersusky, Lucas Piantoni
STUDY DESIGN: Retrospective. Level IV Evidence. OBJECTIVE: To assess the utility of intraoperative neurophysiological monitoring (IONM) to detect and eventually prevent impending neurovascular damage during computed tomography (CT)-guided radiofrequency ablation (RFA) of spinal osteoid osteoma (OO) in children. SUMMARY AND BACKGROUND DATA: To our knowledge, this is the first case series of spinal OO in pediatric patients treated at a single center employing IONM during RFA...
September 2017: Spine Deformity
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