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Neuropediatrics

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https://www.readbyqxmd.com/read/28335042/acute-sensory-neuronopathy-following-enterovirus-infection-in-a-3-year-old-girl
#1
Chih-Chin Chiu, Chen-Ya Yang, Tsui-Fen Yang, Kon-Ping Lin, Shou-Hsien Huang, Jia-Chi Wang
Acute sensory neuronopathy (SNN) is a rapidly developing peripheral nervous system disease that primarily affects sensory neurons in the dorsal root ganglion or trigeminal ganglion, leading to the impairment of sensory axons. SNN is notably uncommon in childhood; only three cases of childhood or adolescent SNN have been reported to date. Moreover, SSN has never been reported in association with enterovirus infection. Here, we report the case of a 3-year-old girl who was initially diagnosed with enterovirus infection based on the presentation of fevers, rashes on all extremities, and ulceration over the posterior pharynx...
March 23, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28335041/the-cerebellum-and-its-wrapping-meninge-developmental-interplay-between-two-major-structures
#2
Martin Catala
Meninges have long been considered as a protective and supportive tissue for the central nervous system. Nevertheless, new developmental roles are now attributed to them. The meninges that surround the cerebellum come from the cephalic mesoderm. They are essential for the cerebellum to develop normally. They induce and maintain the basal lamina and glia limitans. In the absence of these structures, the external granular cells of the cerebellum migrate aberrantly and penetrate the subarachnoid space. The molecules involved in the recognition between the cerebellar primordium and the basal lamina belong to two groups in humans: dystroglycan and laminin on the one hand, and GPR56 and collagen III on the other...
March 23, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28324910/lacosamide-lowers-valproate-and-levetiracetam-levels
#3
Maria Tountopoulou, Bernhard Weschke, Angela M Kaindl
Lacosamide (LCM) due to no known drug interaction and the absence of metabolic enzyme induction is a good candidate for an add-on medication, especially in combination with lamotrigine, levetiracetam (LEV), oxcarbazepine, topiramate, and valproic acid (VPA). Here we report for the first time, to our knowledge, that LCM can lower VPA and LEV serum levels. At present, there are no known explicable mechanisms of action of LCM, which lowers VPA and LEV. Here observed drug interaction of LCM is of clinical significance, which might be useful for other colleagues in the field...
March 21, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28301882/infantile-hemangioma-of-the-posterior-fossa-in-a-newborn-early-management-and-long-term-follow-up
#4
Elsa Haine, Annick Sevely, Sergio Boetto, Marie-Bernadette Delisle, Claude Cances
A 21-day-old male infant was admitted with signs of intracranial hypertension. Brain magnetic resonance imaging (MRI) revealed a voluminous mass in the posterior fossa with an intense peripheral enhancement on T1 images with gadolinium. The child was treated secondarily by surgical decompression of the posterior fossa and the lesion was biopsied. The pathological findings indicated infantile hemangioma. Treatment with oral prednisolone was initiated at 3 months, given the lack of tumor involution. Six months after corticotherapy was stopped, repeated MRIs indicated a significant reduction in tumor size and then complete disappearance...
March 16, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28301881/quality-of-life-and-fitness-in-children-and-adolescents-with-epilepsy-epifit
#5
Markus Rauchenzauner, Claudia Hagn, Romana Walch, Matthias Baumann, Edda Haberlandt, Martin Frühwirth, Kevin Rostasy
Objective The objective of this study was to evaluate the correlation between fitness and health-related quality of life (HRQoL) in children with idiopathic epilepsy compared with a healthy matched control group. Methods In this study, 107 children conducted a 6-minute walk test, anthropometric parameters were measured, and HRQoL was assessed using a standardized questionnaire (KINDL-R). Children were divided into two groups: (1) the patient group (n = 48) and (2) the healthy control group (n = 59)...
March 16, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28255964/mr-imaging-of-acute-cerebellar-involvement-in-pediatric-anti-n-methyl-d-aspartate-receptor-encephalitis
#6
Bruno P Soares, Sonia F Calloni, Shai Shrot, Andrea Poretti
No abstract text is available yet for this article.
March 2, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28249300/4h-leukodystrophy-a-brain-magnetic-resonance-imaging-scoring-system
#7
Suzanne Vrij-van den Bos, Janna A Hol, Roberta La Piana, Inga Harting, Adeline Vanderver, Frederik Barkhof, Ferdy Cayami, Wessel N van Wieringen, Petra J W Pouwels, Marjo S van der Knaap, Geneviève Bernard, Nicole I Wolf
4H (hypomyelination, hypodontia and hypogonadotropic hypogonadism) leukodystrophy (4H) is an autosomal recessive hypomyelinating white matter (WM) disorder with neurologic, dental, and endocrine abnormalities. The aim of this study was to develop and validate a magnetic resonance imaging (MRI) scoring system for 4H. A scoring system (0-54) was developed to quantify hypomyelination and atrophy of different brain regions. Pons diameter and bicaudate ratio were included as measures of cerebral and brainstem atrophy, and reference values were determined using controls...
March 1, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28052304/is-tadpole-pupil-in-an-adolescent-girl-caused-by-denervation-hypersensitivity
#8
Jonas Kjeldbjerg Hansen, Hans Ulrik Møller
Tadpole pupil is a rarely encountered phenomenon caused by episodic, segmental iris dilator muscle spasm of short duration (2-15 minutes), occurring in clusters without a known precipitating factor. It has most commonly been described in women aged 28 to 48 years. A few hypotheses on pathogenesis have been discussed but none has been proved. Here, we present an adolescent girl with bilateral tadpole pupil that appeared during physical exercise. This is the first pediatric case of tadpole pupil, not caused by preceding surgery, to be published...
January 4, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28324907/invitation-and-welcome-to-the-43rd-annual-meeting-of-the-society-for-neuropediatrics
#9
Bernd A Neubauer, Andreas Hahn
No abstract text is available yet for this article.
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28324906/the-importance-of-facts-and-the-role-of-academic-publishers-in-today-s-world-a-publisher-s-view
#10
Daniel Schiff
No abstract text is available yet for this article.
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28282668/plasticity-during-early-brain-development-is-determined-by-ontogenetic-potential
#11
Ingeborg Krägeloh-Mann, Karen Lidzba, Marina A Pavlova, Marko Wilke, Martin Staudt
Two competing hypotheses address neuroplasticity during early brain development: the "Kennard principle" describes the compensatory capacities of the immature developing CNS as superior to those of the adult brain, whereas the "Hebb principle" argues that the young brain is especially sensitive to insults. We provide evidence that these principles are not mutually exclusive. Following early brain lesions that are unilateral, the brain can refer to homotopic areas of the healthy hemisphere. This potential for reorganization is unique to the young brain but available only when, during ontogenesis of brain development, these areas have been used for the functions addressed...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28282667/prof-dr-richard-michaelis-may-30-1931-through-january-17-2017
#12
Ingeborg-Krägeloh Mann
No abstract text is available yet for this article.
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28282666/posterior-reversible-encephalopathy-an-epileptic-syndrome
#13
Hans Hartmann
No abstract text is available yet for this article.
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28282665/honorary-award-of-the-german-speaking-society-of-child-neurology-for-the-year-2016-gesellschaft-f%C3%A3-r-neurop%C3%A3-diatrie-for-ingeborg-sophie-kr%C3%A3-geloh-mann
#14
https://www.readbyqxmd.com/read/28245506/should-posterior-reversible-encephalopathy-syndrome-be-mainly-considered-an-epileptic-disorder-results-of-a-sequential-neurophysiological-study-in-a-pediatric-cohort
#15
Daniele Grioni, Fabio Pavan, Giulia Prunotto, Francesco Canonico, Carlo de Grandi, Attilio Rovelli
Despite a wide number of studies trying to define clinical, physiopathological, and neuroradiological features of posterior reversible encephalopathy syndrome (PRES), the true nature of symptoms is still not fully understood. We studied a standard cohort of 24 pediatric patients, affected by hemato-oncological diseases, with a neuroradiological diagnosis consistent with PRES identified from 2006 to 2013. Ten of them developed PRES after hematopoietic stem cell transplantation. We analyzed the sequence of clinical, radiological, and electrophysiological data...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28245505/change-of-cerebral-oxygenation-during-surfactant-treatment-in-preterm-infants-lisa-versus-insure-procedures
#16
Giovanna Bertini, Caterina Coviello, Elena Gozzini, Tommaso Bianconi, Cecilia Bresci, Valentina Leonardi, Carlo Dani
Objectives The aim of the study was to compare the effects on cerebral oxygenation in preterm infants of two different procedures for surfactant administration: the LISA (low-invasive method of surfactant administration) and the InSurE (Intubation, SURfactant administration, Extubation). Study Design Twenty premature infants with respiratory distress syndrome were assigned to receive surfactant either by "LISA" (n = 10) or "InSurE" (n = 10) procedure. Patients were continuously studied by near-infrared spectroscopy (NIRS) for the measurement of cerebral regional oxygenation (rSO2C) and calculation of cerebral fractional oxygen extraction rate (cFTOE), and NIRS data were recorded 30 minutes before (T0) surfactant administration, during the procedure (Tproc), and 30 (T1), 60 (T2T2), and 120 minutes (T3) afterward...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28196383/health-related-quality-of-life-of-children-adolescents-with-vertigo-retrospective-study-from-the-german-center-of-vertigo-and-balance-disorders
#17
Anna Deissler, Lucia Albers, Rüdiger von Kries, Raphael Weinberger, Thyra Langhagen, Lucia Gerstl, Florian Heinen, Klaus Jahn, A Sebastian Schröder
Purpose To assess the impact of vertigo on health-related quality of life (HrQoL) of children/adolescents and to assess if the impact on HrQoL varies by age group, gender, and type of vertigo diagnoses. Methods A retrospective analysis was performed on the clinical and HrQoL data of children and adolescents referred to the German Center of Vertigo and Balance Disorders (n = 32; male = 17; female = 15; age range: 8-18 years), using the KIDSCREEN-52 questionnaire. For each scale, means of the Z-scores with 95% confidence intervals of the study and norm sample were compared...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28192817/spinocerebellar-ataxia-27-clinical-phenotype-of-twin-sisters-with-fgf14-deletion
#18
Alfonso Amado, Manuel Oscar Blanco, Alfredo Repáraz-Andrade
No abstract text is available yet for this article.
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28192816/rare-variant-of-gm2-gangliosidosis-through-activator-protein-deficiency
#19
Florian Brackmann, Christiane Kehrer, Wibke Kustermann, Judith Böhringer, Ingeborg Krägeloh-Mann, Regina Trollmann
GM2 gangliosidosis, AB variant, is a very rare form of GM2 gangliosidosis due to a deficiency of GM2 activator protein. We report on two patients with typical clinical features suggestive of GM2 gangliosidosis, but normal results for hexosaminidase A and hexosaminidase B as well as their corresponding genes. Genetic analysis of the gene encoding the activator protein, the GM2A gene, elucidated the cause of the disease, adding a novel mutation to the spectrum of GM2 AB variant. This report points out that in typical clinical constellations with normal enzyme results, genetic diagnostic for activator protein defects should be performed...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28122382/auditory-processing-in-children-with-migraine-a-controlled-study
#20
Larissa Mendonça Agessi, Thaís Rodrigues Villa, Deusvenir de Souza Carvalho, Liliane Desgualdo Pereira
Background This study aimed to investigate central auditory processing performance in children with migraine and compared with controls without headache. Methods Twenty-eight children of both sexes, aged between 8 and 12 years, diagnosed with migraine with and without aura, and a control group of the same age range and with no headache history, were included. Gaps-in-noise (GIN), duration pattern test (DPT), synthetic sentence identification (SSI) test, and nonverbal dichotic test (NVDT) were used to assess central auditory processing performance...
April 2017: Neuropediatrics
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