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paediatric neurology

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https://www.readbyqxmd.com/read/29245106/cerebral-arterio-venous-malformations-in-the-paediatric-population-angiographic-characteristics-multimodal-treatment-strategies-and-outcome
#1
Klaus-Peter Stein, Bernd-Otto Huetter, Sophia Goericke, Neriman Oezkan, Rebecca Leyrer, I Erol Sandalcioglu, Michael Forsting, Ulrich Sure, Oliver Mueller
OBJECTIVES: Cerebral arterio-venous malformations (AVM) are considered congenital lesions, emerging as an important cause of haemorrhagic stroke in children. The potential influence of age on clinical presentation and angio-architecture have been analysed extensively in the last years. Yet, comparative studies comprising ascending age groups may be limited in their conclusions, especially when comparing young children and adults. It is the aim of this study to evaluate characteristic clinical and angiographic features of AVM within a paediatric subgroup and their correlation to age at presentation...
December 5, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29208342/pediatric-stroke-related-to-lyme-neuroborreliosis-data-from-the-swiss-neuropaediatric-stroke-registry-and-literature-review
#2
O Monteventi, M Steinlin, M Regényi, E Roulet-Perez, P Weber, J Fluss
BACKGROUND: Cerebrovascular complications of Lyme neuroborreliosis (LNB) are poorly documented in the paediatric population. METHODS: We performed a retrospective analysis from prospectively registered cases of acute ischemic stroke (AIS) from the Swiss NeuroPaediatric Stroke Registry (SNPSR) from 2000 to 2015. Only cases with serologically confirmed LNB were included. In addition, a literature review on paediatric stroke cases secondary to Lyme neuroborreliosis in the same time frame was performed...
November 24, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29196143/intravenous-immunoglobulin-g-as-adjuvant-treatment-in-drug-resistant-childhood-epilepsy
#3
Z González-Castillo, E Solórzano Gómez, A Torres-Gómez, J A Venta Sobero, J Gutiérrez Moctezuma
BACKGROUND: Epilepsy is the most common neurological disease in childhood; depending on the definition of drug-resistant epilepsy, incidence varies from 10% to 23% in the paediatric population. The objective of this study was to account for the decrease in the frequency and/or monthly duration of epileptic seizures in paediatric patients with drug-resistant epilepsy treated with antiepileptic drugs, before and after adding intravenous immunoglobulin G (iIV IgG). METHODS: This is an analytic, observational, retrospective case-control study...
November 28, 2017: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29186350/macrophage-enzyme-and-reduced-inflammation-drive-brain-correction-of-mucopolysaccharidosis-iiib-by-stem-cell-gene-therapy
#4
Rebecca J Holley, Stuart M Ellison, Daniel Fil, Claire O'Leary, John McDermott, Nishanthi Senthivel, Alexander W W Langford-Smith, Fiona L Wilkinson, Zelpha D'Souza, Helen Parker, Aiyin Liao, Samuel Rowlston, Hélène F E Gleitz, Shih-Hsin Kan, Patricia I Dickson, Brian W Bigger
Mucopolysaccharidosis IIIB is a paediatric lysosomal storage disease caused by deficiency of the enzyme α-N-acetylglucosaminidase (NAGLU), involved in the degradation of the glycosaminoglycan heparan sulphate. Absence of NAGLU leads to accumulation of partially degraded heparan sulphate within lysosomes and the extracellular matrix, giving rise to severe CNS degeneration with progressive cognitive impairment and behavioural problems. There are no therapies. Haematopoietic stem cell transplant shows great efficacy in the related disease mucopolysaccharidosis I, where donor-derived monocytes can transmigrate into the brain following bone marrow engraftment, secrete the missing enzyme and cross-correct neighbouring cells...
November 27, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29177080/eular-acr-classification-criteria-for-adult-and-juvenile-idiopathic-inflammatory-myopathies-and-their-major-subgroups-a-methodology-report
#5
Matteo Bottai, Anna Tjärnlund, Giola Santoni, Victoria P Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A Amato, Richard J Barohn, Matthew H Liang, Jasvinder A Singh, Rohit Aggarwal, Snjolaug Arnardottir, Hector Chinoy, Robert G Cooper, Katalin Danko, Mazen M Dimachkie, Brian M Feldman, Ignacio García-De La Torre, Patrick Gordon, Taichi Hayashi, James D Katz, Hitoshi Kohsaka, Peter A Lachenbruch, Bianca A Lang, Yuhui Li, Chester V Oddis, Marzena Olesinka, Ann M Reed, Lidia Rutkowska-Sak, Helga Sanner, Albert Selva-O'Callaghan, Yeong Wook Song, Jiri Vencovsky, Steven R Ytterberg, Frederick W Miller, Lisa G Rider, Ingrid E Lundberg
Objective: To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups. Methods: An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology and paediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children)...
2017: RMD Open
https://www.readbyqxmd.com/read/29174722/functional-progression-of-patients-with-neurological-diseases-in-a-tertiary-paediatric-intensive-care-unit-our-experience
#6
P Madurga Revilla, J López Pisón, P Samper Villagrasa, J P García Íñiguez, R Garcés Gómez, M Domínguez Cajal, I Gil Hernández
INTRODUCTION: Neurological diseases explain a considerable proportion of admissions to paediatric intensive care units (PICU), and are a significant cause of morbidity and mortality. This study aims to analyse the functional progression of children with critical neurological conditions. MATERIAL AND METHODS: Retrospective descriptive study of children admitted to PICU with neurological diseases over a period of 3 years (2012-2014), assessing vital and functional prognosis at PICU discharge and at one year according to the Pediatric Cerebral and Overall Performance Category scales (PCPC-POPC) and the Functional Status Scale (FSS)...
November 23, 2017: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29171110/the-prevalence-of-narcolepsy-in-catalunya-spain
#7
Ester Tió, Carles Gaig, Maria Giner-Soriano, Odile Romero, Maria-José Jurado, Gemma Sansa, Montse Pujol, Oscar Sans, Ion Álvarez-Guerrico, Nuria Caballol, Marta Jimenez, Juan-Luis Becerra, Antonio Escartin, Carmen Monasterio, Albert Molins, Antoni Bove, Jaume Viña, Alex Iranzo, Roser Cambrodi, Gonzalo Calvo, Rosa Morros, Joan Santamaria
Previous studies have estimated an overall prevalence for narcolepsy between 15 and 70 cases per 100 000 inhabitants. We aimed to estimate the prevalence of narcolepsy in Catalunya (Catalonia), a north-east region of Spain (7 424 754 inhabitants), on 31 December 2014 by identifying all living subjects diagnosed with narcolepsy. First, we identified patients diagnosed by one of the 13 sleep, paediatric or neurological departments that perform tests regularly to diagnose narcolepsy. In a second phase, we searched for additional patients with narcolepsy in a clinical database of the primary health-care system...
November 24, 2017: Journal of Sleep Research
https://www.readbyqxmd.com/read/29149389/autoregulation-in-paediatric-tbi-current-evidence-and-implications-for-treatment
#8
REVIEW
Joseph E Donnelly, Adam M H Young, Ken Brady
BACKGROUND: Children who survive acute traumatic brain injury are at risk of death from subsequent brain swelling and secondary injury. Strict physiologic management in the ICU after traumatic brain injury is believed to be key to survival, and cerebral perfusion pressure is a prominent aspect of post brain injury care. However, optimal cerebral perfusion pressure targets for children are not known. Autoregulation monitoring has been used to delineate individualized optimal perfusion pressures for patients with traumatic brain injury...
October 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29132430/hospital-choice-in-germany-from-the-patient-s-perspective-a-cross-sectional-study
#9
Werner de Cruppé, Max Geraedts
BACKGROUND: In many countries health policy encourages patients to choose their hospital, preferably by considering information of performance reports. Previous studies on hospital choice mainly have focused on patients undergoing elective surgery. This study examined a representative sample of hospital inpatients across disciplines and treatment interventions in Germany. Its research questions were: How many patients decide where to go for hospital treatment? How much time do patients have before admission? Which sources of information do they use, and which criteria are relevant to their decision? METHODS: Cross-sectional observational study covering 1925 inpatients of 46 departments at 17 hospitals in 2012...
November 13, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/29129487/-evaluation-of-dysphagia-results-after-one-year-of-incorporating-videofluoroscopy-into-its-study-introduction
#10
Ruth García Romero, Ignacio Ros Arnal, María José Romea Montañés, José Antonio López Calahorra, Cristina Gutiérrez Alonso, Beatriz Izquierdo Hernández, Carlos Martín de Vicente
INTRODUCTION: Dysphagia is very common in children with neurological disabilities. These patients usually suffer from respiratory and nutritional problems. The videofluoroscopic swallowing study (VFSS) is the most recommended test to evaluate dysphagia, as it shows the real situation during swallowing. OBJECTIVES: To analyse the results obtained in our centre after one year of the implementation of VFSS, the clinical improvement after confirmation, and the prescription of an individualised treatment for the patients affected...
November 9, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/29127227/protocol-for-hospital-based-surveillance-of-cerebral-palsy-cp-in-hanoi-using-the-paediatric-active-enhanced-disease-surveillance-mechanism-paeds-vietnam-a-study-towards-developing-hospital-based-disease-surveillance-in-vietnam
#11
Gulam Khandaker, Nguyen Van Bang, Trịnh Quang Dũng, Nguyen Thi Huong Giang, Cao Minh Chau, Nguyen Thi Van Anh, Nguyen Van Thuong, Nadia Badawi, Elizabeth J Elliott
INTRODUCTION: The epidemiology, pathogenesis, management and outcomes of cerebral palsy (CP) in low-income and middle-income countries including Vietnam are unknown because of the lack of mechanisms for standardised collection of data. In this paper, we outline the protocol for developing a hospital-based surveillance system modelled on the Paediatric Active Enhanced Disease Surveillance (PAEDS) system in Australia. Using PAEDS-Vietnam we will define the aetiology, motor function and its severity, associated impairments, and nutritional and rehabilitation status of children with CP in Hanoi, Vietnam...
November 9, 2017: BMJ Open
https://www.readbyqxmd.com/read/29122828/referrals-to-a-perinatal-specialist-palliative-care-consult-service-in-ireland-2012-2015
#12
Des L McMahon, Marie Twomey, Maeve O'Reilly, Mary Devins
OBJECTIVE: To analyse the referral patterns of perinatal patients referred to a specialist palliative care service (SPCS), their demographics, diagnoses, duration of illness, place of death and symptom profile. DESIGN: A retrospective chart review of all perinatal referrals over a 4-year period to the end of 2015. SETTING: A consultant-led paediatric SPCS at Our Lady's Children's Hospital, Crumlin, Dublin, and the Coombe Women & Infants University Hospital, Dublin...
November 9, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29111352/use-of-antibiotics-in-paediatric-long-term-care-facilities
#13
Meghan T Murray, Candace L Johnson, Bevin Cohen, Olivia Jackson, Laney K Jones, Lisa Saiman, Elaine L Larson, Natalie Neu
BACKGROUND: Adult long term care (LTC) have high rates of antibiotic use, raising concerns about antimicrobial resistance. Few studies have examined antibiotic use in paediatric LTC. AIM: We sought to describe antibiotic use in three paediatric LTC facilities and to describe the factors associated with use. METHODS: A retrospective cohort study was conducted September 2012 - December 2015 in three paediatric LTC facilities. Medical records were reviewed for demographics, healthcare-associated infections (HAI), antimicrobial use, and diagnostic testing...
October 27, 2017: Journal of Hospital Infection
https://www.readbyqxmd.com/read/29102527/functional-assessment-of-a-series-of-paediatric-patients-receiving-neurointensive-treatment-new-functional-status-scale
#14
P Madurga-Revilla, J López-Pisón, P Samper-Villagrasa, R Garcés-Gómez, J P García-Íñiguez, M Domínguez-Cajal, I Gil-Hernández, S Viscor-Zárate
INTRODUCTION: Functional health, a reliable parameter of the impact of disease, should be used systematically to assess prognosis in paediatric intensive care units (PICU). Developing scales for the assessment of functional health is therefore essential. The Paediatric Overall and Cerebral Performance Category (POPC, PCPC) scales have traditionally been used in paediatric studies. The new Functional Status Scale (FSS) was designed to provide more objective results. This study aims to confirm the validity of the FSS compared to the classic POPC and PCPC scales, and to evaluate whether it may also be superior to the latter in assessing of neurological function...
November 1, 2017: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29101144/charcot-marie-tooth-disease-in-denmark-a-nationwide-register-based-study-of-mortality-prevalence-and-incidence
#15
Signe Vaeth, Michael Vaeth, Henning Andersen, Rikke Christensen, Uffe Birk Jensen
OBJECTIVES: Charcot-Marie-Tooth disease (CMT) is the most common inherited disorder of the peripheral nervous system, yet no studies have compared the mortality in patients with CMT with that of the general population, and prevalence estimates vary considerably. We performed a nationwide register-based study to investigate the prevalence, incidence and mortality of CMT in Denmark. DESIGN: We used the Danish National Patient Registry to select all records with primary diagnostic codes for CMT between 1977 and 2012 given at a neurological, neurophysiological, paediatric or clinical genetic clinic...
November 3, 2017: BMJ Open
https://www.readbyqxmd.com/read/29079590/2017-european-league-against-rheumatism-american-college-of-rheumatology-classification-criteria-for-adult-and-juvenile-idiopathic-inflammatory-myopathies-and-their-major-subgroups
#16
Ingrid E Lundberg, Anna Tjärnlund, Matteo Bottai, Victoria P Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A Amato, Richard J Barohn, Matthew H Liang, Jasvinder A Singh, Rohit Aggarwal, Snjolaug Arnardottir, Hector Chinoy, Robert G Cooper, Katalin Dankó, Mazen M Dimachkie, Brian M Feldman, Ignacio Garcia-De La Torre, Patrick Gordon, Taichi Hayashi, James D Katz, Hitoshi Kohsaka, Peter A Lachenbruch, Bianca A Lang, Yuhui Li, Chester V Oddis, Marzena Olesinska, Ann M Reed, Lidia Rutkowska-Sak, Helga Sanner, Albert Selva-O'Callaghan, Yeong-Wook Song, Jiri Vencovsky, Steven R Ytterberg, Frederick W Miller, Lisa G Rider
OBJECTIVE: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. METHODS: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Several statistical methods were used to derive the classification criteria. RESULTS: Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived...
December 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29058318/seizure-outcome-after-corpus-callosotomy-in-a-large-paediatric-series
#17
David Graham, Deepak Gill, Russell C Dale, Martin M Tisdall
AIM: To describe 20 years of experience with corpus callosotomy at Great Ormond Street Hospital for Children, London and the Children's Hospital at Westmead, Sydney. METHOD: Records of patients who underwent corpus callosotomy between January 1995 and December 2015 were reviewed. Complications of surgery and changes in seizure type and frequency, injuries, and use of antiepileptic drugs were recorded. Drop attacks were analysed using Kaplan-Meier event-free survival curves...
October 23, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/29056524/conventional-versus-chest-compression-only-cardiopulmonary-resuscitation-by-bystanders-for-children-with-out-of-hospital-cardiac-arrest
#18
Yoshikazu Goto, Akira Funada, Yumiko Goto
AIM: It is unclear whether chest-compression-only cardiopulmonary resuscitation (CC-CPR) is therapeutically equivalent to conventional CPR for children with out-of-hospital cardiac arrest (OHCA). We aimed to determine the association of CC-CPR and conventional CPR with outcomes in discrete child patient populations with OHCA. METHODS: We analysed 6810 children (aged <18years) using Japanese registry from 2007 to 2014. Main outcomes measure was 30-day neurologically intact survival after OHCA, defined as Glasgow-Pittsburgh cerebral performance categories 1 or 2...
October 19, 2017: Resuscitation
https://www.readbyqxmd.com/read/29036580/time-to-epinephrine-and-survival-after-paediatric-out-of-hospital-cardiac-arrest
#19
Tatsuma Fukuda, Yutaka Kondo, Kei Hayashida, Hiroshi Sekiguchi, Ichiro Kukita
Aims: Delay in administration of epinephrine is associated with decreased survival among children with in-hospital cardiac arrest with an initial non-shockable rhythm. Whether this association is applicable to paediatric out-of-hospital cardiac arrest (OHCA) population remains unknown. We aimed to determine whether time to epinephrine administration is associated with outcomes in paediatric OHCA. Methods and results: This was a nation-wide population-based study of paediatric OHCA in Japan from 2005 to 2012 based on data from the All-Japan Utstein Registry...
July 11, 2017: European Heart Journal. Cardiovascular Pharmacotherapy
https://www.readbyqxmd.com/read/29026617/cerebral-vasculitis-and-intracranial-multiple-aneurysms-in-a-child-with-lyme-neuroborreliosis
#20
Elisa Kortela, Jukka Hytönen, Jussi Numminen, Margit Overmyer, Harri Saxen, Jarmo Oksi
Introduction. Lyme borreliosis is a multisystem tick-borne disease caused by Borrelia burgdorferi. Neurological manifestations are reported in up to 15 % of adult patients with Lyme disease, while the frequency among children is higher. The most common manifestations are painful radiculopathy, facial nerve paresis and lymphocytic meningitis. Epileptic seizures and cerebral vasculitis with stroke or aneurysms are very rare complications. Case presentation. We describe a paediatric patient with sensorineural auditory dysfunction, headache, fatigue and epileptic seizures as sequelae of meningoencephalitis/Lyme neuroborreliosis (LNB) caused by B...
April 2017: JMM Case Reports
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