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European Journal of Paediatric Neurology: EJPN

Elegast Monbaliu, Paul De Cock, Lisa Mailleux, Bernard Dan, Hilde Feys
AIM: To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP). METHODS: Fifty-four participants with dyskinetic CP (mean age 14y6m, SD 4y2m, range 6-22y) were included. The Dyskinesia Impairment Scale (DIS) was used to evaluate dystonia and choreoathetosis. Activity, participation and quality of life (QOL) were assessed with the Gross Motor Function Measure (GMFM), the Functional Mobility Scale (FMS), the Jebsen-Taylor Hand Function Test (JTT), the ABILHAND-Kids Questionnaire (ABIL-K), the Life Habits Kids (LIFE-H) and the Quality of Life Questionnaire for children with CP (CP-QOL)...
September 23, 2016: European Journal of Paediatric Neurology: EJPN
Angelina R A Pikstra, Zwany Metting, Johanna M Fock, Joukje van der Naalt
BACKGROUND: Annually 14.000 children with traumatic brain injury (TBI) are admitted to the Emergency Department (ED) in the Netherlands. Presentation varies and a specific entity comprises the juvenile head trauma syndrome (JHTS) with secondary deterioration after a mild trauma. As outcome of JHTS can be fatal, early recognition is essential. AIM: To outline the epidemiology and clinical features of JHTS, in comparison to paediatric mild TBI patients without JHTS...
September 23, 2016: European Journal of Paediatric Neurology: EJPN
Janneke C A W Peijnenborgh, Sandra A M van Abeelen, Petra P M Hurks, Annick M Laridon, Sylvia Klinkenberg, Albert P Aldenkamp, Johan S H Vles, Jos G M Hendriksen
OBJECTIVE: The aim of the current study was to investigate whether total intelligence scores (FSIQ) and/or a discrepancy in intelligence can predict behavioral or emotional problems in children with neurological deficiencies. METHOD: The population consists of children with neurological deficiencies (N = 610, ranging from 6 to 17 years), referred due to concerns on the (educational) development of the child to a tertiary outpatient clinic. All children were tested with the Dutch Wechsler Intelligence Scale for Children - third edition (WISC-III-NL)...
September 23, 2016: European Journal of Paediatric Neurology: EJPN
D W S Chan, T Thomas, M Lim, S Ling, M Woodhall, A Vincent
BACKGROUND: Antibody-associated disorders of the central nervous system are increasingly recognised in adults and children. Some are known to be paraneoplastic, whereas in others an infective trigger is postulated. They include disorders associated with antibodies to N-methyl-d-aspartate receptor (NMDAR), voltage-gated potassium channel-complexes (VGKC-complex), GABAB receptor or glycine receptor (GlyR). With antibodies to NMDAR or VGKC-complexes, distinct clinical patterns are well characterised, but as more antibodies are discovered, the spectra of associated disorders are evolving...
September 12, 2016: European Journal of Paediatric Neurology: EJPN
T H Popperud, M I Boldingh, C Brunborg, K W Faiz, A T Heldal, A H Maniaol, K I Müller, M Rasmussen, K Øymar, E Kerty
BACKGROUND: The aim of this study was to assess the incidence rate and prevalence of autoimmune myasthenia gravis (MG) among children in Norway. METHODS: This retrospective population-based study was performed in Norway from January 2012 to December 2013. Cases of juvenile MG (JMG) with onset < 18 years were identified through searches in coding systems of electronic patient records at the 15 main hospitals in Norway from 1989 to 2013. In addition, the acetylcholine receptor antibody database at Haukeland University Hospital and the clinical nationwide MG database at Oslo University Hospital were searched for cases of JMG...
September 10, 2016: European Journal of Paediatric Neurology: EJPN
Hortensia Gimeno, Jean-Pierre Lin
The multidisciplinary team (MDT) approach illustrates how motor classification systems, assessments and outcome measures currently available have been applied to a national cohort of children and young people with dystonia and other hyperkinetic movement disorders (HMD) particularly with a focus on dyskinetic cerebral palsy (CP). The paper is divided in 3 sections. Firstly, we describe the service model adopted by the Complex Motor Disorders Service (CMDS) at Evelina London Children's Hospital and King's College Hospital (ELCH-KCH) for deep brain stimulation...
September 8, 2016: European Journal of Paediatric Neurology: EJPN
P Vrielynck, P Marique, S Ghariani, F Lienard, V de Borchgrave, K van Rijckevorsel, C Bonnier
OBJECTIVE: Encephalopathy with continuous spike-wave during sleep (CSWS) is a particularly difficult-to-treat childhood epileptic syndrome. This study sought to present the EEG improvement and clinical efficacy of topiramate (TPM), a broad spectrum antiepileptic drug (AED), in a series of 21 children with CSWS encephalopathy. METHODS: We retrospectively reviewed the EEG results and clinical data of children with CSWS followed-up in our institution and treated with TPM...
September 8, 2016: European Journal of Paediatric Neurology: EJPN
Laura Cif, Philippe Coubes
BACKGROUND: Heterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with related disability and limitations in functions of daily living. Consequently, there is an obvious need for efficient symptomatic therapies. METHODS AND RESULTS: Following lesional surgery to basal ganglia (BG) and thalamus, deep brain stimulation (DBS) is a more conservative and adjustable intervention to and validated for internal segment of the globus pallidus (GPi), highly efficient in treating isolated "primary" dystonia and associated symptoms such as subcortical myoclonus...
September 7, 2016: European Journal of Paediatric Neurology: EJPN
Yu Yi M Wong, E Daniëlle van Pelt, Immy A Ketelslegers, Coriene E Catsman-Berrevoets, Rogier Q Hintzen, Rinze F Neuteboom
OBJECTIVE: Acute disseminating encephalomyelitis (ADEM) is an inflammatory demyelinating disease affecting the central nervous system and mainly occurs in young children. Children who initially presented with ADEM can be diagnosed with multiple sclerosis (MS) in case new non-encephalopathic clinical symptoms occur with new lesions on MRI at least three months after onset of ADEM. We aim to study the timing of MRI abnormalities related to the evolution of clinical symptoms in our Dutch paediatric ADEM cohort...
September 6, 2016: European Journal of Paediatric Neurology: EJPN
Rahul Raman Singh, John Livingston, Ming Lim, Ian R Berry, Ata Siddiqui
BACKGROUND: We present an unusual neuroimaging finding in a young girl with genetically confirmed vanishing white matter disease and a possible response to immunotherapy. METHODS AND RESULTS: 2.5 yr old girl, presented with acute onset unsteadiness and encephalopathy following a viral illness. MRI showed global symmetric white matter abnormality, with symmetric enhancement of cranial nerves (III and V) and of cervical and lumbar roots. She received immunotherapy for her encephalopathic illness with white matter changes...
September 6, 2016: European Journal of Paediatric Neurology: EJPN
Allana Austin, Jean-Pierre Lin, Richard Selway, Keyoumars Ashkan, Tamsin Owen
BACKGROUND: Dystonia is characterised by involuntary movements and postures. Deep Brain Stimulation (DBS) is effective in reducing dystonic symptoms in primary dystonia in childhood and to lesser extent in secondary dystonia. How families and children decide to choose DBS surgery has never been explored. AIMS: To explore parental decision-making for DBS in paediatric secondary dystonia. METHODS: Data was gathered using semi-structured interviews with eight parents of children with secondary dystonia who had undergone DBS...
September 6, 2016: European Journal of Paediatric Neurology: EJPN
Babar Kayani, Mathew David Sewell, Johnson Platinum, Andre Olivier, Timothy W R Briggs, Deborah M Eastwood
BACKGROUND: Congenital indifference to pain with anhidrosis (CIPA) is a rare hereditary neuropathy, which is associated with defective sensation to noxious stimuli and autonomic dysfunction. The objective of the study was to report on the orthopaedic manifestations of this condition and provide an evidence-based approach for management. METHODS: Retrospective review of 14 consecutive patients with CIPA referred to a single tertiary centre. Mean age of diagnosis was 2...
September 6, 2016: European Journal of Paediatric Neurology: EJPN
Maëlle Biotteau, Patrice Péran, Nathalie Vayssière, Jessica Tallet, Jean-Michel Albaret, Yves Chaix
OBJECTIVE: Recent theories hypothesize that procedural learning may support the frequent overlap between neurodevelopmental disorders. The neural circuitry supporting procedural learning includes, among others, cortico-cerebellar and cortico-striatal loops. Alteration of these loops may account for the frequent comorbidity between Developmental Coordination Disorder (DCD) and Developmental Dyslexia (DD). The aim of our study was to investigate cerebral changes due to the learning and automatization of a sequence learning task in children with DD, or DCD, or both disorders...
August 12, 2016: European Journal of Paediatric Neurology: EJPN
David Pang
INTRODUCTION: Chronic pain in children has been an under-recognized problem compared to adult pain. The aim of management is to help children and their families cope with the symptoms rather than a cure. Current medical treatments to reduce pain intensity are often short lived, poorly tolerated or ineffective. RESULTS: The use of electrical stimulation to treat pain is the current basis of modern Neuromodulation at the spinal cord and has been well established as spinal cord stimulation in adult practice...
August 9, 2016: European Journal of Paediatric Neurology: EJPN
Fatima Yousif Ismail, Ali Fatemi, Michael V Johnston
BACKGROUND: Neuroplasticity refers to the inherently dynamic biological capacity of the central nervous system (CNS) to undergo maturation, change structurally and functionally in response to experience and to adapt following injury. This malleability is achieved by modulating subsets of genetic, molecular and cellular mechanisms that influence the dynamics of synaptic connections and neural circuitry formation culminating in gain or loss of behavior or function. Neuroplasticity in the healthy developing brain exhibits a heterochronus cortex-specific developmental profile and is heightened during "critical and sensitive periods" of pre and postnatal brain development that enable the construction and consolidation of experience-dependent structural and functional brain connections...
August 9, 2016: European Journal of Paediatric Neurology: EJPN
Margaret Kaminska, Sarah Perides, Daniel E Lumsden, Vasiliki Nakou, Richard Selway, Keyoumars Ashkan, Jean-Pierre Lin
Deep brain stimulation (DBS) has been increasingly used for primary and secondary movement disorders in children and young people. Reports of hardware related complications have been sparse for this population and from small cohorts of patients. We report DBS complications from a single large DBS centre with 10 year experience. Data was collected as a prospective audit and additionally from a questionnaire on recharging of the stimulators. 129 patients with a minimum 6 months follow up were identified, mean age10...
August 3, 2016: European Journal of Paediatric Neurology: EJPN
V E Hudson, A Elniel, I Ughratdar, B Zebian, R Selway, J P Lin
: Cochlear implants for sensorineural deafness in children is one of the most successful neuromodulation techniques known to relieve early chronic neurodisability, improving activity and participation. In 2012 there were 324,000 recipients of cochlear implants globally. AIM: To compare cochlear implant (CI) neuromodulation with deep brain stimulation (DBS) for dystonia in childhood and explore relations between age and duration of symptoms at implantation and outcome...
August 3, 2016: European Journal of Paediatric Neurology: EJPN
Coriene Catsman-Berrevoets
No abstract text is available yet for this article.
November 2016: European Journal of Paediatric Neurology: EJPN
Mathieu Simonin, Pierre Corbeau, Philippe Durand, Michelle Rosenzwajg, Anne Filleron, Tu-Anh Tran
INTRODUCTION: Systemic capillary leak syndrome (SCLS) is a rare disease characterized by recurrent episodes and a triad of "leak attacks": hypovolemic shock, generalized edema, hemoconcentration and paradoxical hypoalbuminemia. CASE STUDY AND DISCUSSION: Here we report a case of pediatric idiopathic SCLS with an episode of cough and fever followed two days later by myalgia, livedo, acrocyanosis, and five days later by edema, tachycardia, hypotension, and generalized tonic-clonic seizure...
November 2016: European Journal of Paediatric Neurology: EJPN
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