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Pediatric demyelinating disease

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https://www.readbyqxmd.com/read/29103426/the-spectrum-of-inflammatory-acquired-demyelinating-syndromes-in-children
#1
Rinze Neuteboom, Colin Wilbur, Danielle Van Pelt, Moses Rodriguez, Ann Yeh
Acquired demyelinating syndromes in childhood comprise a spectrum of monophasic and recurrent inflammatory conditions of the central nervous system. Examples of monophasic conditions include, clinically isolated syndromes such as optic neuritis and transverse myelitis, as well as acute disseminated encephalomyelitis, whereas recurrent disorders include entities such as multiple sclerosis and neuromyelitis optica spectrum disorder. Knowledge about these disorders has expanded due to rigorously evaluated diagnostic criteria, magnetic resonance imaging features, outcomes, and serum biomarkers in these disorders...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29058268/early-diagnosed-cerebrotendinous-xanthomatosis-patients-clinical-neuroradiological-characteristics-and-therapy-results-of-a-single-center-from-turkey
#2
Tanyel Zubarioglu, Ertugrul Kiykim, Gozde Yesil, Duhan Demircioglu, Mehmet Serif Cansever, Cengiz Yalcinkaya, Cigdem Aktuglu-Zeybek
Cerebrotendinous xanthomatosis (CTX) is a lipid storage disorder caused by defective sterol 27-hydroxylase activity. In spite of subtle clinical signs beginning from childhood, CTX is generally diagnosed lately. The aim of this study is to evaluate clinical, neuroradiological findings and therapy responses of pediatric CTX patients and raise awareness to early features of disease. Patients who were molecularly diagnosed as CTX before 18 years of age were included in study. Clinical, epidemiological, radiological and genotypic features of patients and chenodeoxycholic acid (CDCA) therapy responses were reviewed retrospectively...
October 22, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29049805/health-related-quality-of-life-in-pediatric-patients-with-demyelinating-diseases-relevance-of-disability-relapsing-presentation-and-fatigue
#3
Mariella M Self, Aaron Fobian, Katherine Cutitta, Arianne Wallace, Timothy E Lotze
Decreased health-related quality of life (HRQOL) in pediatric patients with multiple sclerosis is established, but little research has examined HRQOL in the broader pediatric demyelinating disease population, and predictors of reduced HRQOL are largely unexplored. We sought to (1) compare generic HRQOL and fatigue of pediatric patients with relapsing (i.e., multiple sclerosis and neuromyelitis optica) versus monophasic demyelinating diseases (i.e., acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, clinically isolated syndrome) and (2) examine the extent to which disability, relapsing disease, and fatigue predict HRQOL...
July 21, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28987645/tumefactive-demyelinating-lesions-tdls-a-case-series-of-clinicoradiological-features
#4
Rajendra Singh Jain, Ibraheem Khan, Kapil Kandelwal, Tushar Desai
OBJECTIVE: Diagnosis of tumefactive demyelination lesions (TDLs) is challenging to neurophysician, neuroradiologist or neurosurgeon. Our objective in this study was to analyze clinicoradiological features of TDLs. PATIENTS AND METHOD: A retrospective analysis with prospective follow-up of 11 cases of TDLs was performed. Study was conducted in the neurology department of a tertiary care centre of North-West India from August 2014 to March 2017. MRI of brain and cervical spine with contrast, visual evoked potential, oligoclonal bands, serum anti-AQP4 antibody and MRS performed in most of the patients...
November 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#5
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/28904571/transcranial-magnetic-stimulation-as-an-additional-diagnostic-tool-in-children-with-acute-inflammatory-demyelinating-polyneuropathy
#6
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/28738749/an-update-on-multiple-sclerosis-in-children-diagnosis-therapies-and-prospects-for-the-future
#7
Eliza Gordon-Lipkin, Brenda Banwell
Multiple sclerosis (MS), a chronic demyelinating disease of the central nervous system, is increasingly being recognized in children and adolescents. Pediatric MS follows a relapsing-remitting course at onset, with a risk for early cognitive impairment. Areas covered: In this review, we discuss the clinical features of acute demyelinating syndromes in children and risk factors that increase the likelihood of a diagnosis of MS. We also address the application of diagnostic criteria for MS in children, immunological features, therapeutic options and psychosocial considerations for children and adolescents with MS...
October 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28675955/psychiatric-disorders-in-children-with-demyelinating-diseases-of-the-central-nervous-system
#8
Julia Pakpoor, Raph Goldacre, Klaus Schmierer, Gavin Giovannoni, Emmanuelle Waubant, Michael J Goldacre
INTRODUCTION: The profile of psychiatric disorders associated with multiple sclerosis (MS) may differ in children. We aimed to assess the risk of psychiatric disorders in children with MS and other demyelinating diseases, and vice versa. PATIENTS AND METHODS: We analyzed linked English Hospital Episode Statistics, and mortality data, 1999-2011. Cohorts were constructed of children admitted with MS and other central nervous system (CNS) demyelinating diseases. We searched for any subsequent episode of care with psychiatric disorders in these cohorts and compared to a reference cohort...
July 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28637379/neuropsychological-outcomes-of-pediatric-demyelinating-diseases-a-review
#9
Alexander Tan, Cole Hague, Benjamin M Greenberg, Lana Harder
Immune-mediated central nervous system (CNS) demyelinating diseases impact various areas of the brain, optic nerves, and/or spinal cord and can result in a wide range of neurologic symptoms including adverse cognitive outcomes. Neuropsychological outcomes in adult multiple sclerosis (MS) are well documented, while literature on such outcomes in pediatric cohorts is more limited. Furthermore, literature on neuropsychological outcomes in pediatric acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), and transverse myelitis (TM) is even more limited...
June 21, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28625338/acute-diplopia-in-the-pediatric-emergency-department-a-cohort-multicenter-italian-study
#10
Umberto Raucci, Pasquale Parisi, Nicola Vanacore, Francesco La Penna, Valentina Ferro, Lucia Calistri, Claudia Bondone, Fabio Midulla, Agnese Suppiej, Raffaele Falsaperla, Duccio Maria Cordelli, Antonella Palmieri, Alberto Verrotti, Sabrina Becciani, Sonia Aguzzi, Mario Mastrangelo, Federica Pelizza, Filippo Greco, Giulia Carbonari, Ramona Tallone, Gabriella Bottone, Italo Trenta, Stefano Masi, Maria Pia Villa, Antonino Reale
BACKGROUND: Acute diplopia (AD) is an uncommon and distressing symptom of numerous ocular and neurological conditions, with potentially serious sequelaes. No data are present in pediatrics on the presentation and management of AD. AIM: This study investigated characteristics, etiology and health care utilization of the pediatric population with AD accessed to pediatric Emergency Departments (ED), trying to identify "red flags" associated with potentially life-threatening (LT) conditions...
June 3, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28535894/association-between-demyelinating-disease-and-autoimmune-rheumatic-disease-in-a-pediatric-population
#11
Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
May 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28533781/myelin-oligodendrocyte-glycoprotein-deciphering-a-target-in-inflammatory-demyelinating-diseases
#12
REVIEW
Patrick Peschl, Monika Bradl, Romana Höftberger, Thomas Berger, Markus Reindl
Myelin oligodendrocyte glycoprotein (MOG), a member of the immunoglobulin (Ig) superfamily, is a myelin protein solely expressed at the outermost surface of myelin sheaths and oligodendrocyte membranes. This makes MOG a potential target of cellular and humoral immune responses in inflammatory demyelinating diseases. Due to its late postnatal developmental expression, MOG is an important marker for oligodendrocyte maturation. Discovered about 30 years ago, it is one of the best-studied autoantigens for experimental autoimmune models for multiple sclerosis (MS)...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28420330/anti-mog-antibody-positive-adem-following-infectious-mononucleosis-due-to-a-primary-ebv-infection-a-case-report
#13
Yoshitsugu Nakamura, Hideto Nakajima, Hiroki Tani, Takafumi Hosokawa, Shimon Ishida, Fumiharu Kimura, Kimihiko Kaneko, Toshiyuki Takahashi, Ichiro Nakashima
BACKGROUND: Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed anti-MOG antibody-positive ADEM following infectious mononucleosis (IM) due to Epstein-Barr virus (EBV) infection. CASE PRESENTATION: A 36-year-old healthy man developed paresthesia of bilateral lower extremities and urinary retention 8 days after the onset of IM due to primary EBV infection...
April 19, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28381515/monophasic-demyelination-reduces-brain-growth-in-children
#14
Bérengère Aubert-Broche, Katrin Weier, Giulia Longoni, Vladimir S Fonov, Amit Bar-Or, Ruth Ann Marrie, E Ann Yeh, Sridar Narayanan, Douglas L Arnold, Leonard H Verhey, Brenda Banwell, D Louis Collins
OBJECTIVE: To investigate how monophasic acquired demyelinating syndromes (ADS) affect age-expected brain growth over time. METHODS: We analyzed 83 pediatric patients imaged serially from initial demyelinating attack: 18 with acute disseminated encephalomyelitis (ADEM) and 65 with other monophasic ADS presentations (monoADS). We further subdivided the monoADS group by the presence (n = 33; monoADSlesion) or absence (n = 32; monoADSnolesion) of T2 lesions involving the brain at onset...
May 2, 2017: Neurology
https://www.readbyqxmd.com/read/28334875/white-matter-changes-in-paediatric-multiple-sclerosis-and-monophasic-demyelinating-disorders
#15
Giulia Longoni, Robert A Brown, Parya MomayyezSiahkal, Colm Elliott, Sridar Narayanan, Amit Bar-Or, Ruth Ann Marrie, E Ann Yeh, Massimo Filippi, Brenda Banwell, Douglas L Arnold
See Hacohen et al. (doi:10.1093/awx075) for a scientific commentary on this article. Most children who experience an acquired demyelinating syndrome of the central nervous system will have a monophasic disease course, with no further clinical or radiological symptoms. A subset will be diagnosed with multiple sclerosis, a life-long disorder. Using linear mixed effects models we examined longitudinal diffusion properties of normal-appearing white matter in 505 serial scans of 132 paediatric participants with acquired demyelinating syndromes followed for a median of 4...
May 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28323645/pediatric-multiple-sclerosis
#16
Anusha K Yeshokumar, Sona Narula, Brenda Banwell
PURPOSE OF REVIEW: This review discusses the epidemiologic factors involved in the pathogenesis of pediatric multiple sclerosis (MS), which have been the focus of numerous studies in the last several years. We also review the clinical features (including diagnostic evaluation and differential diagnosis) of, treatment approach to, and prognosis of pediatric MS. RECENT FINDINGS: Up to 10% of patients with MS have their initial demyelinating before the age of 18 years...
June 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28217155/acute-bulbar-palsy-plus-syndrome-a-rare-variant-of-guillain-barre-syndrome
#17
Sanghamitra Ray, Prakash Chand Jain
Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide both in adult and pediatric population. Although flaccid paralysis is the hallmark of this disease, there are some rare variants which may be easily missed unless suspected. Here, we present a very rare variant of GBS - acute bulbar palsy plus syndrome in a pediatric patient. A 13-year-old female child presented with right-sided lower motor neuron type of facial palsy and palsy of bilateral glossopharyngeal and vagus nerve of 2 weeks duration...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28009079/acute-disseminated-encephalomyelitis-in-china-singapore-and-japan-a-comparison-with-the-usa
#18
D L H Koelman, D C Benkeser, Y Xu, S X Neo, K Tan, M Katsuno, G Sobue, J Natsume, S Chahin, S S Mar, A Venkatesan, T Chitnis, G M Hoganson, A K Yeshokumar, P Barreras, B Majmudar, M Carone, F J Mateen
BACKGROUND AND PURPOSE: Ethnicity-related differences in the incidence of acute disseminated encephalomyelitis (ADEM) and other demyelinating diseases including multiple sclerosis and neuromyelitis optica spectrum disorders have been reported. Little is reported on the influence of ethnicity and geographical location in ADEM. METHODS: Medical records of patients who presented with ADEM (ICD-9 323.61 and 323.81) at large referral hospitals in China, Singapore and Japan (years 1992-2015) were retrospectively reviewed and data were collected in a centralized database...
February 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27974107/-an-interpretation-of-consensus-statements-on-diagnostic-criteria-for-multiple-sclerosis-and-demyelinating-diseases-of-the-central-nervous-system-in-children-2012-version
#19
Kun-Fang Yang, Yu-Cai Chen
The International Pediatric Multiple Sclerosis Study Group (IPMSSG) put forward the 2007 version of the diagnostic criteria for multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children in 2007 ("2007 version" for short). In 2012, IPMSSG proposed the new diagnostic criteria with reference to the latest research achievements of 150 members ("2012 version" for short). The 2012 version of the consensus statements covers the diagnostic criteria for acute disseminated encephalomyelitis, clinically isolated syndrome, neuromyelitis optica, and multiple sclerosis in children...
December 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/27909013/comparison-of-cerebrospinal-fluid-opening-pressure-in-children-with-demyelinating-disease-to-children-with-primary-intracranial-hypertension
#20
Bethanie Morgan-Followell, Shawn C Aylward
The authors aimed to compare the opening pressures of children with demyelinating disease to children with primary intracranial hypertension. Medical records were reviewed for a primary diagnosis of demyelinating disease, or primary intracranial hypertension. Diagnosis of demyelinating disease was made according to either the 2007 or 2012 International Pediatric Multiple Sclerosis Study Group criteria. Primary intracranial hypertension diagnosis was confirmed by presence of elevated opening pressure, normal cerebrospinal fluid composition and neuroimaging...
November 30, 2016: Journal of Child Neurology
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