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Pediatric demyelinating disease

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https://www.readbyqxmd.com/read/27909013/comparison-of-cerebrospinal-fluid-opening-pressure-in-children-with-demyelinating-disease-to-children-with-primary-intracranial-hypertension
#1
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
https://www.readbyqxmd.com/read/27882734/novel-mutations-in-sh3tc2-in-a-young-japanese-girl-with-charcot-marie-tooth-disease-type-4c
#2
Kazushi Ichikawa, Keita Numasawa, Saoko Takeshita, Akihiro Hashiguchi, Hiroshi Takashima
Charcot-Marie-Tooth disease type 4C (CMT4C) is an autosomal recessive demyelinating form of CMT characterized clinically by early onset and severe spinal deformities, and is caused by mutations in SH3TC2. We describe the case of a 10-year-old Japanese girl diagnosed with CMT4C. The patient developed progressive foot deformities such as marked pes cavus and ankle contracture, with mild muscle weakness in both legs, and generalized areflexia. On electrophysiological studies, motor nerve conduction velocity ranged from 22...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27874494/pediatric-multiple-sclerosis-updates-in-epidemiology-clinical-features-and-management
#3
Sona Narula
Consensus definitions for acute demyelinating syndromes in children have led to increased recognition of pediatric multiple sclerosis and improved our understanding of its pathogenesis, epidemiology and treatment. An estimated 2-10% of MS patients experience their first clinical symptom in childhood. Multiple genetic and environmental risk factors have been identified in the pathogenesis of pediatric MS, although further research to determine their interplay is required. Clinical trials of emerging disease-modifying therapies in children are nearing completion...
December 2016: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/27857797/recurrent-acute-disseminated-encephalomyelitis-a-favorable-outcome-among-recurrent-brain-diseases-in-pediatric-patient
#4
Anirban Chatterjee, Supratim Datta
Acute disseminated encephalomyelitis (ADEM) is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) and considered mostly a monophasic course. Recurrence of ADEM is rare entity, posing diagnostic dilemma with multiple sclerosis (MS). There were no definite diagnostic criteria or established treatment for ADEM. International Pediatric MS Study Group laid down first consensus definition. We report a boy presented with recurrent episodes of fever, paraparesis, seizure, and unconsciousness...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27793469/challenges-in-pediatric-chronic-inflammatory-demyelinating-polyneuropathy
#5
Göknur Haliloğlu, Deniz Yüksel, Cağri Mesut Temoçin, Haluk Topaloğlu
Chronic inflammatory demyelinating neuropathy, a treatable immune-mediated disease of the peripheral nervous system is less common in childhood compared to adults. Despite different sets of diagnostic criteria, lack of a reliable biologic marker leads to challenges in diagnosis, follow-up and treatment. Our first aim was to review clinical presentation, course, response to treatment, and prognosis in our childhood patients. We also aimed to document diagnostic and therapeutic pitfalls and challenges at the bedside...
September 22, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27746022/association-between-demyelinating-disease-and-autoimmune-rheumatic-disease-in-a-pediatric-population
#6
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...
September 28, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27648197/pattern-and-outcome-of-acute-disseminated-encephalomyelitis-adem-in-children-experience-in-a-tertiary-center-upper-egypt
#7
Abdelrahim Abdrabou Sadek, Mostafa Ashry Mohamed, Ashraf Abou-Taleb, Marwa Ibrahim Mohammed
INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the brain. Although it occurs in all ages, most reported cases are in children and adolescents. The aims of this study were to study the clinical pattern and outcome of ADEM in children in a tertiary center in Upper Egypt and to determine the effect of combined use of steroids and IVIg on outcome. METHODS: This observational study was carried out from January 2014 through December 2014 in the Pediatric Department of Sohag University Hospital (Egypt)...
July 2016: Electronic Physician
https://www.readbyqxmd.com/read/27640189/multiple-sclerosis-in-pediatrics-current-concepts-and-treatment-options
#8
REVIEW
Jasna Jancic, Blazo Nikolic, Nikola Ivancevic, Vesna Djuric, Ivan Zaletel, Dejan Stevanovic, Sasa Peric, John N van den Anker, Janko Samardzic
Multiple sclerosis (MS) is a chronic, autoimmune, inflammatory, demyelinating disease of the central nervous system. MS is increasingly recognized in the pediatric population, and it is usually diagnosed around 15 years of age. The exact etiology of MS is still not known, although autoimmune, genetic, and environmental factors play important roles in its development, making it a multifactorial disease. The disease in children almost always presents in the relapsing-remittent form. The therapy involves treatment of relapses, and immunomodulatory and symptomatic treatment...
December 2016: Neurology and Therapy
https://www.readbyqxmd.com/read/27572866/consensus-definitions-for-pediatric-ms-and-other-demyelinating-disorders-in-childhood
#9
Marc Tardieu, Brenda Banwell, Jerry S Wolinsky, Daniela Pohl, Lauren B Krupp
In light of the published 2012 International Pediatric Multiple Sclerosis Group definitions for pediatric multiple sclerosis (MS) and related disorders and given that pediatric-onset MS is now formally included in the 2010 McDonald criteria for MS, we sought to review these criteria and summarize their application in children with acquired CNS demyelination. In addition, proposals are made for definitions of no evidence of disease activity and inadequate treatment response that are important because of new therapeutic options and trials...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27572864/pediatric-acquired-cns-demyelinating-syndromes-features-associated-with-multiple-sclerosis
#10
Rogier Q Hintzen, Russell C Dale, Rinze F Neuteboom, Soe Mar, Brenda Banwell
Approximately one-third of children with an acquired demyelinating syndrome (ADS) will be diagnosed with multiple sclerosis (MS), either at onset according to the 2010 McDonald criteria, or on the basis of clinical or MRI evidence of relapsing disease, in the majority of patients within 2-4 years. ADS in adolescents, female patients, and patients with polyfocal deficits is associated with the highest likelihood of MS, while children with acute disseminated encephalomyelitis, those with documented preceding infection, and ADS presentation in young children more commonly portends a monophasic outcome...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27572862/pediatric-optic-neuritis
#11
E Ann Yeh, Jennifer S Graves, Leslie A Benson, Evangeline Wassmer, Amy Waldman
Optic neuritis (ON) is a common presenting symptom in pediatric CNS demyelinating disorders and may be associated with dramatic visual loss. Knowledge regarding clinical presentation, associated diseases, therapy, and outcomes in ON in children has grown over the past decade. These studies have shown that younger children (<10 years of age) are more likely to present with bilateral ON and older children with unilateral ON. Furthermore, studies focusing on visual recovery have shown excellent recovery of high-contrast visual acuity in the majority of children, but functional and structural studies have shown evidence of irreversible injury and functional decline after ON in children...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27572859/acute-disseminated-encephalomyelitis-updates-on-an-inflammatory-cns-syndrome
#12
Daniela Pohl, Gulay Alper, Keith Van Haren, Andrew J Kornberg, Claudia F Lucchinetti, Silvia Tenembaum, Anita L Belman
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrent ADEM has been described and defined as multiphasic disseminated encephalomyelitis. ADEM often occurs postinfectiously, although a causal relationship has never been established. ADEM and multiple sclerosis are currently viewed as distinct entities, generally distinguishable even at disease onset. However, pathologic studies have demonstrated transitional cases of yet unclear significance...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27572858/differential-diagnosis-and-evaluation-in-pediatric-inflammatory-demyelinating-disorders
#13
Kevin Rostasy, Barbara Bajer-Kornek, Sunita Venkateswaran, Cheryl Hemingway, Marc Tardieu
Major advances have been made in the clinical and radiologic characterization of children presenting with the different forms of an acquired inflammatory demyelinating syndrome (ADS) such as acute disseminating encephalomyelitis, neuromyelitis optica spectrum disorders, and clinically isolated syndromes. Nevertheless, a proportion of cases that present with similar symptoms are due to a broad spectrum of other inflammatory disorders affecting the white matter, primary CNS tumors, or neurometabolic diseases...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27572857/environmental-and-genetic-factors-in-pediatric-inflammatory-demyelinating-diseases
#14
Emmanuelle Waubant, Anne-Louise Ponsonby, Maura Pugliatti, Heather Hanwell, Ellen M Mowry, Rogier Q Hintzen
The onset of multiple sclerosis (MS) occurs in childhood in about 5% of all patients with MS. The disease in adults has a complex genetic and environmental inheritability. One of the main risk factors, also confirmed in pediatric MS, is HLA DRB1*1501 In addition to genetic factors, a large part of disease susceptibility in adults is conferred by environmental risk factors such as low vitamin D status, exposure to cigarette smoking, and remote Epstein-Barr virus (EBV) infection. In children, both exposure to cigarette smoking and prior EBV infection have been reported consistently as risk factors for MS...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27572856/immunopathophysiology-of-pediatric-cns-inflammatory-demyelinating-diseases
#15
Amit Bar-Or, Rogier Q Hintzen, Russell C Dale, Kevin Rostasy, Wolfgang Brück, Tanuja Chitnis
Elucidating pathophysiologic mechanisms underlying the spectrum of pediatric-onset CNS demyelinating diseases, particularly those that may distinguish multiple sclerosis (MS) from other entities, promises to both improve diagnostics and guide more-informed therapeutic decisions. Observations that pediatric- and adult-onset MS share the same genetic and environmental risk factors support the view that these conditions represent essentially the same illness manifesting at different ages. Nonetheless, special consideration must be given when CNS inflammation manifests in early life, at a time when multiple organs (including immune and nervous systems) are actively maturing...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27349055/-may-neuroimaging-findings-of-a-child-with-multiple-sclerosis-surprise-us-clinical-and-radiological-observation
#16
Agnieszka Biedroń, Izabela Witek, Ewa Wesołowska
BACKGROUND: Multiple sclerosis (MS) is a chronic disorder characterized by presence of demyelinating changes in the central nervous system. The disease most often affects young adults with a female predominance, but the first symptoms can also occur in children. MS symptoms in childhood are characterized by a higher incidence of sensory, cerebellar and brainstem disorders compared to adults. They are frequently multifocal especially in the youngest age groups, requiring differentiation of acute disseminated encephalomyelitis...
2016: Przegla̧d Lekarski
https://www.readbyqxmd.com/read/27349050/-apt-guidelines-for-applying-immunomodulation-with-interferons-in-the-multiple-sclerosis-at-children
#17
REVIEW
Małgorzata Steczkowska, Barbara Skowronek-Bała, Teresa Bawół
Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system. The disease usually affects young people, although it may also occur in early childhood. As well as the availability of diagnostic methods rises also the possibility of disease-modifying treatment increases. With constant development of diagnostic methods also the possibility of disease-modifying treatment (DMT) increases. Since 2012 in Poland a therapeutic program of the National Health Fund gives the possibility of using first-line therapy treatment in children/adolescents with multiple sclerosis (over 12 years of age), and since June 2015 using the interferon beta in children of 7 years old and body mass below 30 kg has been approved...
2016: Przegla̧d Lekarski
https://www.readbyqxmd.com/read/27253358/acute-disseminated-encephalomyelitis
#18
Matthew Philip Gray, Marc H Gorelick
Acute disseminated encephalomyelitis is a primarily pediatric, immune-mediated disease characterized by demyelination and polyfocal neurologic symptoms that typically occur after a preceding viral infection or recent immunization. This article presents the pathophysiology, diagnostic criteria, and magnetic resonance imaging characteristics of acute disseminated encephalomyelitis. We also present evaluation and management strategies.
June 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27217610/acute-hemorrhagic-leukoencephalitis-in-children-a-case-report
#19
Gholam Reza Khademi, Mohammad Hasan Aelami
Acute hemorrhagic leukoencephalitis (AHLE) is a rare demyelinating disease characterized by an acute rapidly progressive fulminant inflammation of the white matter. In this case report, we introduce a case of AHLE in children with an interesting and lengthy process and successful treatment. A previously healthy 13-year-old girl was admitted to the hospital because of fever and loss of consciousness. After 4 days, she was referred to our pediatric intensive care unit in Mashhad, Iran. On admission, she had right-sided parotiditis...
May 2016: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/27207447/biotinidase-deficiency-mimicking-neuromyelitis-optica-beginning-at-the-age-of-4-a-treatable-disease
#20
Barbara Girard, Chrystèle Bonnemains, Emmanuelle Schmitt, Emmanuel Raffo, Claire Bilbault
BACKGROUND: Metabolic and inflammatory conditions may lead to neurological disorders. Neuromyelitis optica spectrum disorders (NMOSDs) refer to a rare group of demyelinating diseases of the central nervous system which essentially involve the optic nerves and spinal cord. METHODS: We report a case of biotinidase deficiency (BD) initially misdiagnosed as NMOSD in a pediatric patient. RESULTS: An 8-year-old girl was initially diagnosed with NMOSD on the basis of optic neuritis (ON) associated with three episodes of longitudinally extensive transverse myelitis (LETM)...
May 6, 2016: Multiple Sclerosis: Clinical and Laboratory Research
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