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pediatric multiple sclerosis

Jeffrey Lambe, Olwen C Murphy, Shiv Saidha
PURPOSE OF REVIEW: With the recognition that neurodegeneration represents the principal substrate of disability in multiple sclerosis (MS), there has been increased strives towards identifying biomarkers for accurately quantifying and tracking neurodegeneration during the disease course. The retina provides an opportune "window" into the central nervous system (CNS) in MS, with retinal changes in MS reflecting not only local, but also global aspects of neurodegeneration and inflammation operative in the disease...
March 21, 2018: Current Treatment Options in Neurology
Selvakumar Ambika, Krishnakumar Padmalakshmi, Viswanathan Venkatraman, Olma V Noronha
BACKGROUND: Optic neuritis in children is an uncommon disorder which usually occurs after a viral illness or vaccination and, less frequently, occurs as a manifestation of a demyelinating disorder. Pediatric optic neuritis usually is bilateral and presents with optic disc edema, recovers rapidly with steroid therapy, and generally has low conversion rate to multiple sclerosis or neuromyelitis optica spectrum disorder. We report the clinical features and treatment outcomes of pediatric optic neuritis in Indian population, for which little data are available...
March 16, 2018: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Raed Alroughani, Alexey Boyko
BACKGROUND: Pediatric-onset multiple sclerosis (POMS) prevalence and incidence rates are increasing globally. No disease-modifying therapy are approved for MS pediatric population. Hence, we aim to review the literature on POMS to guide treating physicians on the current understanding of diagnosis and management of pediatric MS. METHODS: The authors performed a literature search and reviewed the current understanding on risk factors and disease parameters in order to discuss the challenges in assessing and implementing diagnosis and therapy in clinical practice...
March 9, 2018: BMC Neurology
Sherif M Hamdy, Maged Abdel-Naseer, Nevin M Shalaby, Alaa Elmazny, Marian Girgis, Mona A Nada, Amr Hassan, Husam S Mourad, Mohamed I Hegazy, Ahmed Abdelalim, Nirmeen A Kishk, Noha T Abokrysha, Shaimaa A Genedy, Ehab A Essawy, Hatem S Shehata
Introduction: Although the frequency of pediatric-onset multiple sclerosis (POMS) has increased in recent decades, it is still highly uncommon, which creates a need for the involvement of more registries from various clinical centers. Objective: To characterize the demographic, clinical, and paraclinical features of Egyptian patients with POMS. Patients and methods: A retrospective chart review study was undertaken on 237 Egyptian patients with demyelinating events which started before the age of 18 years who attended one of five tertiary referral centers in Cairo, Egypt...
2018: Neuropsychiatric Disease and Treatment
Katrin Parmar, Brenda L Banwell, Nadine Akbar, Sandra Bigi
Pediatric onset multiple sclerosis (POMS) is a rare disease with an incidence of 0.07 to 2.9/100'000 children per year. It follows a relapsing-remitting disease course and is characterized by rapid accrual of inflammatory lesions, high relapse frequency, and early cognitive impairment. Magnetic resonance imaging (MRI) plays a pivotal role in the diagnosis of POMS, and in the exclusion of other disorders mimicking POMS. Furthermore, MRI aids in disease monitoring, and in the evaluation of therapeutic efficacy in both clinical practice and clinical trials...
February 26, 2018: Neuropediatrics
Tingting Wang, Lingling Liu, Xuyong Chen, Yuqing Shen, Gaojian Lian, Nilay Shah, Andrew M Davidoff, Jun Yang, Ruoning Wang
Heightened aerobic glycolysis and glutaminolysis are characteristic metabolic phenotypes in cancer cells. Neuroblastoma (NBL), a devastating pediatric cancer, is featured by frequent genomic amplification of MYCN, a member of the Myc oncogene family that is primarily expressed in the early stage of embryonic development and required for neural crest development. Here we report that an enriched glutaminolysis gene signature is associated with MYCN amplification in children with NBL. The partial knockdown of MYCN suppresses glutaminolysis in NBL cells...
February 14, 2018: Cell Death & Disease
Tena Rosser
PURPOSE OF REVIEW: This article presents an up-to-date summary of the genetic etiology, diagnostic criteria, clinical features, and current management recommendations for the most common neurocutaneous disorders encountered in clinical adult and pediatric neurology practices. RECENT FINDINGS: The phakomatoses are a phenotypically and genetically diverse group of multisystem disorders that primarily affect the skin and central nervous system. A greater understanding of the genetic and biological underpinnings of numerous neurocutaneous disorders has led to better clinical characterization, more refined diagnostic criteria, and improved treatments in neurofibromatosis type 1, Legius syndrome, neurofibromatosis type 2, Noonan syndrome with multiple lentigines, tuberous sclerosis complex, Sturge-Weber syndrome, and incontinentia pigmenti...
February 2018: Continuum: Lifelong Learning in Neurology
Matthias Baumann, Astrid Grams, Tanja Djurdjevic, Eva-Maria Wendel, Christian Lechner, Bettina Behring, Astrid Blaschek, Katharina Diepold, Astrid Eisenkölbl, Joel Fluss, Michael Karenfort, Johannes Koch, Bahadir Konuşkan, Steffen Leiz, Andreas Merkenschlager, Daniela Pohl, Mareike Schimmel, Charlotte Thiels, Barbara Kornek, Kathrin Schanda, Markus Reindl, Kevin Rostásy
Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution...
February 8, 2018: Journal of Neurology
N Krajnc, J Oražem, Z Rener-Primec, M J Kržan
AIM: The purpose of this study is to review the Slovenian experience with the diagnostics, treatment and outcome in pediatric multiple sclerosis (MS) patients. METHODS: Children and adolescent diagnosed with MS and followed by Department of Child, Adolescent and Developmental Neurology, University Childrens' Hospital Ljubljana, between 1 January 2000 and 31 December 2012 were included. Data from patients' documentation were analyzed retrospectively to record demographic data, clinical presentation, paraclinical findings, disability progression, relapse rate and treatment strategies...
January 31, 2018: Multiple Sclerosis and related Disorders
Jennifer S Graves, Lisa F Barcellos, Steve Simpson, Anita Belman, Rui Lin, Bruce V Taylor, Anne-Louise Ponsonby, Terence Dwyer, Lauren Krupp, Emmanuelle Waubant, Ingrid A F van der Mei
BACKGROUND: While common variant non-HLA (human leukocyte antigen) alleles have been associated with MS risk, their role in disease course is less clear. We sought to determine whether established multiple sclerosis (MS) genetic susceptibility factors are associated with relapse rate in children and an independent cohort of adults with MS. METHODS: Genotyping was performed for 182 children with MS or clinically isolated syndrome with high risk for MS from two Pediatric MS Centers...
January 2018: Multiple Sclerosis and related Disorders
Esther Ganelin-Cohen, Sizilia Golderman, Regina Yeskaraev, Ayal Rozenberg, Avi Livneh, Batia Kaplan
BACKGROUND: Identifying new biomarkers is needed to overcome the diagnostic difficulties of pediatric multiple sclerosis (MS). Recently, we developed a new technique including CSF analysis of free light chain (FLC) monomers and dimers, which can improve diagnosis of adult MS. The present study has been designed to evaluate the utility of our technique for MS diagnosis in children. METHODS: Patients with MS (n=21) and non-MS demyelinating or inflammatory neurological disorders (n=35) participated in the study...
February 6, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
Amy M Lavery, Bradley N Collins, Amy T Waldman, Chantelle N Hart, Amit Bar-Or, Ruth Ann Marrie, Douglas Arnold, Julia O'Mahony, Brenda Banwell
BACKGROUND: Pediatric acquired demyelinating syndromes (ADSs) are monophasic (mono-ADS) in 70% of cases and represent the first attack of multiple sclerosis (MS) in 30%. Secondhand tobacco smoke (SHS) exposure has been implicated as a risk factor for adult-onset MS. Little is known about whether SHS presents an additive risk beyond genetic factors and other environmental exposures associated with pediatric MS. METHODS: This study examined SHS exposure in 216 children with mono-ADS and 81 children with MS...
January 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
A Koptelova, R Bikmullina, M Medvedovsky, S Novikova, A Golovteev, O Grinenko, M Korsakova, A Kozlova, N Arkhipova, A Vorobyev, A Melikyan, R Paetau, T Stroganova, L Metsähonkala
PURPOSE: Drug resistant epilepsy (DRE) is common in patients with tuberous sclerosis (TS). Interictal MEG has been shown as a valuable instrument in the presurgical workup. The goal of our study was to evaluate the role of ictal MEG in epileptogenic tuber selection, especially in patients with multiple irritative zones. METHODS: The clinical and MEG data of 23 patients with TS and DRE from two medical/research centers were reviewed. Seven pediatric patients, who had seizures during MEG recording and underwent resection or disconnection surgery, were included into the study...
December 27, 2017: Epilepsy Research
Damiano Baroncini, Mauro Zaffaroni, Lucia Moiola, Lorena Lorefice, Giuseppe Fenu, Pietro Iaffaldano, Marta Simone, Fulvia Fanelli, Francesco Patti, Emanuele D'Amico, Marco Capobianco, Antonio Bertolotto, Paolo Gallo, Monica Margoni, Silvia Miante, Nicoletta Milani, Maria Pia Amato, Isabella Righini, Paolo Bellantonio, Cinzia Scandellari, Gianfranco Costantino, Elio Scarpini, Roberto Bergamaschi, Giulia Mallucci, Giancarlo Comi, Angelo Ghezzi
BACKGROUND: Few data are available on very long-term follow-up of pediatric multiple sclerosis (MS) patients treated with disease modifying treatments (DMTs). OBJECTIVES: To present a long-term follow-up of a cohort of Pediatric-MS patients starting injectable first-line agents. METHODS: Data regarding treatments, annualized relapse rate (ARR), Expanded Disability Status Scale (EDSS) score, and serious adverse event were collected. Baseline characteristics were tested in multivariate analysis to identify predictors of disease evolution...
January 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
Ingo Lange, Italo Espinoza-Fuenzalida, Mourad Wagdy Ali, Laura Espana Serrano, Dana-Lynn T Koomoa
Neuroblastoma (NB) is the most common extra-cranial pediatric solid tumor. High-risk NB is difficult to treat due to the lack of response to current therapies and aggressive disease progression. Despite novel drugs, alternative treatments and multi-modal treatments, finding an effective treatment strategy for these patients continues to be a major challenge. The current study focuses on examining the effects of FTY-720 or fingolimod, a drug that is FDA-approved for refractory multiple sclerosis, in NB. The results showed that FTY-720 regulates multiple pathways that result in various effects on calcium signaling, ion channel activation and cell survival/death pathways...
December 15, 2017: Oncotarget
John A Snowden, Manuela Badoglio, Myriam Labopin, Sebastian Giebel, Eoin McGrath, Zora Marjanovic, Joachim Burman, John Moore, Montserrat Rovira, Nico M Wulffraat, Majid Kazmi, Raffaella Greco, Emilian Snarski, Tomas Kozak, Kirill Kirgizov, Tobias Alexander, Peter Bader, Riccardo Saccardi, Dominique Farge
Hematopoietic stem cell transplantation (HSCT) has evolved for >20 years as a specific treatment of patients with autoimmune disease (AD). Using European Society for Blood and Marrow Transplantation registry data, we summarized trends and identified factors influencing activity and outcomes in patients with AD undergoing first autologous HSCT (n = 1951; median age, 37 years [3-76]) and allogeneic HSCT (n = 105; median age, 12 years [<1-62]) in 247 centers in 40 countries from 1994 to 2015. Predominant countries of activity were Italy, Germany, Sweden, the United Kingdom, The Netherlands, Spain, France, and Australia...
December 26, 2017: Blood Advances
E Ann Yeh, Stephanie A Grover, Victoria E Powell, Gulay Alper, Brenda L Banwell, Kim Edwards, Mark Gorman, Jennifer Graves, Timothy E Lotze, Jean K Mah, Lauren Mednick, Jayne Ness, Maya Obadia, Ruth Slater, Amy Waldman, Emmanuelle Waubant, Carolyn E Schwartz
The identifying number for the article titled "Impact of an electronic monitoring device and behavioral feedback on adherence to multiple sclerosis therapies in youth: results of a randomized trial" is NCT02234713 (
December 23, 2017: Quality of Life Research
J Nicholas Brenton, Teri Schreiner, Krystle Karoscik, Meg Richter, Samantha Ferrante, Amy Waldman, Brenda Banwell
BACKGROUND: Studies have shown a negative impact on cognition and brain volume in marijuana-using adult multiple sclerosis (MS) patients and healthy adolescents. Given that onset of MS during childhood and adolescence negatively impacts brain growth and the normal maturation of neuronal networks, the addition of marijuana exposure in these youth may be even more harmful. OBJECTIVE: Determine attitudes toward and prevalence of recreational marijuana use in MS youth...
December 22, 2017: Journal of Neurology
Erin Yamamoto, Matthew Ginsberg, Mary Rensel, Manikum Moodley
Pediatric-onset multiple sclerosis (POMS), once thought to be rare, is now being diagnosed in increasing numbers in children. Despite improvements to diagnostic criteria, the diagnosis and management of POMS remains challenging. The aim of this study is to retrospectively describe a growing POMS patient population seen at a single center over a 13 year period. Epidemiologic, clinical, neuroimaging, laboratory features and therapeutic management and outcome data were collected and analyzed. These data support associations between MS and environmental triggers such as obesity and vitamin D deficiency...
January 2018: Journal of Child Neurology
Nedia Ben Achour, Ibtihel Rebai, Sarra Raddadi, Hanene Benrhouma, Hedia Klaa, Aida Rouissi, Ichraf Kraoua, Ilhem Ben Youssef Turki
Introduction: Pediatric multiple sclerosis (pMS) is a rare demyelinating disorder with an onset before the age of 18 years. In this study, we aimed to investigate the characteristics of pMS in Tunisian children. Patients and Methods: We conducted a retrospective study over 11 years (2005-2016) including all patients diagnosed with pMS according to the International Pediatric Multiple Sclerosis Study Group (IPMSSG) criteria of 2012 and followed up in a tertiary care research center...
2017: BioMed Research International
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