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pediatric multiple sclerosis

C C F Vasconcelos, L C S Thuler, B C Rodrigues, A B Calmon, R M P Alvarenga
BACKGROUND: The natural history of multiple sclerosis (MS) in Brazil has been available in different regions of country. There is no nationwide population-based studies that express general data in Brazil. OBJECTIVE: To review and synthesize available data about MS in Brazil. MATERIAL AND METHODS: Systematic review was performed through a search of medical literature databases to identify Brazilian studies published during 1990-2012. DATA SOURCES: PubMed, SciELO, and Lilacs...
September 20, 2016: Clinical Neurology and Neurosurgery
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
Y Yamaguchi, H Torisu, R Kira, Y Ishizaki, Y Sakai, M Sanefuji, T Ichiyama, A Oka, T Kishi, S Kimura, M Kubota, J Takanashi, Y Takahashi, H Tamai, J Natsume, S Hamano, S Hirabayashi, Y Maegaki, M Mizuguchi, K Minagawa, H Yoshikawa, J Kira, S Kusunoki, T Hara
OBJECTIVE: To investigate the clinical and epidemiologic features of pediatric acquired demyelinating syndromes (ADS) of the CNS in Japan. METHODS: We conducted a nationwide survey and collected clinical data on children with ADS aged 15 years or younger, who visited hospitals between 2005 and 2007. RESULTS: Among 977 hospitals enrolled, 723 (74.0%) responded to our inquiries and reported a total of 439 patients as follows: 244 with acute disseminated encephalomyelitis (ADEM), 117 with multiple sclerosis (MS), 14 with neuromyelitis optica (NMO), and 64 with other ADS...
October 14, 2016: Neurology
Jason C Warncke, Katie E Brodie, Erin C Grantham, Salvatore P Catarinicchia, Suhong Tong, Kimi L Kondo, Nicholas G Cost
PURPOSE: Tuberous Sclerosis Complex (TSC) is a genetic disorder characterized by the growth of hamartomas in multiple organs. Up to 80% of TSC patients will have at least one angiomyolipoma (AML) in their lifetime. In this study we aim to describe the incidence and natural history of AMLs in a pediatric TSC population, and analyze tumor growth to determine optimal renal imaging intervals in an effort to improve counseling, follow-up and treatment. METHODS: We performed a retrospective chart review of all TSC patients from 2004-2014...
September 24, 2016: Journal of Urology
Amy M Lavery, Brenda L Banwell, Geraldine Liu, Amy T Waldman
BACKGROUND: The onset of multiple sclerosis (MS) during childhood or adolescence is increasingly recognized in the United States. Administrative databases quantify healthcare utilization as measured by hospital admissions, providing insight into the impact of MS in the pediatric population. OBJECTIVE: We examine the frequency of hospital admissions for pediatric MS in the US using the Pediatric Health Information System (PHIS) database. METHODS: Data was extracted from the PHIS database using the ICD-9 code for MS (340...
September 2016: Multiple Sclerosis and related Disorders
Eda Derle, Aslı Tuncer Kurne, Bahadır Konuşkan, Rana Karabudak, Banu Anlar
BACKGROUND: The prevalence of MS starting under 18 years of age ranges between 2-10% of the total MS population. OBJECTIVE: We aimed to examine the clinical and long term follow-up data of pediatric-onset cases in our institutional MS database. METHOD: We evaluated the clinical data from the MS database of the Departments of Neurology and Pediatric Neurology of Hacettepe University Hospital. RESULTS: The clinical features of 74 patients who had experienced the first attack before age 18 years comprised 3...
September 2016: Multiple Sclerosis and related Disorders
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...
September 17, 2016: Neurology and Therapy
Klaus Rose, Thomas Müller
BACKGROUND: Both the United States (US) Food and Drug Administration (FDA) and the European Union (EU) European Medicines Agency (EMA) order pediatric clinical trials as a condition for approval of new compounds. We evaluate clinical value and likelihood of sufficient recruitment for pediatric multiple sclerosis (pMS) studies and discuss US and EU pediatric legislation with pMS as a paradigm. METHODS: We analyzed pMS clinical trials requested by the FDA and the EMA and industry-sponsored pMS studies registered on www...
September 2016: Therapeutic Advances in Neurological Disorders
Rodolfo Gonzalez, Milton H Hamblin, Jean-Pyo Lee
In neurological disorders, pathological lesions in the central nervous system (CNS) may be globally dispersed throughout the brain or localized to specific regions. Although native neural stem cells (NSCs) are present in the adult mammalian brain, intrinsic self-repair of injured adult CNS tissue is inadequate or ineffective. The brain's poor regenerative ability may be due to the fact that NSCs are restricted to discrete locations, are few in number, or are surrounded by a microenvironment that does not support neuronal differentiation...
August 15, 2016: CNS & Neurological Disorders Drug Targets
Angelo Ghezzi, Maria Pia Amato, Naila Makhani, Teri Shreiner, Jutta Gärtner, Silvia Tenembaum
Many disease-modifying therapies are currently available for adults with relapsing-remitting multiple sclerosis (MS) but none of them has been tested in pediatric MS in randomized placebo-controlled trials. At present, as suggested by observational studies and experts' guidelines, interferon-β and glatiramer acetate continue to be the standard first-line treatments for pediatric MS. Observational studies and some controlled unblinded trials have shown a positive effect of these meditations in reducing relapse rate and delaying disease progression, with an acceptable safety profile...
August 30, 2016: Neurology
Brenda Banwell, Douglas L Arnold, Jan-Mendelt Tillema, Maria A Rocca, Massimo Filippi, Bianca Weinstock-Guttman, Robert Zivadinov, Maria Pia Sormani
MRI plays a pivotal role in the diagnosis of multiple sclerosis (MS) in children, as it does in adults. The presence of multiple lesions in CNS locations commonly affected by MS, along with the presence of both enhancing and nonenhancing lesions, can facilitate a diagnosis of MS at the time of a first attack, whereas the accrual of serial lesions or new clinical attacks over time confirms the diagnosis in patients not meeting such criteria at onset. T2 and enhancing lesion accrual could serve as a primary outcome metric for pediatric MS clinical trials of selected therapies with anti-inflammatory activity in order to facilitate feasible trial size numbers...
August 30, 2016: Neurology
Maria Pia Amato, Lauren B Krupp, Leigh E Charvet, Iris Penner, Christine Till
In comparison with the large body of evidence on cognitive functioning in adults with multiple sclerosis (MS), there is limited information on cognition in pediatric-onset MS (POMS). Unique vulnerabilities in POMS can derive from having a disease that occurs during key periods of age-expected brain growth, active myelination in the CNS, and maturation of neural networks during the learning curve and key formative years in the academic career of the patient. Therefore, the consequences of MS on developing cognitive faculties can be assessed only in the pediatric population and cannot be simply extrapolated from studies carried on in the adult population...
August 30, 2016: Neurology
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
Amy Waldman, Jayne Ness, Daniela Pohl, Isabella Laura Simone, Banu Anlar, Maria Pia Amato, Angelo Ghezzi
Multiple sclerosis (MS) in children manifests with a relapsing-remitting MS (RRMS) disease course. Acute relapses consist of new neurologic deficits persisting greater than 24 hours, in the absence of intercurrent illness, and occur with a higher frequency early in the disease as compared to adult-onset RRMS. Most pediatric patients with MS recover well from these early relapses, and cumulative physical disability is rare in the first 10 years of disease. Brainstem attacks, poor recovery from a single attack, and a higher frequency of attacks portend a greater likelihood of future disability...
August 30, 2016: Neurology
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
Michael Absoud, Benjamin M Greenberg, Ming Lim, Tim Lotze, Terrence Thomas, Kumaran Deiva
Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be differentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the Transverse Myelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some...
August 30, 2016: Neurology
Lauren B Krupp, David Rintell, Leigh E Charvet, Maria Milazzo, Evangeline Wassmer
Supporting young people with pediatric multiple sclerosis can be challenging for families and health care providers. Adolescents may be more resilient than adults in reaction to the diagnosis but can have more difficulty planning for their futures. Appropriate, sensitive, and focused health provision should include consideration of the perspective of both the patient and parents. Multidisciplinary management strategies are often effective, as are referrals to programs that enhance individual and family coping and strengthen a sense of community...
August 30, 2016: Neurology
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
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
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
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