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pediatric neuroimmunology

Murat Özkale, Ilknur Erol, Yasemin Özkale, İlknur Kozanoğlu
Therapeutic plasma exchange (TPE) is used in the treatment of neurological, hematological, renal and autoimmune diseases with known or suspected immune pathogenesis. In comparison with neurological diseases of adults, knowledge about the use of TPE in children is incomplete. We report our experience on TPE in children with neurological diseases in a single institution and describe the underlying etiology, clinical course, treatment and outcome. We retrospectively evaluated 22 consecutive children (12 girls, 10 boys, aged 2-16 years) who underwent TPE in the pediatric intensive care unit between January 2010 and January 2017...
September 2018: Acta Neurologica Belgica
Kshama Ohja, Evelyne Gozal, Margaret Fahnestock, Lu Cai, Jun Cai, Jonathan H Freedman, Andy Switala, Ayman El-Baz, Gregory Neal Barnes
Autism spectrum disorders (ASD) are the most prevalent set of pediatric neurobiological disorders. The etiology of ASD has both genetic and environmental components including possible dysfunction of the immune system. The relationship of the immune system to aberrant neural circuitry output in the form of altered behaviors and communication characterized by ASD is unknown. Dysregulation of neurotrophins such as BDNF and their signaling pathways have been implicated in ASD. While abnormal cortical formation and autistic behaviors in mouse models of immune activation have been described, no one theory has been described to link activation of the immune system to specific brain signaling pathways aberrant in ASD...
June 2018: Neuromolecular Medicine
Margherita Nosadini, Stefano Sartori, Suvasini Sharma, Russell C Dale
Beyond the major advances produced by careful clinical-radiological phenotyping and biomarker development in autoimmune central nervous system disorders, a comprehensive knowledge of the range of available immune therapies and a deeper understanding of their action should benefit therapeutic decision-making. This review discusses the agents used in neuroimmunology and their mechanisms of action. First-line treatments typically include corticosteroids, intravenous immunoglobulin, and plasmapheresis, while for severe disease second-line "induction" agents such as rituximab or cyclophosphamide are used...
August 2017: Seminars in Pediatric Neurology
Wendy G Mitchell, Franz Blaes
Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome. They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks...
August 2017: Seminars in Pediatric Neurology
Michael R Pranzatelli, Elizabeth D Tate
BACKGROUND: Although pulse-dose dexamethasone is increasingly favored for treating pediatric opsoclonus-myoclonus syndrome (OMS), and multimodal immunotherapy is associated with improved clinical response, there have been no neuroimmunologic studies of dexamethasone-based multimodal disease-modifying therapy. METHODS: In this observational retrospective study, 19 children with OMS (with or without associated neuroblastoma) underwent multibiomarker evaluation for neuroinflammation...
August 2017: Pediatric Neurology
Michael R Pranzatelli, Elizabeth D Tate, Tyler J Allison
A longstanding question is whether neuroinflammation is present in children symptomatic for Tourette syndrome (TS) with positive streptococcal serology and throat cultures. The objective was to directly test for it using modern hypothesis-driven approaches. Profiling studies for 14 immune cell types (flow cytometry), 7 chemokines/cytokines (ELISA), oligoclonal bands, and other immunoglobulins were performed in this IRB-approved study of 5 children with TS and streptococcal markers compared to data from 26 non-inflammatory pediatric neurological controls...
August 2017: Cytokine
Anthony C Rodrigues, Peter B Kang
The evaluation and management of childhood pain syndromes of neuromuscular origin have distinct challenges, as the patterns of disease presentation and the ability of a child to describe symptoms may differ from that of an adult. Advances in scientific and clinical knowledge are leading to significant progress in the care of affected children. The genetic origins of Fabry disease and the inherited form of erythromelalgia are better understood. The increasing interest in neuroimmunology among pediatric neurologists has led to more sophisticated diagnostic and therapeutic approaches...
August 2016: Seminars in Pediatric Neurology
Giovanna Vitaliti, Omidreza Tabatabaie, Nassim Matin, Caterina Ledda, Piero Pavone, Riccardo Lubrano, Agostino Serra, Paola Di Mauro, Salvatore Cocuzza, Raffaele Falsaperla
Immunotherapeutic strategies to treat neurodegenerative disorders have inspired the scientific community. The aim of our review is to address the translational aspects of neuroimmunology to describe the efficacy of immunotherapy in the treatment of pediatric neurodegenerative disorders. In the studies we analyzed IVIG were found to be efficient in the treatment of post-streptococcal neurodegenerative disorders, even if in PANDAS, plasma-exchange (PE) showed a higher efficiency. IVIG were also successfully used in ADEM and Guillan-Barré syndrome...
2015: Human Vaccines & Immunotherapeutics
Kevin Rostasy, Hansotto Reiber
Multiple sclerosis (MS) is the most common chronic inflammatory disease of the central nervous system affecting young adults. Although adults and children share important features of the disease, they also differ in some clinical, radiological and laboratory aspects. This review focuses on the neuroimmunological findings in the cerebrospinal fluid of children with MS pointing out that there is already at earliest time of clinical manifestation a neuroimmunological pattern, which differs only in intensity of the humoral immune response but not in frequency and does not support a neuroimmunological difference between early onset from adult onset MS...
June 2009: Acta Neuropsychiatrica
Kyle A Williams, Susan E Swedo
Infections, and the resulting immune response to these infections, have recently received increased recognition as pathogenic mechanisms for neuropsychiatric disorders. Sydenham's chorea (SC), a widely recognized post-streptococcal autoimmune disorder, represents a model for this proposed pathogenesis. In SC, a dysregulated immune response to a streptococcal infection is hypothesized to result in inflammation of neuronal networks, particularly the basal ganglia nuclei. The resulting dysfunction in the basal ganglia nuclei are hypothesized to lead to a constellation of adventitious movements and psychiatric symptoms, which investigations have shown are amenable to immunomodulatory therapies...
August 18, 2015: Brain Research
Manu Hegde, Daniel H Lowenstein
Few would experience greater benefit from the development of biomarkers than those who suffer from epilepsy. Both the timing of individual seizures and the overall course of the disease are highly unpredictable, and the associated morbidity is considerable. Thus, there is an urgent need to develop biomarkers that can predict the progression of epilepsy and treatment response. Doing so may also shed light on the mechanisms of epileptogenesis and pharmacoresistance, which remain elusive despite decades of study...
2014: Biomarkers in Medicine
Amy M L Quek, Andrew McKeon, Vanda A Lennon, Jayawant N Mandrekar, Raffaele Iorio, Yujuan Jiao, Chiara Costanzi, Brian G Weinshenker, Dean M Wingerchuk, Claudia F Lucchinetti, Elizabeth A Shuster, Sean J Pittock
OBJECTIVE: To determine the sex and age distribution of aquaporin-4 (AQP4) autoimmunity using data derived from clinical service laboratory testing of 56,464 patient samples. DESIGN: Observational analysis. SETTING: Mayo Clinic Neuroimmunology Laboratory. PATIENTS: Between October 1, 2005, and January 4, 2011, 56,464 patients were tested for AQP4-IgG; 2960 (5.2%) patients were seropositive. MAIN OUTCOME MEASURE: Seropositivity for AQP4-IgG...
August 2012: Archives of Neurology
Radhika Dhamija, Deborah L Renaud, Sean J Pittock, Andrew McKeon, Daniel H Lachance, Katherine C Nickels, Elaine C Wirrell, Nancy L Kuntz, Mary D King, Vanda A Lennon
Autoimmunity targeting voltage-gated potassium channel complexes have not been systematically documented in children. Identified in the Neuroimmunology Laboratory records of Mayo Clinic were 12 seropositive children, 7 among 252 Mayo Clinic pediatric patients tested on a service basis for serologic evidence of neurologic autoimmunity (June 2008-April 2010), 4 during the assay's preimplementation validation (before June 2008) and 1 non-Mayo patient with available clinical information. Neurologic manifestations were subacute and multifocal...
April 2011: Pediatric Neurology
Adam B Lewin, Eric A Storch, Gary R Geffken, Wayne K Goodman, Tanya K Murphy
Pediatric obsessive-compulsive disorder (OCD) is a chronic neuropsychiatric condition associated with broad impairments in functioning. This paper outlines current etiological theories of OCD, providing a review of neuroanatomical, neurochemical, neuroimmunological, and cognitive-behavioral explanations. Subsequently, first-line treatment modalities are discussed (serotonin reuptake inhibitors [SRIs] and cognitive-behavioral therapy [CBT] with exposure and response prevention [E/RP]) in the context of recent pharmacological, CBT, and combined trials...
March 2006: Neuropsychiatric Disease and Treatment
V Fühlhuber, S Bick, A Kirsten, A Hahn, T Gerriets, M Tschernatsch, M Kaps, K T Preissner, F Blaes, S Altenkämper
Childhood opsoclonus-myoclonus syndrome (OMS) occurs idiopathic or, in association with a neuroblastoma, as a paraneoplastic syndrome. Since autoantibodies were identified in some patients, an autoimmune pathogenesis has been suspected. While the newly discovered B-cell activating factors BAFF and APRIL are involved in systemic autoimmune diseases, their association with neuroimmunological diseases is hardly understood. We here investigated the BAFF and APRIL levels in serum and cerebrospinal fluid (CSF) of OMS patients and their correlation with surface-binding autoantibodies...
May 29, 2009: Journal of Neuroimmunology
Tamás Constantin, Tamás Kálovics, Andrea Ponyi, Eszter Nagy, Krisztina Sallai, Léna Szabó, Miklós Garami, Judit Müller, Péter Gergely, Katalin Dankó, György Fekete, Rozália Kálmánchey
BACKGROUND: Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent venous thrombosis or arterial occlusive events and fetal losses associated with elevated levels of antiphospholipid antibodies (aPLs). MATERIAL/METHODS: The presence of antinuclear, anti-beta2-glycoprotein I, and anticardiolipin antibodies were investigated in 60 consecutive children with epilepsy who were followed up in a single Hungarian center. RESULTS: Almost 50% (28/60) of the patients were ANA positive...
April 2009: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Kendra Harris, Harvey S Singer
The neuroanatomy and neurochemistry underlying tic disorders are thought to involve corticostriatothalamocortical circuits and dysregulation of their component neurotransmitter systems. Tourette syndrome is a tic disorder that begins in childhood and follows a waxing and waning course of tic severity. Although it is generally believed to have a genetic component, its etiology has not been fully elucidated. The clinical entity pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) has led some to suggest that the pathophysiology of tics in some individuals might involve a postinfectious autoimmune component...
August 2006: Journal of Child Neurology
A J Dorta-Contreras, H Reiber
Eosinophilic meningoencephalitis due to the nematode Angiostrongylus cantonensis, which is endemic to Cuba, occurs in children and is due to accidental contact with soil snails. The course is less often fatal than in adult patients in southeastern Asia. Cerebrospinal fluid (CSF) and serum samples from 24 pediatric patients were analyzed and evaluated in CSF/serum quotient diagrams (Reiber graphs) to characterize the neuroimmunological response and the blood-CSF barrier dysfunction that occur in the course of the disease...
July 1998: Clinical and Diagnostic Laboratory Immunology
D M Tucker, J F Leckman, L Scahill, G E Wilf, R LaCamera, L Cardona, P Cohen, S Heidmann, J Goldstein, J Judge, E Snyder, A Bult, B S Peterson, R King, P Lombroso
A 12-year-old girl presented with an atypical, recurrent, increasingly treatment-resistant case of obsessive-compulsive disorder and chronic tic disorder associated with profound separation anxiety, learning difficulty, and intermittent upper respiratory symptoms. In addition to detailed reviews of history and findings from many clinical caretakers from the prior 7 years, current pediatric, psychiatric, neuropsychological, neuroimaging, and clinical laboratory data were also available. Treatment options were considered from multiple perspectives: psychoanalytically oriented psychotherapy, conventional pharmacotherapy, family interventions, cognitive-behavioral therapy, and learning-supportive strategies...
December 1996: Journal of the American Academy of Child and Adolescent Psychiatry
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