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Seminars in Pediatric Neurology

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https://www.readbyqxmd.com/read/29103430/promise-progress-and-pitfalls-in-the-search-for-central-nervous-system-biomarkers-in-neuroimmunological-diseases-a-role-for-cerebrospinal-fluid-immunophenotyping
#1
Bibiana Bielekova, Michael R Pranzatelli
Biomarkers are central to the translational medicine strategic focus, though strict criteria need to be applied to their designation and utility. They are one of the most promising areas of medical research, but the "biomarker life-cycle" must be understood to avoid false-positive and false-negative results. Molecular biomarkers will revolutionize the treatment of neurological diseases, but the rate of progress depends on a bold, visionary stance by neurologists, as well as scientists, biotech and pharmaceutical industries, funding agencies, and regulators...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103429/immunotherapeutics-in-pediatric-autoimmune-central-nervous-system-disease-agents-and-mechanisms
#2
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
https://www.readbyqxmd.com/read/29103428/overview-of-inflammation-in-neurometabolic-diseases
#3
Gregory A Grabowski
Neuroinflammation is an intrinsic component of the neurodegeneration of inborn errors of neurometabolic diseases. Diseases resulting in lysosomal, peroxisomal, and autophagocytic disruption lead to neuroinflammation by different mechanisms relating to accumulated substrates and/or downstream deficiencies that cause presymptomatic microglial activation, axonal instabilities and/or direct hyperactivation of intrinsic inflammatory mechanisms. Only in selected diseases is the blood-brain barrier (BBB) breached, thereby permitting peripheral adaptive immune mechanisms to amplify intrinsic immune reactions in the central nervous system...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103427/neuroinflammation-in-ischemic-pediatric-stroke
#4
Maja Steinlin
Over the last decades, the importance of inflammatory processes in pediatric stroke have become increasingly evident. Ischemia launches a cascade of events: activation and inhibition of inflammation by a large network of cytokines, adhesion and small molecules, protease, and chemokines. There are major differences in the neonatal brain compared to adult brain, but developmental trajectories of the process during childhood are not yet well known. In neonatal stroke ischemia is the leading pathophysiology, but infectious and inflammatory processes have a significant input into the course and degree of tissue damage...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103426/the-spectrum-of-inflammatory-acquired-demyelinating-syndromes-in-children
#5
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/29103425/cancer-and-autoimmunity-paraneoplastic-neurological-disorders-associated-with-neuroblastic-tumors
#6
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
https://www.readbyqxmd.com/read/29103424/autoantibody-associated-movement-disorders-in-children-proven-and-proposed
#7
Harvey S Singer
Movement disorders secondary to autoantibodies in children represent a rapidly expanding group of conditions. Once considered to be limited to poststreptococcal Sydenham's chorea or rare cases of childhood systemic lupus erythematosus, a variety of antibody-related movement abnormalities are now seen as part of noninfectious autoimmune encephalitis or within an expanding list of postinfectious disorders. In this article, several proposed autoantibody-mediated movement disorders in children are reviewed. In each one, there is a hypothesized antibody biomarker that is believed to be pathogenic and cause the clinical symptoms...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103423/autoimmune-epilepsies
#8
Anusha K Yeshokumar, Carlos A Pardo
Autoimmune epilepsies describe clinical syndromes wherein the immune system is suspected to be involved in the pathogenesis of seizures or as a mechanism for neuronal injury following seizures. These diseases typically affect otherwise healthy children and are characterized by explosive onset of focal seizures, encephalopathy, cognitive deterioration, or other focal neurological deficits, or all of these. Traditional neurological diagnostics lack sensitivity and specificity in the diagnosis of autoimmune epilepsies, and results must be considered in the clinical context...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103422/novel-inflammatory-neuropathology-in-immature-brain-1-fetal-tuberous-sclerosis-2-febrile-seizures-3-%C3%AE-b-crystallin-and-4-role-of-astrocytes
#9
Harvey B Sarnat, Morris H Scantlebury
Though the term "inflammation" is traditionally defined as proliferation or infiltration of lymphatic cells of the lymphatic immune system and macrophages or as immunoreactive proteins including cytokines, interleukins and major histocompatibility complexes, recently recognized reactions to tissue injury also are inflammation, often occurring in the central nervous system in conditions where they previously were not anticipated and where they may play a role in both pathogenesis and repair. We highlight 4 such novel inflammatory conditions revealed by neuropathologic studies: (1) inflammatory markers and cells in the brain of human fetuses with tuberous sclerosis complex and perhaps other disorders of the mechanistic target of rapamycin genetic or metabolic pathway, (2) inflammatory markers in the brain related to febrile seizures of infancy and early childhood, (3) heat-shock protein upregulation in glial cells and neurons at sites of chronic epileptic foci, and (4) the emerging role of astrocytes in the presence of and participation in inflammation...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103421/a-neuroprimer-principles-of-central-nervous-system-immunity
#10
Gregory P Owens
Despite longstanding perceptions, robust innate and adaptive immune responses occur within the central nervous system (CNS) in response to infection and tissue damage. Although necessary to control infection, immune responses can lead to severe CNS pathology in the context of both viral infection and autoimmunity. Research into how the central nervous and immune systems communicate has accelerated over the past 20 years leading to a better understanding of pathways controlling immune activation and neuroinflammation that have guided the approval of new disease-modifying therapies to treat CNS immunopathology, particularly the inflammatory demyelinating disease multiple sclerosis...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103420/introduction
#11
EDITORIAL
Michael R Pranzatelli
No abstract text is available yet for this article.
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941532/optic-pathway-gliomas-secondary-to-neurofibromatosis-type-1
#12
Shannon Jeanine Beres, Robert A Avery
Children with neurofibromatosis type 1 frequently manifest optic pathway gliomas-low-grade gliomas intrinsic to the visual pathway. This review describes the molecular and genetic mechanisms driving optic pathway gliomas as well as the clinical symptoms of this relatively common genetic condition. Recommendations for clinical management and descriptions of the newest imaging techniques are discussed.
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941531/disentangling-how-the-brain-is-wired-in-cortical-cerebral-visual-impairment
#13
Lotfi B Merabet, D Luisa Mayer, Corinna M Bauer, Darick Wright, Barry S Kran
Cortical (cerebral) visual impairment (CVI) results from perinatal injury to visual processing structures and pathways of the brain and is the most common cause of severe visual impairment or blindness in children in developed countries. Children with CVI display a wide range of visual deficits including decreased visual acuity, impaired visual field function, as well as impairments in higher-order visual processing and attention. Together, these visual impairments can dramatically influence a child's development and well-being...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941530/introduction
#14
EDITORIAL
Gena Heidary
No abstract text is available yet for this article.
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941529/emerging-applications-of-optical-coherence-tomography-in-pediatric-optic-neuropathies
#15
Sidney M Gospe, M Tariq Bhatti, Mays A El-Dairi
Limited cooperation and attention span often lead to poorly reliable assessments of visual acuity and visual fields in children, making diagnosis and monitoring of pediatric optic neuropathies challenging. As a noninvasive imaging modality, optical coherence tomography (OCT) could offer particular utility in this patient population. OCT provides high-resolution characterization of the optic nerve head, peripapillary retinal nerve fiber layer, and cellular layers of the macula, all of which can be used to assess the severity of optic nerve disease qualitatively and quantitatively...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941528/dominant-optic-atrophy-and-leber-s-hereditary-optic-neuropathy-update-on-clinical-features-and-current-therapeutic-approaches
#16
Bo Young Chun, Joseph F Rizzo
Dominant optic atrophy (DOA) and Leber hereditary optic neuropathy (LHON) are the two most common inherited optic neuropathies encountered in clinical practice. This review provides a summary of recent advances in the understanding of the clinical manifestations, current treatments, and ongoing clinical trials of these two optic neuropathies. Substantial progress has been made in the understanding of the clinical, genetic, and pathophysiological basis of DOA and LHON. Pathogenic OPA1 gene mutations in DOA and 3 primary mutations of mitochondrial DNA in LHON-induced mitochondrial dysfunction, which in turn leads to increased reactive oxygen species levels in mitochondria and possibly insufficient ATP production...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941527/pediatric-optic-neuritis
#17
Melinda Y Chang, Stacy L Pineles
Optic neuritis is rare in children in comparison to adults, but accounts for approximately 25% of pediatric acute demyelinating syndromes. Features of pediatric optic neuritis that differ from adults include a higher rate of bilaterality, poor visual acuity on presentation, and papillitis. Diagnostic work-up includes brain magnetic resonance imaging, lumbar puncture, and blood tests to exclude infectious and inflammatory disorders. Pediatric optic neuritis may occur following infection or vaccination, or in association with a systemic demyelinating process such as acute disseminated encephalomyelitis, neuromyelitis optica, or multiple sclerosis...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941526/pediatric-myasthenia-gravis
#18
Jason H Peragallo
Myasthenia gravis is a disorder of neuromuscular transmission that leads to fatigue of skeletal muscles and fluctuating weakness. Myasthenia that affects children can be classified into the following 3 forms: transient neonatal myasthenia, congenital myasthenic syndromes, and juvenile myasthenia gravis (JMG). JMG is an autoimmune disorder that has a tendency to affect the extraocular muscles, but can also affect all skeletal muscles leading to generalized weakness and fatigability. Respiratory muscles may be involved leading to respiratory failure requiring ventilator support...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941525/pediatric-pseudotumor-cerebri-syndrome-diagnosis-classification-and-underlying-pathophysiology
#19
Claire A Sheldon, Grace L Paley, Shannon J Beres, Shana E McCormack, Grant T Liu
Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS can occur in the pediatric and adult populations and, if untreated, may lead to permanent visual loss. In this review, discussion will focus on PTCS in the pediatric population and will outline its distinct epidemiology and key elements of diagnosis, evaluation and management. Finally, although the precise mechanisms are unclear, the underlying pathophysiology will be considered...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28941524/juvenile-macular-degenerations
#20
Pablo Altschwager, Lucia Ambrosio, Emily A Swanson, Anne Moskowitz, Anne B Fulton
In this article, we review the following 3 common juvenile macular degenerations: Stargardt disease, X-linked retinoschisis, and Best vitelliform macular dystrophy. These are inherited disorders that typically present during childhood, when vision is still developing. They are sufficiently common that they should be included in the differential diagnosis of visual loss in pediatric patients. Diagnosis is secured by a combination of clinical findings, optical coherence tomography imaging, and genetic testing...
May 2017: Seminars in Pediatric Neurology
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