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  3. Demyelinating Disorders of the Central Nervous System in Childhood
  • Cited by 8
Publisher:
Cambridge University Press
Online publication date:
April 2011
Print publication year:
2011
Online ISBN:
9780511974373
DOI:
https://doi.org/10.1017/CBO9780511974373
Subjects:
Neurology and Clinical Neuroscience, Medicine, Pediatrics and Child Health
Information

Book description

Although multiple sclerosis and other disorders of myelin formation and repair are most commonly associated with adults, they can also occur in infants, children and adolescents. Up to 5 percent of those with MS experience symptoms before the age of 18, and the number of cases diagnosed is rising. There is a lack of awareness about these diseases in childhood, however, even amongst pediatric neurologists and MS specialists. Demyelinating Disorders of the Central Nervous System in Childhood provides comprehensive coverage of these diseases, highlighting throughout the differences between management in childhood and in adults. With sections dedicated to the diagnosis, course, treatment and biology of pediatric MS, detailed chapters on other childhood demyelinating diseases, including acute disseminated encephomyelitis, optic neuritis, acute complete transverse myelitis and neuromyelitis optica, are also provided. Essential reading for pediatric neurologists and MS specialists, this book will also be valuable reading for adult neurologists and pediatricians.

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Contents

Page 2 of 2

Contents
  • Chapter 20 - Pediatric optic neuritis
    pp 223-233
  • View abstract

    Summary

    In adult breakthrough disease, treatment strategies include switching to another first-line agent, adding another agent to the on-going therapy, or switching to a second-line drug such as natalizumab or immunosuppression. Therapeutic strategies such as switch to a second-line agent are typically associated with the possibility of more severe adverse events than those seen with first-line agents, including life-threatening conditions such as progressive multifocal leukoencephalopathy (PML), leukemia, secondary cancers, or infections. The primary goal of breakthrough therapy in pediatric multiple sclerosis (MS) is to prevent disease activity and disability progression in patients who have continued disease activity despite appropriate first-line therapy. Methotrexate (MTX) is a dicarboxylic acid used in the treatment of various cancers and autoimmune diseases. Combination therapy of either first-line drugs or first-line drugs with a second-line agent is sometimes used as an approach to obtain better disease control in breakthrough MS.
  • Chapter 21 - Recurrent isolated optic neuritis in the pediatric population
    pp 234-242
  • View abstract

    Summary

    In children and adolescents, multiple sclerosis (MS) has an impact on the developing central nervous system, and can result in transient or fixed deficits of gross motor and/or fine motor skills, sensory perceptual processing, bowel/bladder function, vision, balance, and coordination. In this chapter, the evaluation and care of patients with symptoms of pediatric demyelinating disease are addressed as individual systems with discussion of anatomy and typical features, evaluation measures, and treatment options, including both non-pharmacologic and pharmacologic approaches. Fatigue is the most common symptom experienced by MS patients and is adversely correlated with quality of life. Minimizing fatigue in the pediatric MS population requires a multifaceted approach of eliminating unnecessary energy demands, improving sleep hygiene and optimizing symptomatic medication and disease-modifying treatment (DMT) regimens. The PedsSQ Multidimensional Fatigue Scale has been validated and used in other pediatric chronic diseases such as cancer, inflammatory bowel disease, and rheumatological diseases.
  • Chapter 22 - What is acute transverse myelitis in children?
    pp 243-254
  • View abstract

    Summary

    This chapter covers the nature and course of pediatric-onset multiple sclerosis (POMS) related cognitive impairment, its clinical correlates, assessment considerations, impact on functional activities, and future directions directed towards improving our detection of cognitive impairment, the potential for treatments, and management of POMS-related cognitive impairment in clinical practice. Psychological distress, including depression and anxiety, is quite common in adults with MS and an important factor influencing neuropsychological performance. Cognitive functioning may be a functional result of depressive symptomatology. The onset of POMS can occur during very important developmental stages critical for social, academic, and daily functioning. Cognitive dysfunction clearly represents a major problem in children with MS. These children are at risk for poor academic performance and this could adversely affect their future ability to achieve their academic potential, go on to gainful employment, and be fully independent.
  • Chapter 23 - Neuromyelitis optica in children
    pp 255-271
  • View abstract

    Summary

    This chapter focuses on the influence of multiple sclerosis (MS) on school and psychosocial aspects of life in children and adolescents, offering practical information about how to address potential problems. The MS has substantial impacts on the lives of those diagnosed with the disorder. Both attacks and progressive symptoms associated with MS can contribute to the net burden of disease. The Expanded Disability Status Scale (EDSS) is a tool that provides a nonlinear score based predominantly on the neurologic examination to quantify this burden. MS can affect a child's cognitive functioning, including reasoning, processing, attention span, information processing and retrieval, and other thinking abilities. Cognitive dysfunction can, in turn, impact the learning and memory and, consequently, the academic performance of children with MS. Signs of psychosocial distress in children and teens include unexplained medical complaints, poor compliance with treatment plans, school refusal, and risk-taking behaviors.

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