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Demyelinating Disorders of the Central Nervous System in Childhood
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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 1 of 2


  • Chapter 8 - Plasma exchange and IV immunoglobulin for acute demyelinating relapses
    pp 87-91
  • View abstract

    Summary

    This chapter briefly summarizes the history of pediatric multiple sclerosis (MS) and related diseases, and current clinical and research directions. Pierre Marie observed that MS in children might be related to acute infectious diseases, syphilis or trauma, suggesting that there was some overlap with infectious or post-infectious central nervous system (CNS) diseases such as acute disseminated encephalomyelitis (ADEM). The reported cases of MS in children may have, in fact, been leukodystrophies. ADEM is classically described as monophasic illness, and one that predominantly occurs in childhood, as opposed to MS, a relapsing and remitting disease, which predominantly occurs in young adults. The study of pediatric MS provides a unique opportunity to examine factors contributing to MS pathogenesis in general, since in affected children there is a close temporal proximity between the interplay of biological, genetic and environmental factors leading to clinical expression of the disease.
  • Chapter 9 - Corticosteroids in pediatric multiple sclerosis relapses
    pp 92-95
  • View abstract

    Summary

    This chapter discusses how current operational definitions can apply to clinical practice and research settings of pediatric multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). It emphasizes strengths and limitations of these clinical practice and research settings. ADEM requires the presence of both encephalopathy and polysymptomatic presentation. Three major controversies concerning the criteria of dissemination in time relate to the fact that a consensus has not been reached about when to call pediatric MS a recurrent disease in: patients with a first ADEM-like episode who further develop non- ADEM-like episodes, patients with recurrent ADEM including more than two episodes, and patients with recurrent non-ADEM-like events such as optic neuritis (ON) or transverse myelitis (TM) without brain MRI findings and without neuromyelitis optica (NMO) IgG. The first two situations are fairly specific of the pediatric population, given the higher frequency of ADEM or ADEM-like presentations in children, especially before puberty.
  • Chapter 10 - Disease-modifying therapy and response to first-line treatment in pediatric multiple sclerosis
    pp 96-100
  • View abstract

    Summary

    Pediatric multiple sclerosis (MS), once considered a rare childhood illness, has been increasingly recognized as a disabling acquired pediatric neurological disease requiring early recognition and intervention. Males get affected with before puberty while females are more often hit around or after puberty. Race, ethnicity, and ancestry may also influence disease susceptibility and course differently. Epidemiological data clearly indicate that adult MS is a geographically related disease, with disease rates rising with an increased distance from the equator in both northern and southern hemispheres. A few candidates have been identified as associated with pediatric MS in a few epidemiological studies (neurotropic viruses, Chlamydia pneumonia, passive smoking). Seroepidemiologic and pathologic evidences have strongly suggested that prior infection with members of the Herpesviridae family may be associated with the development of MS in adulthood. The most studied member of the Herpesviridae family in MS patients has been Epstein-Barr virus (EBV).
  • Chapter 12 - Symptomatic therapy in pediatric multiple sclerosis
    pp 112-133
  • View abstract

    Summary

    This chapter articulates the clinical manifestations of multiple sclerosis (MS) in children. Defining the clinical features of MS in children is challenged by the variability of the available literature with respect to consistency in the definition of clinical features and diagnostic criteria for pediatric MS; age of inclusion; duration of clinical observation, a key issue in recognizing features that may not be present at onset or may change during the course of MS in children; and definition of disease onset. Impairment of executive functions, processing speed, working memory, and low functioning in activities related to school performances have been described in children with MS. Kurtzke's Functional System and Expanded Disability Status Scale (EDSS) is the most widely used scale to score neurological impairment in pediatric and adult MS patient. A key facet of care of children with MS involves the use of medications aimed at the reduction of relapse rate.
  • Chapter 13 - Cognitive dysfunction in pediatric-onset multiple sclerosis
    pp 134-143
  • View abstract

    Summary

    This chapter describes the specific magnetic resonance imaging (MRI) features of pediatric multiple sclerosis (MS). It also discusses proposed MRI criteria for pediatric MS and compares these to the MRI diagnostic criteria well established for adult patients who present with a first demyelinating event. MRI studies in children help to understand early pathogenic events that lead to clinical symptoms and signs of MS. MRI detects white matter pathology that represents clinically silent demyelination in adults. The absence of brain or spine lesions on the initial MRI study seems to be associated with very low risk of progression to MS. Studies in adult MS patients have shown that the initial lesion burden on brain imaging may be an important prognostic factor. The MRI criteria for acute disseminated encephalomyelitis (ADEM) include the presence of poorly defined lesions and a high lesion load, associated with thalamus and basal ganglia involvement.
  • Chapter 14 - Living with pediatric multiple sclerosis: patient well-being
    pp 144-156
  • View abstract

    Summary

    This chapter reviews the spectrum of inflammatory, infectious, metabolic, and neurodegenerative disorders that can overlap or simulate the phenotype of demyelinating disorders in children, including its relapsing-remitting nature. Careful clinical documentation, serum and cerebrospinal fluid (CSF) testing and neuroimaging will likely provide the diagnostic specificity desired to differentiate between acquired demyelinating disorders of the CNS in children and the other disorders. Careful but preliminary studies looking at MRI characteristics are attempting to identify reliable findings to differentiate between pediatric multiple sclerosis and other, clinically relapsing neurologic disorders. Validation of these early findings as well as studies to define additional risk factors, clinical features and biomarkers are needed to further improve our ability to recognize acquired CNS demyelinating disease and to differentiate it from other types of CNS lesions in children. Newer imaging modalities such as diffusion tensor imaging, magnetization transfer ratios, and volumetric analysis will likely play a future role.
  • Chapter 15 - Pediatric MS: biological presentation and research update
    pp 157-168
  • View abstract

    Summary

    In childhood-onset multiple sclerosis (MS), the cerebrospinal fluid (CSF) profile is not fundamentally different from the adult-onset MS. Magnetic resonance imaging (MRI) is the best imaging technique to detect lesions suggestive of MS in children. The initial clinical course in most patients with childhood-onset MS is relapsing-remitting. In the UCSF cohort, the initial MS event was moderate or severe in 86% of children compared with 56% of adult-onset MS. In the UCSF pediatric-onset cohort, complete recovery was seen in 66%, which was similar to adults seen at the same center, questioning whether children have less irreversible neuronal injury during their first event or/and have a better ability to repair. MS is likely the result of a complex interplay between genes and environment. Potential prognostics factors associated with the assignment of disability milestones have been examined in several cohort studies from adult centers and one cohort from child neurology centers.
  • 17 - Clinical and biological features of acute disseminated encephalomyelitis
    pp 183-201
  • View abstract

    Summary

    Plasma exchange involves non-selectively removing plasma components that are potentially pathogenic in immune-mediated diseases from a patient's blood. Intravenous immunoglobulin used for therapeutic purposes are pooled human immunoglobulins derived from the plasma of 10000-100000 healthy donors. The major component of intravenous immunoglobulins (IVIg) preparations is IgG; very small amounts of other immunoglobulins, immunoglobulin fragments, and other smaller plasma proteins are also present. IVIg affect several components of the immune system and have been used successfully in the treatment of several neuroimmunological disorders. IVIg therapy has been evaluated both as a treatment of acute demyelinating relapses as well as a maintenance therapy to alter the disease course in adult patients with multiple sclerosis (MS). This chapter focuses on studies pertaining to the treatment of acute relapses. Plasma exchange might be an effective and relatively well-tolerated treatment option for severe demyelinating relapses in MS patients.
  • Chapter 18 - MRI features of acute disseminated encephalomyelitis
    pp 202-211
  • View abstract

    Summary

    This chapter reviews the use of corticosteroids for acute relapses in pediatric multiple sclerosis (MS). Corticosteroids are used to treat inflammatory conditions as they modulate the immune system through multiple mechanisms. Corticosteroids are considered type A drugs for treating MS relapses in adults. In patients with optic neuritis (ON), high-dose intravenous methylprednisolone (IVMP) 1 g/day for 3 days has been shown to be effective at hastening recovery. Another study showed that patients receiving IVMP within 8 weeks of an acute relapse had lower mean Expanded Disability Scale Scores (EDSS) assessed at 1 and 4 weeks compared to placebo. Most adult studies suggest that high-dose IVMP should be dosed at 500-1000 mg daily for 3-5 days. Long-term use of corticosteroids is associated with numerous and substantial adverse effects. The side effects of prolonged exposure to corticosteroids include osteoporosis, neutropenia, weight gain, adrenal suppression, acne, increased skin fragility, hypertension, and psychosis.
  • Chapter 19 - Treatment and prognosis of acute disseminated encephalomyelitis
    pp 212-222
  • View abstract

    Summary

    This chapter presents the literature review on disease-modifying therapies (DMT) for children with multiple sclerosis (MS). Four first-line DMTs have been approved for treatment of relapsing-remitting (RR) MS in the adult population. They include glatiramer acetate, interferon beta (IFNB)-1a IM, IFNB-1a SC, and IFNB-1b SC. Large phase III studies showed that chronic administration of recombinant IFNB reduced the number of relapses and slowed progression of physical disability in adult patients with RR MS. Abnormalities in liver function tests (LFTs) may be pronounced in younger children taking interferon. The glatiramer acetate is designed to mimic human myelin basic protein and is postulated to induce the myelin-specific response of suppressor T-lymphocytes and to inhibit specific effector T-lymphocytes. Breakthrough disease is a concern in the pediatric MS population. Proposed consensus criteria for breakthrough disease in adults include increase in relapse number, new or recurrent MRI lesions, and worsening of cognitive or motor disability.

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