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9 results

  • Age of Onset as a Moderator of Cognitive Decline in Pediatric-Onset Multiple Sclerosis

  • Banafsheh Hosseini, David B. Flora, Brenda L. Banwell, Christine Till
  • Journal:
    Journal of the International Neuropsychological Society / Volume 20 / Issue 8 / September 2014
    Published online by Cambridge University Press:
    17 July 2014, pp. 796-804

  • Cognitive impairment is often reported in pediatric-onset multiple sclerosis (MS). Using serial cognitive data from 35 individuals with pediatric-onset MS, this study examined how age at disease-onset and proxies of cognitive reserve may impact cognitive maturation over the course of childhood and adolescence. Neuropsychological evaluations were conducted at baseline and up to four more assessments. Of the 35 participants, 7 completed only one assessment, 5 completed two assessments, 13 completed three assessments, 10 completed four or more assessments. Growth curve modeling was used to assess longitudinal trajectories on the Trail Making Test-Part B (TMT-B) and the Symbol Digit Modalities (SDMT; oral version) and to examine how age at disease onset, baseline Full Scale IQ, and social status may moderate rate of change on these measures. Mean number of evaluations completed per patient was 2.8. Younger age at disease onset was associated with a greater likelihood of cognitive decline on both the TMT-B (p=.001) and SDMT (p=.005). Baseline IQ and parental social status did not moderate any of the cognitive trajectories. Findings suggest that younger age at disease-onset increases the vulnerability for disrupted performance on measures of information processing, visual scanning, perceptual/motor speed, and working memory. Proxies of cognitive reserve did not protect against the progression of decline on these measures. Young patients with MS should be advised to seek follow-up cognitive evaluation to assess cognitive maturation and to screen for the potential late emergence of cognitive deficits. (JINS, 2014, 20, 1–9)

  • Chapter 21 - Recurrent isolated optic neuritis in the pediatric population

  • from Section 5 - Optic Neuritis in Children
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 234-242

  • 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 14 - Living with pediatric multiple sclerosis: patient well-being

  • from Section 2 - Pediatric MS Course and Treatment
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 144-156

  • 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 9 - Corticosteroids in pediatric multiple sclerosis relapses

  • from Section 2 - Pediatric MS Course and Treatment
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 92-95

  • 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 13 - Cognitive dysfunction in pediatric-onset multiple sclerosis

  • from Section 2 - Pediatric MS Course and Treatment
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 134-143

  • 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 8 - Plasma exchange and IV immunoglobulin for acute demyelinating relapses

  • from Section 2 - Pediatric MS Course and Treatment
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 87-91

  • 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 19 - Treatment and prognosis of acute disseminated encephalomyelitis

  • from Section 4 - ADEM
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 212-222

  • 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.

  • Chapter 18 - MRI features of acute disseminated encephalomyelitis

  • from Section 4 - ADEM
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 202-211

  • 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 20 - Pediatric optic neuritis

  • from Section 5 - Optic Neuritis in Children
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 223-233

  • 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.