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August 30, 2016; 87 (9 Supplement 2) Article

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

Amit Bar-Or, Rogier Q. Hintzen, Russell C. Dale, Kevin Rostasy, Wolfgang Brück, Tanuja Chitnis
First published August 29, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002821
Amit Bar-Or
From the Neuroimmunology Unit and Experimental Therapeutics Program (A.B.-O.), Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada; Department of Neurology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Neuroimmunology Group (R.C.D.), Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Australia; Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Datteln, Germany; Department of Neuropathology (W.B.), University Medical Center Göttingen, Georg-August-University, Göttingen, Germany; and Partners Pediatric MS Center (T.C.), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
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Rogier Q. Hintzen
From the Neuroimmunology Unit and Experimental Therapeutics Program (A.B.-O.), Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada; Department of Neurology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Neuroimmunology Group (R.C.D.), Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Australia; Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Datteln, Germany; Department of Neuropathology (W.B.), University Medical Center Göttingen, Georg-August-University, Göttingen, Germany; and Partners Pediatric MS Center (T.C.), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
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Russell C. Dale
From the Neuroimmunology Unit and Experimental Therapeutics Program (A.B.-O.), Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada; Department of Neurology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Neuroimmunology Group (R.C.D.), Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Australia; Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Datteln, Germany; Department of Neuropathology (W.B.), University Medical Center Göttingen, Georg-August-University, Göttingen, Germany; and Partners Pediatric MS Center (T.C.), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
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Kevin Rostasy
From the Neuroimmunology Unit and Experimental Therapeutics Program (A.B.-O.), Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada; Department of Neurology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Neuroimmunology Group (R.C.D.), Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Australia; Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Datteln, Germany; Department of Neuropathology (W.B.), University Medical Center Göttingen, Georg-August-University, Göttingen, Germany; and Partners Pediatric MS Center (T.C.), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
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Wolfgang Brück
From the Neuroimmunology Unit and Experimental Therapeutics Program (A.B.-O.), Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada; Department of Neurology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Neuroimmunology Group (R.C.D.), Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Australia; Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Datteln, Germany; Department of Neuropathology (W.B.), University Medical Center Göttingen, Georg-August-University, Göttingen, Germany; and Partners Pediatric MS Center (T.C.), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
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Tanuja Chitnis
From the Neuroimmunology Unit and Experimental Therapeutics Program (A.B.-O.), Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada; Department of Neurology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Neuroimmunology Group (R.C.D.), Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Australia; Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Datteln, Germany; Department of Neuropathology (W.B.), University Medical Center Göttingen, Georg-August-University, Göttingen, Germany; and Partners Pediatric MS Center (T.C.), Massachusetts General Hospital, Harvard Medical School, Boston, MA.
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Citation
Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases
Amit Bar-Or, Rogier Q. Hintzen, Russell C. Dale, Kevin Rostasy, Wolfgang Brück, Tanuja Chitnis
Neurology Aug 2016, 87 (9 Supplement 2) S12-S19; DOI: 10.1212/WNL.0000000000002821

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Abstract

Elucidating pathophysiologic mechanisms underlying the spectrum of pediatric-onset CNS demyelinating diseases, particularly those that may distinguish multiple sclerosis (MS) from other entities, promises to both improve diagnostics and guide more-informed therapeutic decisions. Observations that pediatric- and adult-onset MS share the same genetic and environmental risk factors support the view that these conditions represent essentially the same illness manifesting at different ages. Nonetheless, special consideration must be given when CNS inflammation manifests in early life, at a time when multiple organs (including immune and nervous systems) are actively maturing. CSF analysis in pediatric-onset MS points to chronic CNS inflammation, supported by observations from limited pathologic material available for study. Emerging results implicate abnormalities in both effector and regulatory T cell subsets, and potentially immune senescence, in children with MS. Although CNS-directed antibodies (including antibodies recognizing myelin antigens; Kir4.1) can be documented in pediatric-onset MS, their pathophysiologic significance (as in adults) remains unclear. This is in contrast to the presence of serum and/or CSF antibodies recognizing aquaporin-4, which, when measured using validated cell-based assays, supports the diagnosis of a neuromyelitis optica spectrum disorder, distinct from MS. Presence of anti–myelin oligodendrocyte glycoprotein antibodies documented with similar cell-based assays may also be associated with pathophysiologically distinct disease phenotypes in children. The substantial impact of pediatric-onset MS on normal brain development and function underscores the importance of elucidating both the immunobiology and neurobiology of disease. Ongoing efforts are aimed at developing and validating biological measures that define pathophysiologically distinct monophasic and chronic forms of pediatric CNS demyelination.

Glossary

ADEM=
acute disseminated encephalomyelitis;
AQP4=
aquaporin-4;
BBB=
blood-brain barrier;
CBA=
cell-based assay;
EDSS=
Expanded Disability Status Scale;
MBP=
myelin basic protein;
MOG=
myelin oligodendrocyte glycoprotein;
MS=
multiple sclerosis;
NMO=
neuromyelitis optica;
NMOSD=
NMO spectrum disorder;
OCB=
oligoclonal band;
ON=
optic neuritis;
OPC=
oligodendrocyte precursor cell;
TM=
transverse myelitis

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Received August 19, 2015.
  • Accepted in final form February 19, 2016.
  • © 2016 American Academy of Neurology
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  • Article
    • Abstract
    • Glossary
    • GENERAL ASPECTS OF CNS INFLAMMATORY DEMYELINATING DISEASES MANIFESTING IN THE YOUNG
    • PATHOLOGY OF MS
    • CSF PROFILES
    • EFFECTOR AND REGULATORY T CELLS IN PEDIATRIC MS
    • CNS-DIRECTED ANTIBODIES IN PEDIATRIC DEMYELINATING DISEASE
    • CONCLUDING COMMENTS
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • Footnotes
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