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

Neuromyelitis optica spectrum disorders in children and adolescents

Silvia Tenembaum, Tanuja Chitnis, Ichiro Nakashima, Nicolas Collongues, Andrew McKeon, Michael Levy, Kevin Rostasy
First published August 29, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002824
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Abstract

Neuromyelitis optica (NMO) is a severe autoimmune disease of the CNS characterized by recurrent inflammatory events primarily involving the optic nerves and spinal cord. NMO is infrequent in children, but early recognition is important to start adequate treatment. In this article, we review the evolving diagnostic criteria of NMO and provide an update on the clinical and neuroimaging spectrum of the disorder in pediatric patients, including current knowledge on immunopathogenesis and treatment recommendations for children with NMO.

GLOSSARY

AQP4=
aquaporin-4;
AZA=
azathioprine;
CDC=
complement-mediated cytotoxicity;
IgG=
immunoglobulin G;
IPND=
International Panel for NMO Diagnosis;
IVIg=
IV immunoglobulin;
LETM=
longitudinally extensive transverse myelitis;
MMF=
mycophenolate mofetil;
MOG=
myelin oligodendrocyte glycoprotein;
MP=
methylprednisolone;
MS=
multiple sclerosis;
NMO=
neuromyelitis optica;
NMOSD=
neuromyelitis optica spectrum disorder;
ON=
optic neuritis;
PE=
plasma exchange;
RTX=
rituximab;
SLE=
systemic lupus erythematosus;
Th17=
T-helper 17

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 January 4, 2016.
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