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

Pediatric transverse myelitis

Michael Absoud, Benjamin M. Greenberg, Ming Lim, Tim Lotze, Terrence Thomas, Kumaran Deiva
First published August 29, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002820
Michael Absoud
From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France.
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Benjamin M. Greenberg
From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France.
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Ming Lim
From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France.
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Tim Lotze
From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France.
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Terrence Thomas
From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France.
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Kumaran Deiva
From Children's Neurosciences (M.A., M.L.), Evelina London Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK; Department of Neurology and Neurotherapeutics and Department of Pediatrics (B.M.G.), UT Southwestern and Childrens Health, Dallas, TX; Division of Child Neurology (T.L.), Baylor College of Medicine, Houston, TX; Texas Children's Hospital (T.L.), Houston, TX; Neurology Service (T.T.), Department of Paediatrics, KK Women's and Children's Hospital, Singapore; and Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud (K.D.), Le Kremlin-Bicêtre, France.
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Citation
Pediatric transverse myelitis
Michael Absoud, Benjamin M. Greenberg, Ming Lim, Tim Lotze, Terrence Thomas, Kumaran Deiva
Neurology Aug 2016, 87 (9 Supplement 2) S46-S52; DOI: 10.1212/WNL.0000000000002820

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Abstract

Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be differentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the Transverse Myelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some. MRI lesions are often centrally located with high T2 signal intensity involving gray and neighboring white matter. Longitudinally extensive ATM occurs in the majority. Asymptomatic lesions on brain MRI are seen in more than one-third and predict MS or NMO. The role of antibodies such as myelin oligodendrocyte glycoprotein in monophasic and relapsing ATM and their significance in therapeutic approaches remain unclear. ATM is a potentially devastating condition with variable outcome and presents significant cumulative demands on health and social care resources. Children generally have a better outcome than adults, with one-half making a complete recovery by 2 years. There is need for standardization of clinical assessment and investigation protocols to enable international collaborative studies to delineate prognostic factors for disability and relapse. There are no robust controlled trials in children or adults to inform optimal treatment of ATM, with one study currently open to recruitment. This review provides an overview of current knowledge of clinical features, investigative workup, pathogenesis, and management of ATM and suggests future directions.

Glossary

ADS=
acquired demyelinating syndrome;
AFM=
acute flaccid myelitis;
AQP4=
aquaporin-4;
ASIA=
American Spinal Injury Association;
ATM=
acute transverse myelitis;
GBS=
Guillain-Barré syndrome;
IL=
interleukin;
IVIg=
IV immunoglobulin;
LETM=
longitudinally extensive TM;
MOG=
myelin oligodendrocyte glycoprotein;
MS=
multiple sclerosis;
NMO=
neuromyelitis optica;
PLEX=
plasmapheresis;
SLE=
systemic lupus erythematosus;
TMCWG=
Transverse Myelitis Consortium Working Group

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.
  • © 2016 American Academy of Neurology
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  • Article
    • Abstract
    • Glossary
    • DEMOGRAPHICS AND CLINICAL FEATURES
    • DIFFERENTIAL DIAGNOSIS AND EVALUATION
    • MRI IN CHILDREN WITH ATM
    • PATHOLOGY, PATHOPHYSIOLOGY, AND PATHOGENESIS OF IDIOPATHIC TM
    • OUTCOMES, PROGNOSIS, AND MEASUREMENT
    • THERAPEUTIC CONSIDERATIONS
    • CONCLUSIONS AND FUTURE DIRECTIONS
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • Footnotes
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  • All Demyelinating disease (CNS)
  • Autoimmune diseases
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