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

Differential diagnosis and evaluation in pediatric inflammatory demyelinating disorders

Kevin Rostasy, Barbara Bajer-Kornek, Sunita Venkateswaran, Cheryl Hemingway, Marc Tardieu
First published August 29, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002878
Kevin Rostasy
From the Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, University Witten/Herdecke, Germany; Department of Neurology (B.B.-K.), Medical University of Vienna, Austria; Division of Neurology (S.V.), Children's Hospital of Eastern Ontario, Ottawa, Canada; Pediatric Neurology (C.H.), Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; and National Reference Center for Inflammatory Diseases of the Brain (M.T.), Hôpitaux Universitaires Paris-Sud, University Paris-Sud, France.
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Barbara Bajer-Kornek
From the Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, University Witten/Herdecke, Germany; Department of Neurology (B.B.-K.), Medical University of Vienna, Austria; Division of Neurology (S.V.), Children's Hospital of Eastern Ontario, Ottawa, Canada; Pediatric Neurology (C.H.), Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; and National Reference Center for Inflammatory Diseases of the Brain (M.T.), Hôpitaux Universitaires Paris-Sud, University Paris-Sud, France.
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Sunita Venkateswaran
From the Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, University Witten/Herdecke, Germany; Department of Neurology (B.B.-K.), Medical University of Vienna, Austria; Division of Neurology (S.V.), Children's Hospital of Eastern Ontario, Ottawa, Canada; Pediatric Neurology (C.H.), Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; and National Reference Center for Inflammatory Diseases of the Brain (M.T.), Hôpitaux Universitaires Paris-Sud, University Paris-Sud, France.
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Cheryl Hemingway
From the Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, University Witten/Herdecke, Germany; Department of Neurology (B.B.-K.), Medical University of Vienna, Austria; Division of Neurology (S.V.), Children's Hospital of Eastern Ontario, Ottawa, Canada; Pediatric Neurology (C.H.), Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; and National Reference Center for Inflammatory Diseases of the Brain (M.T.), Hôpitaux Universitaires Paris-Sud, University Paris-Sud, France.
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Marc Tardieu
From the Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, University Witten/Herdecke, Germany; Department of Neurology (B.B.-K.), Medical University of Vienna, Austria; Division of Neurology (S.V.), Children's Hospital of Eastern Ontario, Ottawa, Canada; Pediatric Neurology (C.H.), Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; and National Reference Center for Inflammatory Diseases of the Brain (M.T.), Hôpitaux Universitaires Paris-Sud, University Paris-Sud, France.
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Citation
Differential diagnosis and evaluation in pediatric inflammatory demyelinating disorders
Kevin Rostasy, Barbara Bajer-Kornek, Sunita Venkateswaran, Cheryl Hemingway, Marc Tardieu
Neurology Aug 2016, 87 (9 Supplement 2) S28-S37; DOI: 10.1212/WNL.0000000000002878

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Abstract

Major advances have been made in the clinical and radiologic characterization of children presenting with the different forms of an acquired inflammatory demyelinating syndrome (ADS) such as acute disseminating encephalomyelitis, neuromyelitis optica spectrum disorders, and clinically isolated syndromes. Nevertheless, a proportion of cases that present with similar symptoms are due to a broad spectrum of other inflammatory disorders affecting the white matter, primary CNS tumors, or neurometabolic diseases. The clinician therefore has to be aware of the different forms of ADS, the risk factors for a chronic-relapsing course, and features that indicate an alternative diagnosis. The goal of this article is therefore to provide an outline of a pathway for evaluating pediatric patients with a presumed inflammatory demyelinating disorder and discussing the spectrum of the more common differential diagnoses.

GLOSSARY

ADEM=
acute disseminating encephalomyelitis;
ADS=
acquired demyelinating syndromes;
ANE=
acute necrotizing encephalopathy;
AQP4=
aquaporin-4;
BBE=
Bickerstaff brainstem encephalitis;
BBGD=
biotin-responsive basal ganglia disease;
CIS=
clinically isolated syndromes;
DIS=
dissemination in space;
DIT=
dissemination in time;
GBS=
Guillain-Barré syndrome;
HLH=
hemophagocytic lymphohistiocytosis;
IgG=
immunoglobulin G;
LCH=
Langerhans cell histiocytosis;
LETM=
longitudinally extensive transverse myelitis;
MOG=
myelin oligodendrocyte glycoprotein;
MS=
multiple sclerosis;
NMOSD=
neuromyelitis optica spectrum disorders;
ON=
optic neuritis;
PACNS=
primary angiitis of the CNS;
PCNSL=
primary CNS lymphoma;
PML=
progressive multifocal leukoencephalopathy;
SLE=
systemic lupus erythematosus;
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 4, 2016.
  • © 2016 American Academy of Neurology
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  • Article
    • Abstract
    • GLOSSARY
    • ACQUIRED DEMYELINATING SYNDROMES
    • RED FLAGS SUGGESTING AN ALTERNATIVE DIAGNOSIS IN CHILDREN WITH ADS
    • INFLAMMATORY DISEASES OF THE WHITE MATTER OTHER THAN ADS
    • TUMORS
    • NEUROMETABOLIC DISEASES
    • MIGRAINE
    • DISCUSSION
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • Footnotes
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