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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