Acute Disseminated Encephalomyelitis and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Josep Dalmau; Francesc Graus

doi:10.1017/9781108696722.011

Chapter 10 - Acute Disseminated Encephalomyelitis and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

from Section 3 - Specific Syndromes and Diseases

Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

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Josep Dalmau: Affiliation:
Universitat de Barcelona
Francesc Graus: Affiliation:
Universitat de Barcelona

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Summary

This chapter focuses on the MOG-antibody-associated disease as a distinct neurological disorder that includes several demyelinating syndromes, and it follows a monophasic or more frequently a relapsing–remitting course. In children, MOG antibody-associated disease usually presents as acute disseminated encephalomyelitis (ADEM), ADEM variants, or encephalitis that may present with seizures and isolated or predominant cortical hyperintense lesions in FLAIR MRI studies (FLAMES). In teenagers and adults the common clinical presentation is optic neuritis, myelitis, or brainstem syndromes. Some of the patients fulfil criteria of neuromyelitis optica spectrum disorders (NMOSD). Persistence of MOG antibodies is common in patients with relapses. The optimal treatment to prevent relapses has not been established. ADEM is the most frequent autoimmune encephalitis in children. The syndrome was characterized before the description of MOG antibodies and associates with distinct clinical and neuroimaging features. Brain MRI shows multiple hyperintense T2 lesions similar to those seen in anti-GABAaR encephalitis. As occur with NMOSD, ADEM is probably caused by different pathogenic mechanisms as MOG antibodies are only found in ~60% of patients. Besides MOG antibody-associated disease there are two other antibody-associated neurological syndromes that target oligodendrocytes as part of an immune attack: anti-NMDAR encephalitis and paraneoplastic encephalomyelitis with CRMP5 antibodies.

Keywords

Acute disseminated encephalomyelitis (ADEM)myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease anti-GABAaR encephalitis neuromyelitis optica spectrum disorders (NMOSD)FLAIR-hyperintense lesions in anti-MOG associated encephalitis with seizures (FLAMES)anti-NMDAR encephalitis paraneoplastic encephalomyelitis and CRMP5 antibodies

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Type: Chapter
Information: Autoimmune Encephalitis and Related Disorders of the Nervous System , pp. 290 - 314

DOI: https://doi.org/10.1017/9781108696722.011 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2022

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