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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
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 diseaseanti-GABAaR encephalitisneuromyelitis optica spectrum disorders (NMOSD)FLAIR-hyperintense lesions in anti-MOG associated encephalitis with seizures (FLAMES)anti-NMDAR encephalitisparaneoplastic encephalomyelitis and CRMP5 antibodies
Type
Chapter
Information
Autoimmune Encephalitis and Related Disorders of the Nervous System , pp. 290 - 314
Publisher: Cambridge University Press
Print publication year: 2022

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