Abstract Details

Title Case of Myelin Oligodentrocyte Glycoprotein Antibody-associated Disease (MOGAD) with FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES) in a Pediatric Patient

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (8:00 AM-9:00 AM)

Poster/Presentation
Number 14-012

Objective To describe the disease process and workup of Myelin Oligodendrocyte Glycoprotein Antibody associated Disease (MOGAD) with FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis With Seizures (FLAMES).

Background A new anti MOG Ab entity has recently been proposed, with a distinct clinical presentation from previously described MOG antibody-associated disease (MOGAD). This new entity has findings which include unilateral cortical inflammation, seen as FLAIR-hyperintense lesions, with positive anti MOG Ab, and a good response to steroids. This new entity is entitled FLAMES - FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis With Seizures (FLAMES). Here, we describe a patient with FLAMES, with unique features of clinical course. The patient is a 12-year-old female who initially experienced a new onset of left sided severe headaches, lasting several hours, and was associated with nausea, vomiting, and worsening with abrupt change in her position.

Design/Methods Her workup during admissions spanning over the course of several months included a series of serum and CSF infectious and rheumatological studies, MRIs corresponding to her varying presenting symptoms and EEGs correlating with her stereotypical events. Initial treatment included high dose steroids, plasmapheresis, IVIG, Rituximab, and mycophenolate mofetil.

Results Initial MRI of the brain with and without contrast showed T2/FLAIR hyperintensities with subtle diffusion restriction involving the left medial temporal-occipital lobes. Continuous video EEG captured multiple left temporal focal seizures. CSF showed worsening lymphocytic leukocytosis and elevated protein. MOG studies resulted positive with a titer of 1:1000.

Conclusions The entity of MOGAD with FLAMES is relatively new, as the test for MOG Antibodies has only recently become available commercially. In the setting of pediatrics, it is essential to keep in mind that its clinical presentation and trajectory is very different from MOGAD with FLAMES syndrome present in adult patients, presenting more often with acute disseminated encephalomyelitis, fevers and seizures and less often with localized optic neuritis and motor defects.

Authors/Disclosures
Michaela Scott, MD (Loma Linda University Children's Hospital) PRESENTER	Dr. Scott has nothing to disclose.
Heidi Duarte	No disclosure on file
Sally You, DO (Loma Linda University, Department of Neurology)	Dr. You has nothing to disclose.
Gregory S. Aaen, MD (Loma Linda University School of Medicine)	Dr. Aaen has nothing to disclose.

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