![]() Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome. Image adapted and re-used with permission from Springer Nature: Budhram A, Mirian A, Le C, Hosseini-Moghaddam SM, Sharma M, Nicolle MW. Corresponding subtle darkness on apparent diffusion coefficient map was seen, compatible with true diffusion restriction (not shown). On axial diffusion-weighted image there is brightness of the cortex with sparing of the subarachnoid space. The cortical hyperintensity is not well visualized on axial T2-weighted image (C). On axial T1-weighted image post-gadolinium, corresponding leptomeningeal enhancement is also seen. On axial T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) image pre-gadolinium, cortical swelling and hyperintensity of both the left frontal cortex and adjacent sulci is seen, with hypointensity of the adjacent juxtacortical white matter. Brain magnetic resonance imaging of unilateral cortical FLAIR-hyperintense Lesion in Anti-MOG-associated Encephalitis with Seizures (FLAMES)/Unilateral cerebral cortical encephalitis (UCCE). We herein review these meningo-cortical manifestations of MOGAD, with the aim of facilitating their prompt recognition when encountered in clinical practice.įigure 1. In recent years, this possibility has been supported by the dramatic rise in reports of anti-MOG-positive patients with a variety of cortical and meningeal presentations ( 19– 24). Although initially reported to be a unilateral cortical encephalitis, we noted the presence of bilateral cortical involvement and possible meningeal inflammation in a subset of cases that was suggestive of a broader disease spectrum. In 2019, we systematically reviewed the literature to better characterize this unique syndrome and proposed the term unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES Figure 1), which has since been adopted in the literature ( 12– 18). All four patients had seizures, unilateral cortical hyperintensities on brain magnetic resonance imaging (MRI) T2-fluid-attenuated inversion recovery (T2-FLAIR) sequences, and anti-MOG positivity ( 11). reported four patients who they described as having MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy, indicating a novel phenotype of MOGAD ( 11). The focus on ADEM as the primary encephalitic manifestation of MOGAD was highlighted in early recommendations on anti-MOG testing and diagnosis, which did not emphasize any other cerebral disease presentations ( 9, 10). In particular, mention of encephalitis in MOGAD has historically been in reference to acute disseminated encephalomyelitis (ADEM), which is a multifocal inflammatory demyelinating disease that presents with encephalopathy and large T2-hyperintense lesions predominantly involving the cerebral white matter ( 8). Classical manifestations of MOG antibody-associated disease (MOGAD) that were initially described in the literature included optic neuritis, myelitis, brainstem demyelination and encephalitis. Over the last decade, antibodies against myelin oligodendrocyte glycoprotein (MOG) have emerged as a biomarker of inflammatory demyelinating disease that is distinct from multiple sclerosis ( 1– 7). Subsequently, anti-MOG positivity in patients with a variety of cortical and meningeal disease presentations has been reported, indicating a broader spectrum of meningo-cortical manifestations in MOGAD that we review herein. In 2019, we systematically reviewed the literature to better characterize this unique syndrome, which we referred to as unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES). ![]() described four patients with seizures, unilateral cortical hyperintensities on brain magnetic resonance imaging T2-fluid-attenuated inversion recovery sequences, and anti-MOG positivity, indicating a potentially novel form of encephalitis in MOGAD. Initial manifestations of MOGAD that were reported in the literature included optic neuritis, myelitis, brainstem demyelination and encephalitis, with emphasis placed on acute disseminated encephalomyelitis (ADEM) as the primary encephalitic presentation. Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disease that is distinct from multiple sclerosis.
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