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Unilateral Cortical Fluid-Attenuated Inversion Recovery–Hyperintense Lesions in Anti-Myelin Oligodendrocyte Glycoprotein–Associated Encephalitis With Seizures (FLAMES): An Under-recognized Entity

      We read with interest the article by Tutmaher et al.
      • Tutmaher M.S.
      • Chen D.F.
      • Hallman-Cooper J.
      • Holt P.J.
      • Philbrook B.
      • Gombolay G.Y.
      A stroke mimic: anti-MOG antibody-associated disorder presenting as acute hemiparesis.
      They report an 11-year-old girl with acute-onset right hemiparesis concerning for stroke. Brain magnetic resonance imaging revealed mild left frontoparietal cortical T2-weighted fluid-attenuated inversion recovery (FLAIR) hyperintensity. She was ultimately diagnosed with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)–associated disease (MOGAD). MOGAD is a recently recognized subset of central nervous system inflammatory demyelinating diseases, to which children and young adults seem particularly predisposed.
      • Jurynczyk M.
      • Messina S.
      • Woodhall M.R.
      • et al.
      Clinical presentation and prognosis in MOG-antibody disease: a UK study.
      Optic neuritis is the most common manifestation followed by acute disseminated encephalomyelitis and transverse myelitis; patients can have a monophasic or relapsing course.
      • Jurynczyk M.
      • Messina S.
      • Woodhall M.R.
      • et al.
      Clinical presentation and prognosis in MOG-antibody disease: a UK study.
      This patient’s clinicoradiographic presentation was consistent with a recently characterized MOG-IgG-positive unilateral cortical encephalitic syndrome, which is known as unilateral cortical FLAIR-hyperintense lesions in anti-MOG–associated encephalitis with seizures (FLAMES). This syndrome is defined by the presence of unilateral cortical T2-weighted FLAIR hyperintensity without involvement of the adjacent juxtacortical white matter on brain magnetic resonance imaging (Fig), some combination of seizures, headache, fever, and cortical symptoms referable to the FLAMES location (e.g., hemiparesis, aphasia) clinically, MOG-IgG positivity by cell-based assay, and exclusion of alternative diagnoses.
      • Ogawa R.
      • Nakashima I.
      • Takahashi T.
      • et al.
      MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy.
      ,
      • Budhram A.
      • Mirian A.
      • Le C.
      • Hosseini-Moghaddam S.M.
      • Sharma M.
      • Nicolle M.W.
      Unilateral cortical FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome.
      Leptomeningeal enhancement was noted in this case, which has previously been described and likely reflects a broader spectrum of MOG-IgG-positive meningoencephalitis.
      • Budhram A.
      • Mirian A.
      • Le C.
      • Hosseini-Moghaddam S.M.
      • Sharma M.
      • Nicolle M.W.
      Unilateral cortical FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome.
      ,
      • Budhram A.
      • Kunchok A.C.
      • Flanagan E.P.
      Unilateral leptomeningeal enhancement in myelin oligodendrocyte glycoprotein immunoglobulin G-associated disease.
      Evidence regarding treatment of unilateral cortical FLAMES is limited to case series, but patients seem to respond well to corticosteroids.
      • Budhram A.
      • Mirian A.
      • Le C.
      • Hosseini-Moghaddam S.M.
      • Sharma M.
      • Nicolle M.W.
      Unilateral cortical FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome.
      Recognition of this entity thus helps to ensure prompt use of immunosuppressive therapy after exclusion of other diseases; otherwise, persistent concern for infectious meningoencephalitis may understandably deter use of immunosuppressive drugs as was stated in this case. This report adds to the growing literature surrounding unilateral cortical FLAMES, a hallmark manifestation of MOGAD that pediatric and adult neurologists should consider in the appropriate clinical context.
      Figure thumbnail gr1
      FIGUREUnilateral cortical FLAMES. On brain MRI, axial T2-weighted FLAIR imaging reveals right hemispheric cortical hyperintensity without involvement of the adjacent juxtacortical white matter (red circle). FLAIR, fluid-attenuated inversion recovery; FLAMES, FLAIR-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein–associated encephalitis with seizures; MRI, magnetic resonance imaging. The color version of this figure is available in the online edition.
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      References

        • Tutmaher M.S.
        • Chen D.F.
        • Hallman-Cooper J.
        • Holt P.J.
        • Philbrook B.
        • Gombolay G.Y.
        A stroke mimic: anti-MOG antibody-associated disorder presenting as acute hemiparesis.
        Pediatr Neurol. 2020; 108: 123-125
        • Jurynczyk M.
        • Messina S.
        • Woodhall M.R.
        • et al.
        Clinical presentation and prognosis in MOG-antibody disease: a UK study.
        Brain. 2017; 140: 3128-3138
        • Ogawa R.
        • Nakashima I.
        • Takahashi T.
        • et al.
        MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy.
        Neurol Neuroimmunol Neuroinflamm. 2017; 4: e322
        • Budhram A.
        • Mirian A.
        • Le C.
        • Hosseini-Moghaddam S.M.
        • Sharma M.
        • Nicolle M.W.
        Unilateral cortical FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome.
        J Neurol. 2019; 266: 2481-2487
        • Budhram A.
        • Kunchok A.C.
        • Flanagan E.P.
        Unilateral leptomeningeal enhancement in myelin oligodendrocyte glycoprotein immunoglobulin G-associated disease.
        JAMA Neurol. 2020; 77: 648-649

      Linked Article

      • Author Response to Budhram, et al.
        Pediatric NeurologyVol. 111
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          We would like to thank Budhram et al. for their interest in our report, “A Stroke Mimic: Anti-MOG Antibody-Associated Disorder Presenting as Acute Hemiparesis.”1 As the authors pointed out,2 our patient resembles other cases that have been termed unilateral cortical FLAMES (FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures).3 In the initial description of unilateral cortical FLAMES, two pediatric patients were included in the literature review, one of whom did present with hemiparesis and lethargy.
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