TY - JOUR
T1 - Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease
AU - Valencia-Sanchez, Cristina
AU - Guo, Yong
AU - Krecke, Karl N.
AU - Chen, John
AU - Redenbaugh, Vyanka
AU - Montalvo, Mayra
AU - Elsbernd, Paul M.
AU - Tillema, Jan Mendelt
AU - Lopez-Chiriboga, Sebastian
AU - Budhram, Adrian
AU - Sechi, Elia
AU - Kunchok, Amy
AU - Dubey, Divyanshu
AU - Pittock, Sean J.
AU - Lucchinetti, Claudia F.
AU - Flanagan, Eoin P.
N1 - Funding Information:
This study was funded by grant funding from the NIH National Institute of Neurological Disorders and Stroke (R01NS113828).
Funding Information:
C.V.‐S., Y.G., E.S., M.M., K.N.K., P.M.E., V.R., J.‐M.T., A.B., C.F.L., report no conflicts of interest. J.J.C. has served as a consultant for Roche and UCB, which have upcoming treatment trials in MOGAD. S.L.‐C. and A.K. have served on an advisory board for Genentech/Roche, which has an upcoming treatment trial in MOGAD. D.D. has consulted for UCB, which has an upcoming treatment trial in MOGAD. S.J.P. reports grants, personal fees, and nonfinancial support from Roche/Genentech, and personal fees for consulting services from UCB, which have upcoming treatment trials in MOGAD. E.P.F. has participated in advisory boards for Roche and UCB who have upcoming treatment trials in MOGAD. E.P.F. has received funding from the NIH (R01NS113828). Mayo Clinic Laboratories offer commercial testing for MOG‐IgG, but none of the authors receive financial compensation for this.
Publisher Copyright:
© 2022 The Authors. Annals of Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.
PY - 2022
Y1 - 2022
N2 - Cerebral cortical encephalitis (CCE) is a recently described myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) phenotype. In this observational retrospective study, we characterized 19 CCE patients (6.7% of our MOGAD cohort). Headache (n = 15, 79%), seizures (n = 13, 68%), and encephalopathy (n = 12, 63%) were frequent. Magnetic resonance imaging revealed unilateral (n = 12, 63%) or bilateral (n = 7, 37%) cortical T2 hyperintensity and leptomeningeal enhancement (n = 17, 89%). N-Methyl-D-aspartate receptor autoantibodies coexisted in 2 of 15 tested (13%). CCE pathology (n = 2) showed extensive subpial cortical demyelination (n = 2), microglial reactivity (n = 2), and inflammatory infiltrates (perivascular, n = 1; meningeal, n = 1). Most received high-dose steroids (n = 17, 89%), and all improved, but 3 had CCE relapses. This study highlights the CCE spectrum and provides insight into its pathogenesis. ANN NEUROL 2022.
AB - Cerebral cortical encephalitis (CCE) is a recently described myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) phenotype. In this observational retrospective study, we characterized 19 CCE patients (6.7% of our MOGAD cohort). Headache (n = 15, 79%), seizures (n = 13, 68%), and encephalopathy (n = 12, 63%) were frequent. Magnetic resonance imaging revealed unilateral (n = 12, 63%) or bilateral (n = 7, 37%) cortical T2 hyperintensity and leptomeningeal enhancement (n = 17, 89%). N-Methyl-D-aspartate receptor autoantibodies coexisted in 2 of 15 tested (13%). CCE pathology (n = 2) showed extensive subpial cortical demyelination (n = 2), microglial reactivity (n = 2), and inflammatory infiltrates (perivascular, n = 1; meningeal, n = 1). Most received high-dose steroids (n = 17, 89%), and all improved, but 3 had CCE relapses. This study highlights the CCE spectrum and provides insight into its pathogenesis. ANN NEUROL 2022.
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U2 - 10.1002/ana.26549
DO - 10.1002/ana.26549
M3 - Article
C2 - 36372941
AN - SCOPUS:85143398564
JO - Annals of Neurology
JF - Annals of Neurology
SN - 0364-5134
ER -