Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.
Eoin Patrick FlanaganYong GuoKarl N KreckeJohn J ChenVyanka RedenbaughMayra MontalvoPaul M ElsberndJan-Mendelt TillemaAlfonso Sebastian Lopez-ChiribogaAdrian BudhramElia SechiAmy KunchokDivyanshu DubeySean J PittockClaudia F LucchinettiEoin P FlanaganPublished in: Annals of neurology (2022)
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.
Keyphrases
- magnetic resonance imaging
- high dose
- end stage renal disease
- subarachnoid hemorrhage
- newly diagnosed
- ejection fraction
- chronic kidney disease
- low dose
- white matter
- computed tomography
- early onset
- peritoneal dialysis
- lipopolysaccharide induced
- magnetic resonance
- multiple sclerosis
- spinal cord
- cross sectional
- binding protein
- small cell lung cancer
- patient reported