Characteristics of myelin oligodendrocyte glycoprotein antibody-associated meningoencephalitis

Background Meningoencephalitis can occur in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). Objective To assess the clinical and radiologic features of MOG-IgG meningoencephalitis. Methods Multicenter retrospective cases series of MOG-IgG meningoencephalitis and literature review of MOG-IgG-positive patients with the clinical syndrome of meningoencephalitis. Results Ninety MOG-IgG-positive patients were identified from three academic medicine centers. 8/90 (8.9%) patients presented with a clinical syndrome of meningoencephalitis (age: 4–57 years; 5/8 male; 4/8 Caucasian; MOG-IgG titers 1:20–1:1000), which was the initial presentation in 7/8 patients. Symptoms included headaches (n = 8/8), encephalopathy (n = 7/8), seizures (n = 4/8), meningismus (n = 3/8), and aphasia (n = 3/8). Cerebrospinal fluid (CSF) pleocytosis was present in 7/8 patients (12–1745 cells/mm 3 ) and frequently neutrophilic (>25%; n = 4/6). Magnetic resonance imaging (MRI) was notable for leptomeningeal enhancement with (n = 5/8) or without cortical edema (n = 1/8), focal dural enhancement (n = 1/8), and leptomeningeal loss of FLAIR suppression (n = 1/8). 7/8 patients sustained a relapsing disease course. Literature review identified 150 additional cases with MOG-IgG meningoencephalitis (initial attack in 86.7%) with median age of 20 (1–67) years, Asian (90.4%) and male (54.1%) predilection, and CSF pleocytosis in 87.9% of patients (82 [0–887] cells/cm 3 ; ≥100 cells in 44.3%), which was frequently (51.7%) neutrophilic. Conclusions MOG-IgG meningoencephalitis may represent the initial presentation of MOGAD with neutrophilic pleocytosis in CSF and meningo-cortical involvement on MRI.