Cerebral Attack With Cortical-Leptomeningeal Involvement and Seizures in AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder.

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) rarely presents with corticalleptomeningeal involvement. A 47-year-old woman with aquaporin-4 immunoglobulin G (AQP4-IgG)-positive NMOSD developed acute headache, seizures, motor aphasia, and worsening right arm weakness. Brain magnetic resonance imaging revealed a tumefactive-appearing left frontal cortical-subcortical lesion with leptomeningeal enhancement. The clinical and radiological abnormalities improved after immunotherapy. This case illustrates a rare cerebral manifestation of AQP4-IgG-positive NMOSD with cortical-leptomeningeal involvement, seizures, and a tumefactive lesion.