Extensive Cerebral Cortical Involvement in Aquaporin 4 Antibody Positive Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorders (NMOSDs) usually manifest with features of long-segment myelitis and/or optic neuritis. Area postrema involvement presents with nausea, vomiting, and intractable hiccups. Cerebral cortical involvement is a relatively uncommon phenomenon. This report describes an interesting case of aquaporin 4 antibody positive NMOSD with extensive cerebral cortical involvement in addition to area postrema and cervicomedullary lesions observed on magnetic resonance imaging. Following immunosuppressive therapy, good clinical response and near-complete resolution of brain imaging abnormalities were observed.