Teaching NeuroImages: Cortical damage with leptomeningeal enhancement in neuromyelitis optica spectrum disorder

Abstract

A 27-year-old woman presented with headache, weakness, and numbness in both lower extremities for 2 weeks. Two years ago, she had refractory hiccups and vomiting with associated area postrema lesions on MRI (figure, A–C). Workup for infectious disease was negative. Serum aquaporin-4–immunoglobulin G was positive. Recent MRI showed cortical damage adjacent to the cerebral falx with leptomeningeal enhancement (figure, D–F). Treatment with immunoglobulin and high-dose methylprednisolone produced a significant improvement of the symptoms and the follow-up MRI (figure, G–I). This patient's case is unusual, as neuromyelitis optica spectrum disorders rarely involve the cortex and pia mater.1,2