Extensive reversible periependymal ventricular enhancement on magnetic resonance imaging scan in a patient with neuromyelitis optica

Introduction: Neuromyelitis optica (NMO) is an inflammatory central nervous system (CNS) disorder which predominately affects the optic nerves, chiasm, tract and spinal cord. Both symptomatic and asymptomatic brain lesions have been reported. We describe a patient with neuromyelitis optica spectrum disorder (NMOSD) with suspected prior thoracic cord ependymoma and unusual radiological picture. Case Report: A 69-year-old woman with history of thoracic cord ependymoma ten years earlier was diagnosed with NMOSD with elevated aquaporin (AQP4) antibodies. In addition to contrast enhancement along optic chiasm and optic tract, brain MRI scan showed increased signal on FLAIR and post-contrast enhancement in the subependymal region around the aqueduct and lateral ventricles. Spinal MRI showed thoracic cord atrophy. After corticosteroids and plasma exchange (PLEX), vision improved and imaging 2 weeks later showed resolution of lesions except periaqueductal enhancement, which resolved completely after three months. Conclusion: This case report is striking for two reasons. The patient had virtually all of the previously described MRI lesions described in literature at different stages of the NMOSD. Enhancing lesions outside the brain and optic pathways, seen in this patient are rare in NMOSD. Complete resolution of enhancing lesions after IVMP and PLEX on several occasions was striking. Occurrence of NMOSD in patients with ependymoma has not been reported but can be reasonably speculated in this patient as some cases of NMOSD represent a paraneoplastic disorder. Moreover, ependymal cells are having abundant AQP4 channels raising a possibility of molecular mimicry. (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 7 No. 12, December 2016. ISSN – [0976-3198] Int J Case Rep Images 2016;7(12):810–814. www.ijcasereportsandimages.com Memon et al. 810 CASE REPORT OPEN ACCESS Extensive reversible periependymal ventricular enhancement on magnetic resonance imaging scan in a patient with neuromyelitis optica Anza B. Memon, Kumar Rajamani, Robert P. Lisak