Case Report
 
Extensive reversible periependymal ventricular enhancement on magnetic resonance imaging scan in a patient with neuromyelitis optica
Anza B. Memon1, Kumar Rajamani2, Robert P. Lisak3
1MD, Clinical Neurophysiology Fellow, Department of Neurology, Henry Ford Hospital, Detroit, Michigan, USA.
2MD, Associate professor, Department of Neurology, Wayne State University, Detroit Medical Center, Detroit, Michigan, USA.
3MD, Professor, Department of Neurology, Wayne State University, Detroit Medical Center, Detroit, Michigan, USA.

Article ID: Z01201612CR10729AM
doi:10.5348/ijcri-2016141-CR-10729

Address correspondence to:
Anza B. Memon
2799 W Grand Blvd, Detroit
Michigan
USA, 48202

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How to cite this article
Memon AB, Rajamani K, Lisak RP. Extensive reversible periependymal ventricular enhancement on magnetic resonance imaging scan in a patient with neuromyelitis optica. Int J Case Rep Images 2016;7(12):810–814.


Abstract
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.

Keywords: Devic's disease, Neuromyelitis optica, Neuromyelitis optica spectrum disorder (NMOSD)


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Author Contributions
Anza B. Memon – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Kumar Rajamani – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Robert P. Lisak – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Anza B. Memon et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.