Abstract
Objective: We present a case of a young man who presented to the hospital with aseptic meningitis. He progressed and developed bilateral optic-neuritis and myelopathy consistent with Neuromyelitis Optica Spectrum Disorder (NMOSD). He was later found to have myelin-oligodendrocyte (MOG) antibody in the CSF.
Background: Diagnostic criteria for Aquaporin-4 (AQP-4) IgG negative NMOSD includes the presence of two core clinical-characteristics [optic-neuritis, acute longitudinally-extensive transverse myelitis (LETM), or area-postrema syndrome] and exclusion of alternative etiology. Anti-MOG antibodies have been detected in a subset of these patients, but due to absence of commercial testing in the United States these cases remain undiagnosed. This may have implications on immuno-therapeutic decision making.
Design/Methods: A 29 year-old Caucasian man presented to outside-hospital with headache, fever and neck-stiffness. MRI brain showed leptomeningeal enhancement. CSF was remarkable for elevated protein and pleocytosis. Analysis for infectious etiologies was negative. He was transferred to our hospital for potential meningeal biopsy. Over the next few days, he developed blurred vision, paraplegia, and urinary retention. Repeat MRI brain showed new-enhancing lesions in the cortex and optic-chiasm. MRI spinal-cord was consistent with LETM. Serum AQP-4 cell-based assay was negative. CT chest/abdomen/pelvis was unremarkable for malignancy/lymphadenopathy. Due to the presence of bilateral optic-neuritis and LETM, diagnosis of AQP-4-IgG negative NMOSD was considered. CSF was sent to the oxford-laboratory for further analysis on a research basis. He was treated with high-dose intravenous methyl-prednisolone for 5 days with significant improvement in his symptoms. He was discharged on oral prednisone.
Results: The anti-MOG antibody resulted positive after discharge. He was tapered off corticosteroids over the next 2 months and remained relapse free for more than 18-month
Conclusions: Anti-MOG antibody is a biomarker that has been associated with NMOSD. This test should be considered in all sero-negative NMOSD patients, as it may impact duration of immunomodulation.
Disclosure: Dr. Truong has nothing to disclose. Dr. Hitt has nothing to disclose. Dr. Dubey has nothing to disclose. Dr. Ortstadt has nothing to disclose.
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