THE CURIOUS CASE OF NEUROMYELITIS OPTICA L Gawey BS, AQ Nguyen DO, PhD Department of Internal Medicine, Louisiana State University Health New Orleans, LA
INTRODUCTION: Neuromyelitis Optica (NMO) is an autoimmune inflammatory demyelinating disease that tends to affect the central nervous system. NMOSDs are very rare with prevalence ranging from 0.37 to 10 per 100,000 with median age of onset 32-41 years.
CASE: A 24-year-old female with migraine headaches presented with a one-month history of rapidly progressive right-sided vision loss and right-sided headache associated with photophobia, pain, and flashes with eye closing. Vitals were unremarkable. Physical exam was significant for complete right eye blindness without visual field defect. Dilated ophthalmology exam showed Grade III optic disc edema with surrounding disc flame hemorrhages in the right eye. Laboratory studies were all within normal limits. Magnetic Resonance Imaging and Magnetic Resonance Angiography of the brain showed abnormal contrast enhancement and T2 hyperintensity involving all segments of the right optic nerve and proximal right optic tract, suggesting severe optic neuritis. Further serologic testing revealed a positive AQP-4 receptor antibody titer of 15.3 U/ mL (negative < 2.9 U/mL), and final diagnosis of Neuromyelitis Optica. Computed Topography of the chest, to determine the primary etiology of NMO, revealed a pulmonary lesion. Pathology confirmed a mucinous lung adenocarcinoma. She subsequently underwent right lower lobe lobectomy with mediastinal lymph node dissection that was negative for metastatic disease. She initially received four rounds of plasmapheresis and rituximab with stabilization of symptoms for NMO. Her symptoms improved after treatment for NMO and lobectomy. DISCUSSION: NMO is often associated with antibodies to aquaporin-4 (AQP-4) channels, which are the most abundant water channels in the central nervous system. Hallmark features of NMO spectrum disorder (NMOSDs) include acute attacks of bilateral or rapidly sequential optic neuritis or transverse myelitis in conjunction with seropositive for AQP-4 antibodies. Areas of CNS inflammation in NMO tend to correlate with the expression pattern of AQP-4 and corelate in frequency with relapsing disease, optic neuritis, or longitudinally extensive transverse myelitis. NMO spectrum disorders (NMOSDs) can often develop as paraneoplastic disease secondary to cancer particularly of breast and lung. AQP-4 positivity in NMOSD can serve should raise concern for potential malignancy screening in individuals positive for AQP-4.
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