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- W4283586609 abstract "Neuromyelitis optica spectrum disorder (NMOSD) is a rare, autoimmune disease that targets astrocytes in the central nervous system. Rarely, it can occur as a paraneoplastic phenomenon and has been associated with a myriad of different cancers. We present a unique association of anti-AQP4 seronegative NMOSD and undifferentiated carcinoma to provide a didactic case-oriented review of paraneoplastic NMOSD. A 54-year-old female undergoing systemic treatment for a high-grade undifferentiated carcinoma developed recurrent episodes of visual loss and limb weakness. Neurological exam and neuroimaging revealed the diagnosis of optic neuritis and a longitudinally extensive transverse myelitis. Anti-AQP4 CBA-based assays were negative. We made the diagnosis of seronegative NMOSD. She was treated with immunotherapy and underwent resection of the tumoral mass. Despite immunosuppression with methylprednisolone and plasma exchange, associated with the treatment of the tumor, our patient did not present significant improvement of her neurological deficits and she died of sepsis related to catheter associated infection. The “take-away” lesson of this case report is that when patients with undifferentiated carcinoma develop visual loss or weakness, we should consider the differential diagnosis of NMOSD, even in anti-AQP4 negative patients." @default.
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- W4283586609 date "2022-01-01" @default.
- W4283586609 modified "2023-09-30" @default.
- W4283586609 title "Paraneoplastic AQP-4 IgG negative neuromyelitis optica spectrum disorder (NMOSD) associated with large-cell high-grade undifferentiated carcinoma of unknown primary site: A case report and review of paraneoplastic NMOSD" @default.
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- W4283586609 doi "https://doi.org/10.1016/j.nerep.2022.100110" @default.
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