From intractable hiccups to optic neuritis: paraneoplastic neuromyelitis optica with leptomeningeal carcinomatosis in the setting of immature ovarian teratoma.

BACKGROUND: Paraneoplastic syndromes are associated with 10% of ovarian cancers. Neuromyelitis optica spectrum disorders (NMOSD), which can present with vision changes, paraplegia, vomiting, and/or intractable hiccups, are rarely associated with ovarian cancers. We present the first recorded case of NMOSD in the setting of malignant immature ovarian teratoma, with concurrent leptomeningeal carcinomatosis contributing to clinical presentation.

CASE: We describe a 27-year-old patient with Stage IVB Immature Ovarian Teratoma complicated by leptomeningeal carcinomatosis, who initially presented with intractable hiccups, nausea, and vomiting, and eventually developed attenuated vision. Neurological workup revealed AQP4-IgG antibodies, suggesting neuromyelitis optica (NMO) as a paraneoplastic syndrome (PNS). The patient's cancer was treated with tumor resection, BEP (bleomycin, etoposide, cisplatin) therapy, maintenance bevacizumab, and brain radiation. While symptoms suspicious for NMO presented early on, it was not until after tumor resection and the development of double vision that NMO was diagnosed. NMO was promptly treated with plasma exchange and high dose steroids. While therapies did lead to disease stabilization, she was left with residual neurologic deficits requiring gait assistance.

CONCLUSION: Early symptoms such as intractable hiccups, nausea, or vomiting may precede optic neuritis or myelitis and serve as initial warning signs of NMOSD. Clinicians should maintain a high index of suspicion and low threshold for neurology consultation and AQP4-IgG antibody titers, especially when neurologic symptoms are not fully explained by metastatic disease.