An abdominal CT scan shows a tumor in the right ovary, in which calcification and fat were observed, and suggests an ovarian teratoma (arrowhead) (A). induced by additional underlying pathogenic antibodyCantigen reactions. Keywords: neuromyelitis optic, autoimmune disease, neuro-oncology, aquaporin-4, malignancy Intro Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune, astrocytopathic diseases influencing the central nervous system (CNS). Aquaporin 4 (AQP4) was identified as the main target protein of NMOSD in 2005 (1), which enabled NMOSD to be an independent entity, apart from multiple sclerosis. Aquaporin 4-immunoglobulin G (AQP4-IgG) can be recognized in about Rabbit polyclonal to HSD3B7 80% of individuals with NMOSD (2). Among individuals with AQP4-IgG-seronegative, antibodies to myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) account for 42% of all instances (3). Compared to AQP4-IgG-seropositive NMOSD, diagnostic criteria for AQP4-IgG-seronegative NMOSD are more stringent and require critical clinical criteria and additional neuroimaging findings (4). Even Scutellarein though incidence is extremely low, NMOSD were reported to be associated with different types of cancer, of which genitourinary, breast, and lung cancers are most frequently involved (5). NMOSD are considered paraneoplastic neurologic syndrome (PNS) as NMOSD matches the diagnostic criteria (6). We reported three NMOSD instances associated with malignancy, which are teratoma and lung adenocarcinoma, teratoma, and transverse colon adenocarcinoma, respectively. Immunohistochemistry staining of the tumor sections all exposed an AQP4 high manifestation. Methods This study reports three instances and was authorized by the Ethics Committee of Soochow University or college, China. Written educated consent was from all instances. Case 1 A 30-year-old female presented with transient loss of consciousness, blurred vision, binaural hearing loss, tinnitus, and slurring conversation. Before presenting in our division, she kept visiting the gastroenterology division and was treated there for more than 3 years because of recurrent epigastric pain, nausea, and vomiting. She underwent peroral enteroscopy and transanal enteroscopy, and no obvious abnormalities were found. The patient underwent remaining ovarian teratoma ablation at the age of 23 years, and she was confirmed to have teratoma in the right ovary when she was 26 years old but did not receive any treatment (Number 1A). Her cerebrospinal fluid (CSF) shown 1 leukocyte/L, moderately Scutellarein elevated protein (72 mg/dL), and negativity for oligoclonal immunoglobulin Scutellarein G (IgG) bands (OCBs), and no neoplastic cells were found. She tested for CSF and serum AQP4-IgG, MOG-IgG, glial fibrillary acidic protein antibody (GFAP-IgG), and the autoimmune encephalitis antibody panel (N-methyl-D-aspartate receptor (NMDAR)-IgG, leucine-rich, glioma-inactivated 1 protein (LGI1)-IgG, anti-contactin-associated protein-like 2 (CASPR2)-IgG, -aminobutyric acid receptor (GABABR)-IgG, alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionate (AMPA) receptor 1 (AMPAR1)-IgG, Alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionate (AMPA) receptor 2 (AMPAR2)-IgG, IgLON Family Member 5 (IgLON5-IgG), dipeptidyl aminopeptidase-like protein 6 (DPPX)-IgG, 65-kDa glutamic acid decarboxylase (GAD65)-IgG, metabotropic glutamate receptor 5 [mGluR5)-IgG, glycine receptor (GlyR)-IgG, and anti-dopamine-2 receptor (D2R)-IgG)], Scutellarein which were all bad (analysis having a cell-based assay). Mind magnetic resonance imaging (MRI) showed fluid-attenuated inversion recovery (FLAIR) hyperintense and contrast-enhancing lesions in the thalamus, hypothalamus, and area postrema (Numbers 1E,?,F).F). MRI was also carried out within the spinal wire, but no lesions were remarkable. She presented with the negativity of sero-AQP4-IgG and two core clinical characteristics (optic neuritis and area postrema syndrome); consequently, she was diagnosed with AQP4-IgG-seronegative NMOSD. She was treated with intravenous immunoglobulins (IVIG) (0.4 g/kg/d*5 d) and subsequent methylprednisolone (400 mg*3 d, 200 mg*3 d, 80 mg*3 d, 40 mg*3 d) and managed with oral steroids. Six months later, her visual and hearing symptoms gradually improved, and.