Case of neuromyelitis optica with recurrent stomach carcinoma

Abstract

Background Neuromyelitis optica (NMO) is an autoimmune astrocytopathy associated with anti-aquaporin-4 (AQP4). The development of NMO in the setting of cancer suggests that AQP4 autoimmunity might in some cases be paraneoplastic. Case presentation We report an 85-year-old woman who developed a right optic neuritis and a longitudinally extensive transverse myelitis associated with AQP4 autoantibodies after the relapse of stomach cancer. Histopathological observations in several case reports have led to the hypothesis that the NMO associated with the malignancy is caused by an autoimmunity against abnormally expressed AQP4 on tumor cells. However, we found no AQP4 antigen on the cancer cells in our resected tissues. Our findings did not support the concept of a paraneoplastic process in the narrow sense. Conclusions The exact mechanisms of cancer-related NMO, which might be multifactorial, remain controversial.

Paraneoplastic neuromyelitis optica spectrum disorder associated with malignant melanoma: A case report

“…10,11 Some cases were also identified as paraneoplastic NMOSD, including various cancers. 10,[12][13][14][15][16][17][18] Only one case of malignant melanoma complicated with anti-AQP4 antibody-positive NMOSD has been reported. 19 However, that patient developed NMOSD while under interferon treatment, and the authors ruled out paraneoplastic NMOSD due to no recurrence of melanoma and a 48-month interval between melanoma excision and the development of NMOSD.…”

“…10 A case report detailing the development of anti-AQP4 antibody-positive NMOSD immediately after the relapse of stomach cancer might support this concept, although there was no immunohistochemical confirmation of AQP4 in cancer cells. 18 Furthermore, paraneoplastic NMOSD has previously been reported in a patient with adenocarcinoma of the esophagogastric (EG) junction that exhibited immunoreactivity to AQP4 and an elevated serum AQP4-IgG level that turned to be undetectable after surgical resection of the tumor, 19 suggestive of an induction of anti-AQP4 antibodies by the tumor cells that lead to NNMOSD. In our patient, although the onset of NMOSD could be coincidental, it was also speculated that occult malignant melanoma for four years led to the development of NMOSD.…”