Paraneoplastic neuromyelitis optica spectrum disorder associated with metastatic carcinoid expressing aquaporin-4.

Figueroa, Michelle; Guo, Yong; Tselis, Alexandros; Pittock, Sean J; Lennon, Vanda A; Lucchinetti, Claudia F; Lisak, Robert P · JAMA Neurol · 2014

case_report · Level V

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Abstract

Reports of neuromyelitis optica spectrum disorder (NMOSD) occurring in the setting of neoplasia suggest that aquaporin-4 autoimmunity may in some cases have a paraneoplastic basis. In this case report, we describe a patient with NMOSD whose test results were seropositive for aquaporin-4 IgG and who had a hepatic metastasis from a small-bowel neuroendocrine tumor. The tumor cells expressed aquaporin-4 immunoreactivity. She presented to the Neurology Department at Wayne State University with bilateral leg weakness, ascending paresthesias, and decreased sensation. This case extends the context of NMOSD as a paraneoplastic disorder.

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