Center for pathophysiology Toulouse Purpan, INSERM U1043, CNRS 5282, Université Toulouse III, 31024 Toulouse, France; CRC-SEP, Pôle Neurosciences, CHU de Toulouse, 31059 Toulouse, France; Neurosciences and Medicine Departments, Centre hospitalier universitaire vaudois (CHUV), 1011 Lausanne, Switzerland.
Service de neurologie, sclérose en plaques, pathologies de la myéline et neuro-inflammation and Centre de référence pour les maladies inflammatoires rares du cerveau et de la moelle (MIRCEM)- Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon, Lyon, F-6977, France; Lyon's Neuroscience Research Center, U1028 INSERM, UMR5292 CNRS, FLUID Team F-69008 Lyon, France.
Mult Scler Relat Disord. 2019 Jun;31:97-100. doi: 10.1016/j.msard.2019.03.031. Epub 2019 Apr 1.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease of the central nervous system, characterized by the presence of auto-antibodies directed against aquaporin-4 (AQP4) expressed on astrocyte end-feet. Despite NMOSD does not primarily belong to the spectrum of paraneoplastic neurological syndromes, rare cases of association with neoplasia have been outlined. Here, we report the association of NMOSD with ovarian teratoma in 3 cases. Pathological analysis of teratomas revealed glial component strongly expressing AQP4 and closely localized to immune infiltrates. Our series highlight the rare association of teratoma with NMOSD and the possible paraneoplastic mechanism.
视神经脊髓炎谱系疾病(NMOSD)是一种罕见的中枢神经系统炎症性疾病,其特征是存在针对星形胶质细胞终足表达的水通道蛋白-4(AQP4)的自身抗体。尽管 NMOSD 主要不属于副肿瘤性神经综合征的范畴,但已有少数与肿瘤相关的病例被描述。在这里,我们报告了 3 例 NMOSD 与卵巢畸胎瘤相关的病例。畸胎瘤的病理分析显示,胶质成分强烈表达 AQP4,且与免疫浸润密切相关。我们的系列研究强调了畸胎瘤与 NMOSD 的罕见关联及其可能的副肿瘤机制。