Department of Neurology, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
J Neuroimmunol. 2013 Oct 15;263(1-2):145-7. doi: 10.1016/j.jneuroim.2013.07.003. Epub 2013 Jul 27.
Paraneoplastic myelitis is a rare inflammatory disorder most frequently associated with solid tumors or lymphoproliferative disorders. Patients often harbor onconeuronal antibodies and their prognosis is usually poor. Here we report a 42-year old woman with longitudinally extensive transverse myelitis and aquaporin-4 (AQP4) antibodies that led to the diagnosis of ovarian teratoma. After tumor removal and immune therapy (including corticosteroids, plasma exchange, intravenous immunoglobulins and rituximab) the patient progressively improved achieving complete recovery. Histological study of the teratoma demonstrated neural tissue containing AQP4 expressing cells and intense inflammatory infiltrates, providing evidence for a possible paraneoplastic link between both disorders.
副肿瘤性脊髓炎是一种罕见的炎症性疾病,最常与实体瘤或淋巴增生性疾病相关。患者常携带神经元抗体,预后通常较差。本文报告了一例 42 岁女性,患有广泛的横贯性脊髓炎和水通道蛋白-4(AQP4)抗体,导致卵巢畸胎瘤的诊断。在肿瘤切除和免疫治疗(包括皮质类固醇、血浆置换、静脉注射免疫球蛋白和利妥昔单抗)后,患者逐渐改善,达到完全恢复。畸胎瘤的组织学研究显示含有表达 AQP4 的神经组织和强烈的炎症浸润,为这两种疾病之间可能存在的副肿瘤联系提供了证据。