Reyes-Botero Germán, Uribe Carlos S, Hernandez-Ortiz Olga E, Ciro Juan, Guerra Alejandro, Dalmau-Obrador Josep
Facultad de Medicina, Universidad de Antioquia, Departamento de Neurología Clínica, Medellín, Colombia.
Rev Neurol. 2011 May 1;52(9):536-40.
A paraneoplastic syndrome characterized by neuropsychiatric symptoms, involuntary movements and seizures has been recently associated with antibodies targeting NMDA (N-methyl-D-aspartate) receptor in patients with an ovarian teratoma. Severe neurological impairment is frequent and treatment in the intensive care unit is often required because of ventilatory failure and life-threatening autonomic instability. Tumor removal is curative in many cases and neurological improvement is demonstrated shortly after surgery.
Here we report on a patient with paraneoplastic encephalitis manifested by unconsciousness and coreo-athetosic movements related to NMDA receptor antibodies associated with an immature ovarian teratoma grade III. She made a complete recovery after oophorectomy, intravenous immunoglobulin and corticosteroids.
Treatment of paraneoplastic syndromes is based on specific therapy for underlying tumor associated to immunomodulators. As in this case, anti-NMDA encephalitis may significantly improve after tumor removal and intra-venous immunoglobuline.
一种以神经精神症状、不自主运动和癫痫发作为特征的副肿瘤综合征,最近被发现与卵巢畸胎瘤患者体内靶向N-甲基-D-天冬氨酸(NMDA)受体的抗体有关。严重的神经功能障碍很常见,由于呼吸衰竭和危及生命的自主神经不稳定,常常需要在重症监护病房进行治疗。在许多病例中,切除肿瘤可治愈疾病,术后不久神经功能即可改善。
在此,我们报告一名患有副肿瘤性脑炎的患者,表现为昏迷和与NMDA受体抗体相关的舞蹈徐动症,该抗体与III级未成熟卵巢畸胎瘤有关。她在接受卵巢切除、静脉注射免疫球蛋白和皮质类固醇治疗后完全康复。
副肿瘤综合征的治疗基于针对相关肿瘤的特异性治疗以及免疫调节剂。如本病例所示,切除肿瘤和静脉注射免疫球蛋白后,抗NMDA脑炎可能会显著改善。