Uchida Yuto, Kato Daisuke, Yamashita Yoriko, Ozaki Yasuhiko, Matsukawa Noriyuki
Department of Neurology.
Department of Experimental Pathology.
Neuropsychiatr Dis Treat. 2018 Jan 22;14:339-342. doi: 10.2147/NDT.S156603. eCollection 2018.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a type of autoimmune encephalitis that can be paraneoplastic and usually responds to tumor resection and immunotherapy. More than 75% of patients with anti-NMDAR encephalitis fully recover or have only mild sequelae, whereas the remainder experience severe disability. It remains unknown why certain cases have refractory clinical disease courses. We report a case of anti-NMDAR encephalitis with bilateral ovarian teratomas who was refractory to tumor resection and early initiation of immunotherapy. During intensive care, immunohistochemical analyses of her cerebrospinal fluid showed persistently high reactivity of NMDAR antibody over time. Six months after the operation, pelvic computed tomography detected a recurrent ovarian teratoma. After total enucleation of the bilateral ovaries, with significant pathological findings of bilateral mature cystic teratomas, her clinical condition improved rapidly, paralleled by a decrease in anti-NMDAR reactivity. This case illustrates the need to keep considering why extensive treatment fails to influence the disease when we encounter patients with refractory anti-NMDAR encephalitis. Failure to improve after ovarian resection could be a marker of recurrent ovarian teratoma in anti-NMDAR encephalitis.
抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是一种自身免疫性脑炎,可呈副肿瘤性,通常对肿瘤切除和免疫治疗有反应。超过75%的抗NMDAR脑炎患者可完全康复或仅遗留轻度后遗症,而其余患者则会出现严重残疾。某些病例为何会有难治的临床病程仍不清楚。我们报告一例患有双侧卵巢畸胎瘤的抗NMDAR脑炎患者,该患者对肿瘤切除和早期开始的免疫治疗均无反应。在重症监护期间,对其脑脊液进行的免疫组化分析显示,随着时间推移,NMDAR抗体反应性持续居高不下。术后6个月,盆腔计算机断层扫描检测到复发性卵巢畸胎瘤。在双侧卵巢完全摘除后,病理检查发现双侧为成熟囊性畸胎瘤,其临床状况迅速改善,同时抗NMDAR反应性降低。该病例表明,当我们遇到难治性抗NMDAR脑炎患者时,需要不断思考为何广泛治疗未能影响病情。在抗NMDAR脑炎中,卵巢切除后病情未改善可能是复发性卵巢畸胎瘤的一个标志。