Anderson Dustin, Nathoo Nabeela, Henry Monica, Wood George, Smyth Penelope, McCombe Jennifer
Critical Care, University of Alberta, Edmonton, Canada.
Medicine (Neurology), University of Alberta, Edmonton, Canada.
Pract Neurol. 2020 Oct 8. doi: 10.1136/practneurol-2020-002676.
Ovarian teratomas are found in one-third of females presenting with anti--methyl-D-aspartate receptor (NMDAR) encephalitis. When a teratoma is detected on imaging, its removal is first-line therapy. Even with multiple imaging modalities, occasionally, the teratoma is found only on subsequent imaging, long after initial presentation. Very rarely, patients have undergone oophorectomy despite negative imaging, with pathology demonstrating teratoma, and resulting clinical improvement. We present a patient in whom removal of a teratoma, not visible on conventional imaging, resulted in marked clinical improvement. Such cases present a major clinical challenge, needing to consider the risks of oophorectomy, including sterilisation and early menopause, versus the possibility of death in the absence of response to first-line (eg, corticosteroids, plasma exchange, intravenous immunoglobulin), second-line (eg, rituximab) and third-line (eg, bortezomib) immunosuppression. This decision is made more difficult as patients are usually females of childbearing age who at the time lack capacity to make medical decisions. This case also highlights the lack of consensus and guidelines for imaging modalities used to detect teratoma and when to pursue oophorectomy.
在患有抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的女性患者中,三分之一可发现卵巢畸胎瘤。当影像学检查发现畸胎瘤时,切除畸胎瘤是一线治疗方法。即便使用了多种影像学检查手段,有时畸胎瘤在初次就诊很久之后的后续影像学检查中才被发现。极少情况下,尽管影像学检查结果为阴性,但患者接受了卵巢切除术,术后病理显示为畸胎瘤,且临床症状得到改善。我们报告了1例患者,其切除了常规影像学检查未发现的畸胎瘤后,临床症状显著改善。此类病例带来了重大临床挑战,需要权衡卵巢切除术的风险,包括绝育和过早绝经,以及在一线治疗(如皮质类固醇、血浆置换、静脉注射免疫球蛋白)、二线治疗(如利妥昔单抗)和三线治疗(如硼替佐米)免疫抑制无效时死亡的可能性。由于患者通常为育龄期女性,当时缺乏做出医疗决策的能力,这一决策变得更加困难。该病例还凸显了在用于检测畸胎瘤的影像学检查方法以及何时进行卵巢切除术方面缺乏共识和指南。