Mesh : Child Female Humans Anti-N-Methyl-D-Aspartate Receptor Encephalitis / complications Encephalitis Hashimoto Disease Ovarian Neoplasms / diagnosis pathology therapy Receptors, Amino Acid Receptors, N-Methyl-D-Aspartate Retrospective Studies Teratoma / complications diagnosis pathology

来  源:   DOI:10.1097/WNF.0000000000000581

Abstract:
OBJECTIVE: Teratoma is a type of germ cell tumor that derived from early embryonic stem cells and germ cell lines, which can lead to a rare complication known as paraneoplastic encephalitis syndrome. Delayed removal of teratoma allows for continuing antigen presentation, inducing affinity maturation of the antibody and the generation of long-lived plasma cells that infiltrate both bone marrow and brain, which makes the patient nonresponsive to later removal of teratoma and refractory to immunotherapy. We present this rare case to remind clinicians to be vigilant for the recognition and removal of teratoma during the treatment of autoimmune encephalitis.
METHODS: We retrospectively reviewed the clinical record of this 12-year 5-month-old female patient diagnosed with anti- N -methyl- d -aspartate receptor (anti-NMDAR) autoimmune encephalitis; her ovarian teratoma was unidentified on admission. She did not respond to immunosuppressive therapy until the mature ovarian teratoma identified 45 days after admission and removed the following day, nearly 2 months after symptom onset. This patient experienced nearly complete resolution of symptoms within the subsequent 2 weeks. In addition, we conducted a literature review of the clinical presentations and treatment of anti-NMDAR autoimmune encephalitis associated with ovarian teratoma in the pediatric population.
RESULTS: Our findings suggest that clinicians should be vigilant for the recognition and removal of teratoma during the treatment of autoimmune encephalitis.
CONCLUSIONS: Female pediatric patients with suspected anti-NMDAR encephalitis should be screened for ovarian tumors immediately and treated in a multidisciplinary setting including neurology and obstetrics and gynecology.
摘要:
目的:畸胎瘤是一种来源于早期胚胎干细胞和生殖细胞系的生殖细胞肿瘤,这可能导致一种罕见的并发症,称为副肿瘤性脑炎综合征。畸胎瘤的延迟清除允许持续的抗原呈递,诱导抗体的亲和力成熟和生成渗入骨髓和大脑的长寿命浆细胞,这使得患者对后来的畸胎瘤切除无反应,对免疫疗法难以治疗。我们介绍这种罕见的情况,以提醒临床医生在自身免疫性脑炎的治疗过程中警惕畸胎瘤的识别和清除。
方法:我们回顾性回顾了这名12岁5个月大的女性患者的临床记录,该患者被诊断患有抗N-甲基-d-天冬氨酸受体(抗NMDAR)自身免疫性脑炎;她的卵巢畸胎瘤入院时身份不明。直到入院45天后发现成熟的卵巢畸胎瘤并在第二天切除,她才对免疫抑制治疗产生反应。症状发作后近2个月。该患者在随后的2周内症状几乎完全缓解。此外,我们对儿科人群中与卵巢畸胎瘤相关的抗NMDAR自身免疫性脑炎的临床表现和治疗进行了文献综述.
结果:我们的研究结果表明,在自身免疫性脑炎的治疗过程中,临床医生应警惕畸胎瘤的识别和清除。
结论:疑似抗NMDAR脑炎的女性儿科患者应立即筛查卵巢肿瘤,并在包括神经科和妇产科在内的多学科环境中治疗。
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