Westchester Medical Center, Valhalla, New York.
Montefiore Medical Center, Bronx, New York.
Reumatismo. 2023 Dec 19;75(4). doi: 10.4081/reumatismo.2023.1598.
Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is a B-cell-mediated autoimmune encephalitis with wide non-specific symptoms like acute-onset psychiatric or neurological ones mimicking various other conditions. A careful history and appropriate workup, including cerebrospinal fluid analysis for anti-NMDAR antibodies, imaging, and electroencephalogram, should be conducted, considering all differential diagnoses that can mimic its presentation. Combination therapy with high-dose steroids, plasma exchange, or immunoglobulin therapy has been shown to be more efficacious. In patients who fail first-line therapy, rituximab or cyclophosphamide should be considered. It is essential to rule out ovarian teratoma or other occult malignancies that can cause NMDARE, as removal of the tumor itself resolves this condition. Timely diagnosis and early intervention are necessary to avoid an untoward outcome.
抗 N-甲基-D-天冬氨酸受体脑炎(NMDARE)是一种 B 细胞介导的自身免疫性脑炎,具有广泛的非特异性症状,如急性发作的精神或神经症状,类似于各种其他疾病。应进行详细的病史采集和适当的检查,包括针对抗 NMDAR 抗体的脑脊液分析、影像学和脑电图,以考虑所有可能模仿其表现的鉴别诊断。大剂量类固醇、血浆置换或免疫球蛋白联合治疗已被证明更有效。对于一线治疗失败的患者,应考虑使用利妥昔单抗或环磷酰胺。排除可能导致 NMDARE 的卵巢畸胎瘤或其他隐匿性恶性肿瘤至关重要,因为肿瘤本身的切除可以解决这种情况。及时诊断和早期干预是必要的,以避免不良后果。