Mirza M Kamran, Pogoriler Jennifer, Paral Kristen, Ananthanarayanan Vijayalakshmi, Mandal Saptarshi, Mazin Abdul, Baron Beverly, Richa Elie
Department of Pathology, Biological Sciences Division, Section of Blood Bank and Transfusion Medicine, The University of Chicago, Illinois, USA.
J Clin Apher. 2011 Dec;26(6):362-5. doi: 10.1002/jca.20312. Epub 2011 Oct 29.
Encephalitis associated with autoantibodies directed against the N-methyl-D-aspartate receptor (NMDAR) is usually a paraneoplastic syndrome that presents in young females with ovarian teratomas. We report a case of a previously healthy 14-year-old girl with sudden-onset paranoia, hallucinations, hyperactivity, increased speech, decreased sleep, seizures, and violent behavior deteriorating to catatonia. Her cerebrospinal fluid tested positive for anti-NMDAR antibodies. She was treated with five sessions of therapeutic plasma exchange (TPE) after having failed therapy with antibiotics, intravenous steroids, intravenous immunoglobulin (IVIG), one dose of rituximab, and seven sessions of electroconvulsive therapy (ECT). The American Society for Apheresis assigns a Category III (Grade 2C) recommendation for TPE in paraneoplastic neurologic syndromes; however, apheresis specifically for anti-NMDAR encephalitis has not been well studied. Literature review revealed two case reports describing outstanding improvement in patients with anti-NMDAR encephalitis following TPE. We report no improvement in our patient's symptoms after plasma exchange and discuss possible reasons for why it failed along with review of the literature.
与抗N-甲基-D-天冬氨酸受体（NMDAR）自身抗体相关的脑炎通常是一种副肿瘤综合征，多见于患有卵巢畸胎瘤的年轻女性。我们报告一例病例，患者为一名14岁既往健康的女孩，突发妄想、幻觉、多动、言语增多、睡眠减少、癫痫发作及暴力行为，病情逐渐发展为紧张症。她的脑脊液抗NMDAR抗体检测呈阳性。在使用抗生素、静脉注射类固醇、静脉注射免疫球蛋白（IVIG）、一剂利妥昔单抗及七次电休克治疗（ECT）均无效后，她接受了五次治疗性血浆置换（TPE）。美国单采学会对副肿瘤性神经综合征的TPE治疗给出了III类（2C级）推荐；然而，专门针对抗NMDAR脑炎的血液成分单采尚未得到充分研究。文献综述显示，有两篇病例报告描述了TPE治疗后抗NMDAR脑炎患者症状显著改善。我们报告该患者血浆置换后症状无改善，并结合文献综述讨论了治疗失败的可能原因。
