患有抗α-氨基-3-羟基-5-甲基-4-异构烯丙酸受体(AMPAR)脑炎的患者特征和预后。
Patient characteristics and outcome in patients with anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis.
发表日期:2023 Apr 03
作者:
Jingfang Lin, Jierui Wang, Jinmei Li
来源:
Brain Structure & Function
摘要:
抗α-氨基-3-羟基-5-甲基-4-异唑烷丙酸受体(AMPAR)脑炎是一种罕见的自身免疫性脑炎亚型。本文报道了中国西部被诊断为抗AMPAR脑炎的患者,并重点关注他们的临床表现,影像结果,治疗策略和预后。回顾性收集和分析了2018年8月至2021年7月期间在中国西部医院神经病学中心被诊断为抗AMPAR脑炎的患者数据。根据自身免疫性脑炎的诊断标准,共纳入了9例患者。其中4名患者(44%)为男性,呈现病症时的中位年龄为54岁(范围,25-85岁)。短期记忆丧失是最常见的首发症状。在3名患者中发现了其他类型的自身抗体。其中4名患者在呈现病症后被发现有肿瘤:2名患有小细胞肺癌,1名患有卵巢畸胎瘤,还有1名患有胸腺瘤。所有患者接受了一线免疫治疗,并对其中8名患者进行了随访(中位数20周,范围4-78周)。在最后一次随访中,有3名患者表现出良好预后(改良RANKIN量表[ mRS] 0-2; 37.5%)。有5名患者表现出不良预后(mRS 3-6; 62.5%):2人的情况发生了极小的变化并一直住院治疗,2人残留了严重的认知障碍,1名患者在随访期间死亡。患有肿瘤的患者预后更差。最后,在随访期间只有1名患者发生了复发。临床医生应考虑抗AMPAR脑炎作为中老年患者主要出现急性或亚急性短期记忆损伤的可选择诊断之一。长期预后与肿瘤存在有关。 ©2023. Fondazione Società Italiana di Neurologia。
Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis is a rare subtype of autoimmune encephalitis. We report patients diagnosed with anti-AMPAR encephalitis in western China, focusing on their clinical presentations, imaging results, treatment strategies, and prognosis.Data from patients diagnosed with anti-AMPAR encephalitis in the neurology center of West China Hospital from August 2018 to July 2021 were retrospectively collected and analyzed. Based on the diagnostic criteria of autoimmune encephalitis, nine cases were included.Four patients (44%) were males, and the median age at presentation was 54 years (range, 25-85). Short-term memory loss was the most common initial symptom. Additional types of autoantibodies were identified in three patients. After presentation, four patients were found to have tumors: two with small cell lung cancer, one with ovarian teratoma, and one with thymoma. All patients accepted first-line immune therapy, and follow-up was available from 8 patients (median 20 weeks, range 4-78). At the last follow-up, three patients showed good outcomes (modified Rankin scale [mRS] 0-2; 37.5%). Five patients showed poor outcomes (mRS 3-6; 62.5%): two had minimal changes and remained hospitalized, two had residual severe cognitive impairments, and one patient died during follow-up. Outcomes were worse among patients with tumors. Finally, only one patient experienced relapse during follow-up.Anti-AMPAR encephalitis should be considered in the differential diagnosis for middle- and senior-aged patients who present with predominantly acute or subacute short-term memory impairment. The long-term prognosis is correlated with the presence of a tumor.© 2023. Fondazione Società Italiana di Neurologia.