Samad Navira, Wong Jennifer
Department of Endocrinology, Monash Health, Melbourne, Victoria, Australia.
BMJ Case Rep. 2018 Aug 27;2018:bcr-2018-225745. doi: 10.1136/bcr-2018-225745.
AMPA receptor (AMPAR) antibodies are a group of recently discovered antibodies which target the neuronal synaptic proteins causing B-cell (immune) mediated neuronal damage, resulting in various neurologic syndromes depending on the area of central nervous system involvement. These syndromes are mostly reversible if treated early. Tumour association has been reported in up to 60% of cases in the most recent case series with lung, breast, ovarian cancers and thymomas being the most commonly associated malignancies with these antibodies. We present here the first case of AMPAR encephalitis associated with medullary thyroid cancer. Our patient presented with cognitive dysfunction and behavioural changes over a period of 3 weeks, with a full recovery after starting immunotherapy, once the diagnosis of AMPAR limbic encephalitis was established. This case highlights the importance of early diagnosis and management of AMPAR encephalitis as these patients respond well to immunotherapy and can have an almost complete recovery.
α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体(AMPAR)抗体是一组最近发现的抗体,其靶向神经元突触蛋白,导致B细胞(免疫)介导的神经元损伤,根据中枢神经系统受累区域的不同,会引发各种神经综合征。如果早期治疗,这些综合征大多是可逆的。在最近的病例系列中,高达60%的病例报告了肿瘤相关性,其中肺癌、乳腺癌、卵巢癌和胸腺瘤是与这些抗体最常相关的恶性肿瘤。我们在此报告首例与甲状腺髓样癌相关的AMPAR脑炎病例。我们的患者在3周内出现认知功能障碍和行为改变,一旦确诊为AMPAR边缘叶脑炎并开始免疫治疗后完全康复。该病例强调了早期诊断和治疗AMPAR脑炎的重要性,因为这些患者对免疫治疗反应良好,几乎可以完全康复。