Lancaster Eric
Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Curr Opin Neurol. 2017 Jun;30(3):354-360. doi: 10.1097/WCO.0000000000000448.
Autoantibodies to Central nervous system (CNS) metabotropic receptors are associated with a growing family of autoimmune brain diseases, including encephalitis, basal ganglia encephalitis, Ophelia syndrome, and cerebellitis. The purpose of this review is to summarize the state of knowledge regarding the target receptors, the neurological autoimmune disorders, and the pathogenic mechanisms.
Antibodies to the γ-aminobutyric acid B receptor are associate with limbic encephalitis and severe seizures, often with small cell lung cancers. Metabotropic glutamate receptor 5 (mGluR5) antibodies associate with Ophelia syndrome, a relatively mild form of encephalitis linked to Hodgkin lymphoma. mGluR1 antibodies associate with a form of cerebellar degeneration, and also Hodgkin lymphoma. Antibodies to Homer 3, a protein associated with mGluR1, have also been reported in two patients with cerebellar syndromes. Dopamine-2 receptor antibodies have been reported by one group in children with basal ganglia encephalitis and other disorders.
CNS metabotropic receptor antibodies may exert direct inhibitory effects on their target receptors, but the evidence is more limited than with autoantibodies to ionotropic glutamate receptors. In the future, improved recognition of these patients may lead to better outcomes. Understanding the molecular mechanisms of the diseases may uncover novel treatment strategies.
针对中枢神经系统（CNS）代谢型受体的自身抗体与越来越多的自身免疫性脑疾病相关，包括脑炎、基底节脑炎、奥菲莉亚综合征和小脑炎。本综述的目的是总结关于靶受体、神经自身免疫性疾病和致病机制的知识现状。
γ-氨基丁酸B受体抗体与边缘性脑炎和严重癫痫发作相关，常与小细胞肺癌有关。代谢型谷氨酸受体5（mGluR5）抗体与奥菲莉亚综合征相关，这是一种与霍奇金淋巴瘤相关的相对轻度的脑炎形式。mGluR1抗体与一种小脑变性形式相关，也与霍奇金淋巴瘤有关。在两名患有小脑综合征的患者中也报道了与mGluR1相关的蛋白质荷马3的抗体。一组研究人员在患有基底节脑炎和其他疾病的儿童中报道了多巴胺-2受体抗体。
中枢神经系统代谢型受体抗体可能对其靶受体产生直接抑制作用，但证据比离子型谷氨酸受体自身抗体更有限。未来，对这些患者的更好识别可能会带来更好的治疗结果。了解这些疾病的分子机制可能会揭示新的治疗策略。
