Urriola Nicolás, Soosapilla Kavie, Drummond James, Thieben Mark
Department of Neurology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
Junior Medical Unit, Royal North Shore Hospital.
BMJ Case Rep. 2019 Feb 21;12(2):e227893. doi: 10.1136/bcr-2018-227893.
Autoimmune encephalitides are a potentially devastating group of treatable disorders with a wide variety of clinical presentations. The most studied autoimmune encephalitis is caused by antibodies to the N-methyl-D-aspartate glutamate receptor. A rarer cause is due to antibodies against the evolutionarily related α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR). The full assortment of electroencephalogram (EEG) and clinical descriptions of the latter are yet to be fully described. A 44-year-old woman with impaired consciousness and subsequent coma characterised by an isoelectric EEG was diagnosed with AMPAR-antibody limbic encephalitis. MRI revealed temporal T2 hyperintensities that improved with immunosuppression, although leaving marked cortical atrophy. Gradual clinical improvement saw the development of aggressive bruxism requiring botulinum toxin injection with eventual meaningful clinical recovery. This case expands the clinical spectrum of AMPAR limbic encephalitis to include aggressive bruxism, and highlights that despite poor clinical and EEG findings at the outset, recovery is still possible.
自身免疫性脑炎是一组具有潜在毁灭性的可治疗疾病，临床表现多种多样。研究最多的自身免疫性脑炎是由针对N-甲基-D-天冬氨酸谷氨酸受体的抗体引起的。一种较罕见的病因是由于针对进化相关的α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体（AMPAR）的抗体。后者完整的脑电图（EEG）表现和临床描述尚未得到充分阐述。一名44岁意识障碍并随后昏迷的女性，脑电图表现为等电位，被诊断为AMPAR抗体边缘性脑炎。磁共振成像（MRI）显示颞叶T2高信号，经免疫抑制治疗后有所改善，尽管仍遗留明显的皮质萎缩。随着临床逐渐改善，出现了严重的磨牙症，需要注射肉毒杆菌毒素，最终实现了有意义的临床康复。该病例扩展了AMPAR边缘性脑炎的临床谱，使其包括严重磨牙症，并强调尽管一开始临床和脑电图表现不佳，但仍有可能康复。
