Rössling Rosa, Prüss Harald
Department of Neurology and Experimental Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany.
German Center for Neurodegenerative Diseases (DZNE) Berlin, 10117 Berlin, Germany.
J Clin Med. 2020 Aug 19;9(9):2683. doi: 10.3390/jcm9092683.
Autoimmune encephalitis (AE) is a rapidly progressive inflammatory neurological disease. Underlying autoantibodies can bind to neuronal surfaces and synaptic proteins resulting in psychiatric symptoms, focal neurological signs, autonomic dysfunction and cognitive decline. Early and effective treatment is mandatory to reduce clinical symptoms and to achieve remission. Therapeutic apheresis, involving both plasma exchange (PE) and immunoadsorption (IA), can rapidly remove pathogenic antibodies from the circulation, thus representing an important first-line treatment in AE patients. We here review the most relevant studies regarding therapeutic apheresis in AE, summarizing the outcome for patients and the expanding clinical spectrum of treatment-responsive clinical conditions. For example, patients with slowly progressing cognitive impairment suggesting a neurodegenerative dementia can have underlying autoantibodies and improve with therapeutic apheresis. Findings are encouraging and have led to the first ongoing clinical studies assessing the therapeutic effect of IA in patients with anti-neuronal autoantibodies and the clinical presentation of dementia. Therapeutic apheresis is an established and well tolerated option for first-line therapy in AE and, potentially, other antibody-mediated central nervous system diseases.
自身免疫性脑炎（AE）是一种快速进展的炎症性神经疾病。潜在的自身抗体可与神经元表面和突触蛋白结合，导致精神症状、局灶性神经体征、自主神经功能障碍和认知功能下降。早期有效的治疗对于减轻临床症状和实现缓解至关重要。治疗性血液成分单采，包括血浆置换（PE）和免疫吸附（IA），可迅速从循环中清除致病性抗体，因此是AE患者重要的一线治疗方法。我们在此回顾了关于AE治疗性血液成分单采的最相关研究，总结了患者的治疗结果以及治疗反应性临床情况不断扩大的临床谱。例如，表现为缓慢进展的认知障碍提示神经退行性痴呆的患者可能存在潜在的自身抗体，并可通过治疗性血液成分单采得到改善。这些发现令人鼓舞，并促成了第一项正在进行的临床研究，评估IA对患有抗神经元自身抗体和痴呆临床表现患者的治疗效果。治疗性血液成分单采是AE以及可能的其他抗体介导的中枢神经系统疾病一线治疗的既定且耐受性良好的选择。
