Department of Neurology, Mayo Clinic Rochester Minnesota, 200 1st ST SW, Rochester, MN, 55905, USA.
Neurocrit Care. 2020 Feb;32(1):286-294. doi: 10.1007/s12028-019-00875-5.
Immune response against neuronal and glial cell surface and cytosolic antigens is an important cause of encephalitis. It may be triggered by activation of the immune system in response to an infection (para-infectious), cancer (paraneoplastic), or due to a patient's tendency toward autoimmunity. Antibodies directed toward neuronal cell surface antigens are directly pathogenic, whereas antibodies with intracellular targets may become pathogenic if the antigen is transiently exposed to the cell surface or via activation of cytotoxic T cells. Immune-mediated encephalitis is well recognized and may require intensive care due to status epilepticus, need for invasive ventilation, or dysautonomia. Patients with immune-mediated encephalitis may become critically ill and display clinically complex and challenging to treat movement disorders in over 80% of the cases (Zhang et al. in Neurocrit Care 29(2):264-272, 2018). Treatment options include immunotherapy and symptomatic agents affecting dopamine or acetylcholine neurotransmission. There has been no prior published guidance for management of these movement disorders for the intensivist. Herein, we discuss the immune-mediated encephalitis most likely to cause critical illness, clinical features and mechanisms of movement disorders and propose a management algorithm.
针对神经元和神经胶质细胞表面及细胞内抗原的免疫反应是脑炎的一个重要病因。它可能由免疫系统对感染(副感染性)、癌症(副肿瘤性)的激活而引发,也可能是由于患者自身免疫倾向所致。针对神经元细胞表面抗原的抗体具有直接致病性,而针对细胞内靶标的抗体如果抗原短暂暴露于细胞表面或通过细胞毒性 T 细胞的激活,则可能具有致病性。免疫介导性脑炎是众所周知的,可能需要重症监护,因为可能出现癫痫持续状态、需要有创通气或自主神经功能障碍。超过 80%的免疫介导性脑炎患者可能会出现严重疾病,并表现出临床上复杂且具有挑战性的运动障碍(Zhang 等人,Neurocrit Care 29(2):264-272, 2018)。治疗选择包括免疫疗法和影响多巴胺或乙酰胆碱神经递质的对症药物。对于重症监护医生来说,之前没有针对这些运动障碍的管理的已发表指南。在此,我们讨论了最有可能导致严重疾病的免疫介导性脑炎、运动障碍的临床特征和机制,并提出了一种管理算法。