Dale Russell C, Gorman Mark P, Lim Ming
aInstitute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Sydney, Australia bBoston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA cEvelina London Children's Hospital and Faculty of Life Sciences and Medicine, King's College London, London, UK.
Curr Opin Neurol. 2017 Jun;30(3):334-344. doi: 10.1097/WCO.0000000000000443.
Auto-antibodies that bind to conformational extracellular epitopes of neuronal receptors or synaptic proteins have provided clinicians with essential biomarkers in acute neurology. This review summarizes the current status and challenges in the field.
In children, anti-N-methyl-D-aspartate receptor encephalitis remains the most identifiable autoimmune encephalitis, although many patients have a clinical syndrome of brain inflammation in which no antibodies are identified. Anti-myelin oligodendrocyte glycoprotein antibody associated demyelination is now recognized as a major cause of monophasic and relapsing demyelination, often presenting with encephalopathy. We discuss the importance of auto-antibody detection methodology and the possible influence of intrathecal antibody synthesis on the speed of recovery and response to immune therapy. The current, often pragmatic rather than evidence-based therapeutic pathway will be discussed, highlighting key challenges such as the timing of second-line therapy, monitoring of disease activity, and identifying the patient who is responding poorly to treatment.
Although there have been significant developments, future priorities include the need for paediatric-specific consensus definitions for seronegative suspected autoimmune encephalitis, novel tools for monitoring patients with autoimmune encephalitis, consensus treatment recommendations, and neuroprotective strategies.
与神经元受体或突触蛋白的细胞外表位构象结合的自身抗体为临床医生提供了急性神经病学中的重要生物标志物。本综述总结了该领域的现状和挑战。
在儿童中，抗N-甲基-D-天冬氨酸受体脑炎仍然是最易识别的自身免疫性脑炎，尽管许多患者存在脑部炎症的临床综合征但未发现抗体。抗髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘现在被认为是单相和复发性脱髓鞘的主要原因，常伴有脑病。我们讨论了自身抗体检测方法的重要性以及鞘内抗体合成对恢复速度和免疫治疗反应的可能影响。将讨论当前通常务实而非基于证据的治疗途径，强调二线治疗的时机、疾病活动监测以及识别对治疗反应不佳的患者等关键挑战。
尽管取得了重大进展，但未来的优先事项包括需要针对血清阴性疑似自身免疫性脑炎的儿科特定共识定义、监测自身免疫性脑炎患者的新工具、共识治疗建议和神经保护策略。
