Department of Neurology, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC, 20010, USA.
Curr Neurol Neurosci Rep. 2011 Apr;11(2):187-94. doi: 10.1007/s11910-010-0169-4.
Paraneoplastic neurologic syndromes are rare disorders that have potentially devastating effects on the developing brain. Recently, there has been increased interest in possible immunotherapy for these disorders. Recognition of paraneoplastic syndromes in children may lead to early detection and treatment of the pediatric cancer and may diminish the neurologic damage that is the major source of morbidity in children with successfully treated tumors. This article reviews the presenting symptoms, immunology, long-term sequelae, and management options for paraneoplastic neurologic syndromes, focusing on those most commonly reported in children: opsoclonus-myoclonus ataxia, limbic encephalitis, and anti-NMDAR encephalitis. The child neurologist plays an important role in recognizing these disorders, initiating a tumor search, and directing ongoing treatment and management of neurologic symptoms after oncologic treatment is complete. Given the rarity of these conditions, multisite collaborative efforts are needed to develop standardized approaches to characterization and treatment.
副肿瘤性神经系统综合征是罕见的疾病,可能对发育中的大脑产生毁灭性的影响。最近,人们对这些疾病的可能免疫治疗产生了浓厚的兴趣。在儿童中识别副肿瘤性综合征可能导致对儿科癌症的早期发现和治疗,并可能减少成功治疗肿瘤的儿童中发病率的主要来源的神经损伤。本文综述了副肿瘤性神经系统综合征的临床表现、免疫学、长期后果和治疗选择,重点介绍了儿童中最常见的几种综合征:眼阵挛-肌阵挛-共济失调、边缘性脑炎和抗 NMDAR 脑炎。儿童神经病学家在识别这些疾病、启动肿瘤筛查以及在肿瘤治疗完成后指导神经症状的持续治疗和管理方面发挥着重要作用。鉴于这些情况的罕见性,需要多地点合作努力制定标准化的方法来进行特征描述和治疗。