儿童中枢神经系统副肿瘤综合征的研究进展

Progress of paraneoplastic neurological syndrome of central nervous system in children

神经系统副肿瘤综合征(paraneoplastic neurological syndrome,PNS)是非神经系统肿瘤通过远隔效应损伤神经系统的一组临床综合征。儿童PNS常以神经系统异常起病,临床表现不典型,易误诊。肿瘤相关神经抗体是PNS诊断、分类及判断预后的重要指标。PNS的治疗应尽早进行,主要以原发肿瘤治疗和免疫治疗为主。该文通过对儿童常见的中枢神经系统PNS,如眼阵挛-肌阵挛综合征、边缘性脑炎和自身免疫性脑炎等进行综述,以提高临床医师的认识。

Paraneoplastic neurological syndrome(PNS)is a group diseases of nervous system,which is associated with cancer in remote immune-mediated mechanisms.PNS is rare and easy to be misdiagnosed because of its atypical clinical manifestations.Onconeural associated antibodies are important to diagnose and forecast the prognosis of PNS.Potential tumor treatment and immunotherapy are essential for relieving nervous system abnormalities.This article reviews common childhood PNS,such as opsoclonus-myoclonus syndrome,limbic encephalitis and anti-NMDAR encephalitis,in order to increase awareness of PNS,reduce misdiagnosis and improve prognosis.