摘要
吉兰―巴雷综合征(GBS)是一种以快速进行性四肢麻木无力为特点的急性炎性脱髓鞘性多发性多神经根神经病。随着外源性神经节苷脂在临床的广泛应用,该药引起的副作用也逐渐显现,外源性神经节苷脂相关性GBS是其最严重的并发症,临床上主要表现为轴索型GBS,以四肢弛缓性瘫痪为首发症状,表现为急性、严重且快速进展的周围神经受累,较其他轴索型GBS病情重,恢复时间长,预后差。目前发病机制尚不明确。静脉注射人免疫球蛋白是其具有循证医学证据的治疗方法,目前已取代血浆置换成为GBS首选治疗方法,推荐剂量为0.4 g/(kg·d),连续静滴5 d,大剂量激素治疗的效果还有待进一步探讨。早发现、早诊断、尽早停用外源性神经节苷脂、及时应用人血免疫球蛋白冲击治疗和康复治疗,可改善预后。
Guillain-Barrésyndrome(GBS)is a type of acute inflammatory demyelinating polyneuropathy characterized by rapid progressive numbness and weakness of the limbs.As the clinical application of exogenous gangliosides increases,the side effects of this treatment have gradually emerged.Exogenous ganglioside-associated GBS(EG-GBS)is the most serious side effect of this treatment.The primary clinical manifestation of EG-GBS is axonal GBS,which is characterized by acute,serious and rapid progressive peripheral nerve involvement that results in limb flaccid paralysis.EG-GBS is more serious than other types of axonal GBS and has a long recovery time and poor prognosis.However,the pathogenesis of EG-GBS is currently unclear.Intravenous human immunoglobulin(hIg)is an evidence-based medical treatment that has replaced plasma exchange as the preferred treatment for EG-GBS.This treatment is administered at a recommended dose of 0.4 g/(kg·d)by continuous intravenous drip for 5 days.Although high-dose hormonal therapy is also a promising treatment for EG-GBS,its efficacy requires further evaluation.In summary,early detection,early diagnosis,early discontinuation of exogenous ganglioside,and timely application of hIg pulse therapy and rehabilitation therapy can improve the prognosis of EG-GBS.
作者
李元贵
杨晓苏
LI Yuan-Gui;YANG Xiao-Su(Department of Neurology,General Hospital of Ningxia Medical University,Yinchuan 750004,Ningxia,China;Department of Neurology,Xiangya Hospital,Central South University,Changsha 410008,Hunan,China)
出处
《国际神经病学神经外科学杂志》
2020年第6期633-636,共4页
Journal of International Neurology and Neurosurgery
