Evans O B, Vedanarayanan V
Department of Pediatrics, University of Mississippi Medical Center, Jackson.
Pediatr Rev. 1997 Jan;18(1):10-6. doi: 10.1542/pir.18-1-10.
GBS is an acquired, monophasic illness of the peripheral nervous system that usually presents with a gait disturbance and clinical features of pain, weakness, and areflexia. The etiology of the disease is immune-mediated and directed against the peripheral nervous system myelin, axon, or both. A careful history, physical examination, and routine laboratory tests are necessary to make a clinical diagnosis and to exclude other disorders that cause acute weakness. Laboratory tests that support the diagnosis, such as an increased CSF protein and abnormal electrodiagnostic studies, may be normal early in the illness. The most serious complications during the acute phase of the disease are respiratory failure and autonomic disturbances. Plasma exchange or IVIG shortens the duration and severity of the disease significantly. The prognosis for children who have GBS generally is excellent for full and functional recovery, using modern intensive care for respiratory support and the management of other complications.
吉兰-巴雷综合征(GBS)是一种获得性单相周围神经系统疾病,通常表现为步态障碍以及疼痛、无力和腱反射消失等临床特征。该疾病的病因是免疫介导的,针对周围神经系统的髓鞘、轴突或两者。进行仔细的病史询问、体格检查和常规实验室检查对于做出临床诊断以及排除其他导致急性无力的疾病是必要的。支持诊断的实验室检查,如脑脊液蛋白升高和电诊断研究异常,在疾病早期可能是正常的。疾病急性期最严重的并发症是呼吸衰竭和自主神经功能紊乱。血浆置换或静脉注射免疫球蛋白(IVIG)可显著缩短疾病的持续时间和严重程度。对于患有GBS的儿童,采用现代重症监护进行呼吸支持和处理其他并发症,总体预后良好,有望完全且功能恢复。