摘要
吉兰-巴雷综合征(Guillian-Barre syndrome,GBS)是脊髓灰质炎消火后最常见的小儿急性迟缓性瘫痪性疾病,主要表现为快速进展的瘫痪、腱反射消失和脑脊液蛋白细胞分离。根据病理和神经电生理特点,可将其分为4种主要亚型:急性炎症性脱髓鞘性多发性神经炎(acute inflammatory demyelinating polyneuropathy, AIDP)、急性运动轴索性神经病(acute motor axonal neuropathy,AMAN)、急性运动感觉轴索性神经病(acute motor- sensory axonal neuropaty,AMSAN)和Miller-Fisher综合征(MFS)。目前一致认为它是一种免疫介导的器官特异性周围神经病,体液免疫和细胞免疫不同程度地参与了各个亚型病变的发生。根据其典型临床表现及神经传导功能检测,不仅能明确GBS的诊断,还有助于临床分型。免疫调节治疗能有效地缓解神经损伤,加速患者恢复。文章着重介绍近年有关该病的分型、病因及发病机制、临床及电生理特征、鉴别诊断和治疗方面的研究进展。
Guillian-Barré syndrome （GBS） is the most frequent cause of acute flaccid paralysis in the post-polio era, which is characterized clinically by rapidly progressing paralysis, areflexia, and albumino-cytological dissociation in cerebral-spinal fluids. Based on the electroneurophysiologic and pathologic characteristics, this disorder could be classified into four different patterns such as acute inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, acute motor sensory axonal neuropathy, and the Miller-Fisher syndrome. It was commonly considered that GBS is an organ specific immune-mediated peripheral neuropathy, both cell-mediated and humoral immune pathways variably contribute to the pathogenesis of each pattern. Typical clinical manifestations and nerve conduction studies may help in correct diagnosis and classification of suspected GBS patients. Treatment with immunomedulation can effectively ameliorate nerve damage and accelerate recovery of patients. This review emphasizes the recent advances in understanding of the subtyping, pathogenesis, etiology, clinical features, differential diagnosis, electrophysiological diagnostic criteria and treatment of GBS.
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2007年第2期81-84,共4页
Journal of Clinical Pediatrics
关键词
吉兰-巴雷综合征
分型
病因
发病机制
治疗
Guillian - Barré syndrome
subtyping
etiology
pathogenesis
treatment
