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吉兰-巴雷综合征致 3 例闭锁综合征

Locked-in syndrome in three children with Guillain-Barré syndrome.

机构信息

Department of Pediatric Neurology, School of Medicine, Pereira Rossell Children's Hospital, Montevideo, Uruguay.

出版信息

Pediatr Neurol. 2011 Aug;45(2):125-8. doi: 10.1016/j.pediatrneurol.2011.03.005.

DOI:10.1016/j.pediatrneurol.2011.03.005
PMID:21763955
Abstract

Locked-in syndrome is a rare disorder in childhood. It resembles brain death, but patients are fully conscious, and incapable of communicating because of the complete paralysis of voluntary muscles. Although it can be caused by Guillain-Barré syndrome, it is rarely reported in pediatrics. We describe three pediatric cases of locked-in syndrome in patients with Guillain-Barré syndrome presenting acute tetraplegia, areflexia, cranial nerve involvement, and albuminocytologic dissociation in the cerebrospinal fluid. Electrophysiologic studies indicated acute motor axonal polyradiculoneuropathy in one patient, and acute motor sensory axonal polyradiculoneuropathy in the other two. Most Guillain-Barré syndrome patients with locked-in syndrome demonstrate nerve inexcitability in neurophysiologic studies, poor clinical outcomes, and increased risk of sequelae.

摘要

闭锁综合征在儿童中较为罕见。它类似于脑死亡,但患者意识完全清醒,由于随意肌完全瘫痪而无法进行交流。尽管它可能由格林-巴利综合征引起,但在儿科中很少见。我们描述了三例儿童闭锁综合征病例,这些病例均由格林-巴利综合征引起,表现为急性四肢瘫痪、反射消失、颅神经受累以及脑脊液中的白蛋白细胞分离。电生理研究表明,其中 1 例为急性运动轴索性多发性神经病,另外 2 例为急性运动感觉轴索性多发性神经病。大多数患有闭锁综合征的格林-巴利综合征患者在神经生理研究中表现出神经兴奋性降低、临床预后不良以及后遗症风险增加。

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