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儿童吉兰-巴雷综合征的临床变异型。

Clinical variants of Guillain-Barré syndrome in children.

机构信息

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.

出版信息

Pediatr Neurol. 2012 Aug;47(2):91-6. doi: 10.1016/j.pediatrneurol.2012.05.011.

Abstract

Guillain-Barré syndrome is characterized by acute progressive weakness, areflexia, and maximal motor disability that occur within 4 weeks of onset. Various clinical subtypes have been described since the original description of the syndrome. This study aimed to identify characteristics of clinical variants of Guillain-Barré syndrome through retrospective review of cases in Chang Gung Children's Hospital from 2000-2010. Forty-three Guillain-Barré syndrome patients were evaluated based on clinical presentations and an electrodiagnostic study. The most frequent variant of Guillain-Barré syndrome was demyelinating polyneuropathy (67.4%), followed by acute axonal neuropathy (7.0%), Miller Fisher syndrome (7.0%), Bickerstaff brainstem encephalitis (7.0%), pharyngo-cervical-brachial variant (4.7%), and polyneuritis cranialis (4.7%). Follow-up revealed that 35 recovered satisfactorily, eight were persistently disabled, and none died during hospitalization. At the earliest stage, differentiating clinical variants from typical Guillain-Barré syndrome was difficult. Children with clinical variants of Guillain-Barré syndrome are more likely to manifest rapid onset from disease onset to nadir, increasing the severity of disability, cranial nerve involvement, urine incontinence, respiratory impairment, and need for ventilator support than in typical Guillain-Barré syndrome.

摘要

格林-巴利综合征的特点是急性进行性无力、反射消失和最大运动功能障碍,这些症状在发病后 4 周内出现。自该综合征最初描述以来,已经描述了各种临床亚型。本研究旨在通过回顾性分析长庚儿童医院 2000-2010 年的病例,确定格林-巴利综合征临床变异型的特征。根据临床表现和电诊断研究,评估了 43 例格林-巴利综合征患者。最常见的格林-巴利综合征变异型为脱髓鞘性多神经病(67.4%),其次为急性轴索性神经病(7.0%)、米勒-费舍尔综合征(7.0%)、比克斯特夫脑干脑炎(7.0%)、咽颈臂变异型(4.7%)和颅神经病(4.7%)。随访发现,35 例患者恢复良好,8 例持续残疾,住院期间无死亡。在最早阶段,区分临床变异型与典型格林-巴利综合征较为困难。与典型格林-巴利综合征相比,格林-巴利综合征临床变异型患儿从发病到疾病最低点的发病更快,残疾程度加重,更易出现颅神经受累、尿失禁、呼吸功能障碍和需要呼吸机支持。

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