Paradiso G, Tripoli J, Galicchio S, Fejerman N
Hospital Nacional de Pediatría Juan Garrahan, Department of Neurology, Buenos Aires, Argentina.
Ann Neurol. 1999 Nov;46(5):701-7. doi: 10.1002/1531-8249(199911)46:5<701::aid-ana4>3.0.co;2-7.
We evaluated 61 children with Guillain-Barré syndrome, 14 months to 14 years of age, admitted to the Hospital Nacional de Pediatria in Buenos Aires. According to the electrodiagnostic findings, they fit into two groups, those with acute motor axonal neuropathy (AMAN) (18 patients) and those with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (43 patients). Ninety percent of the children with AMAN resided in suburban or rural areas without running water, whereas half of the AIDP patients lived in a metropolitan district. Summer and winter months showed a higher incidence of both variants. Children with AMAN were younger, evolved more acutely, reached a higher maximum disability score, required assisted ventilation more often, had lower mean level of cerebrospinal fluid protein, improved more slowly, and had a poorer outcome 6 months and 12 months after onset. Electrophysiological findings in those with AIDP revealed a pattern of severe diffuse slowing in children 5 years old or younger and a multifocal pattern in children 6 years old or older. This difference was not reflected in the clinical picture. In contrast, AMAN showed a uniform pattern with normal sensory conduction, severely reduced compound muscle action potential amplitude, near normal conduction velocity, and early denervation. Epidemiological, clinical, electrodiagnostic, cerebrospinal fluid, and prognostic data indicate that these variants of Guillain-Barré syndrome should be regarded as different entities.
我们评估了61名年龄在14个月至14岁之间、入住布宜诺斯艾利斯国立儿科医院的吉兰-巴雷综合征患儿。根据电诊断结果,他们分为两组,急性运动轴索性神经病(AMAN)组(18例患者)和急性炎症性脱髓鞘性多发性神经根神经病(AIDP)组(43例患者)。90%的AMAN患儿居住在没有自来水的郊区或农村地区,而AIDP患者中有一半生活在市区。夏季和冬季这两种类型的发病率都较高。AMAN患儿年龄更小,病情进展更急,达到的最高残疾评分更高,更常需要辅助通气,脑脊液蛋白平均水平更低,恢复更慢,发病后6个月和12个月时预后更差。AIDP患儿的电生理结果显示,5岁及以下儿童表现为严重弥漫性减慢模式,6岁及以上儿童表现为多灶性模式。这种差异在临床表现中未体现出来。相比之下,AMAN表现为一种统一模式,感觉传导正常,复合肌肉动作电位幅度严重降低,传导速度接近正常,且早期出现失神经改变。流行病学、临床、电诊断、脑脊液和预后数据表明,吉兰-巴雷综合征的这些类型应被视为不同的疾病实体。