儿童吉兰-巴雷综合征的临床、电生理亚型和抗神经节苷脂抗体。

Clinical, electrophysiological subtypes and antiganglioside antibodies in childhood Guillain-Barré syndrome.

机构信息

Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.

出版信息

Neurol India. 2011 Sep-Oct;59(5):727-32. doi: 10.4103/0028-3886.86549.

DOI:10.4103/0028-3886.86549

PMID:22019659

Abstract

BACKGROUND

Guillain-Barré syndrome (GBS) has been the most common cause of flaccid paralysis in children after the decline in the incidence of poliomyelitis. There are not any published data from the Indian subcontinent documenting electrophysiological patterns and antiganglioside antibodies in pediatric GBS.

MATERIALS AND METHODS

The study population included children with GBS referred for electrodiagnostic evaluation and also children with GBS admitted to our institute between August 2006 and July 2007. Nerve conduction studies were done to determine GBS subtypes and serum antiganglioside antibodies were measured using enzyme-linked immunosorbent assay (ELISA). Clinical and electrophysiological features were correlated with antiganglioside antibody results.

RESULTS

Of the 43 (male to female ratio = 2.1:1) children studied, 97.6% had motor weakness, 76.7% had cranial nerve palsies, 13.9% had autonomic disturbances and respiratory paralysis was found in 9.3% children. Antecedent illness was recorded in 69.8% children. The GBS subtype distribution was as follows: acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 21 (48.8%), acute motor axonal neuropathy (AMAN) in 19 (44.2%), and 3 (6.9%) children were unclassified. The severity of illness was similar in both AMAN and AIDP subtypes and the recovery in both the subtypes was complete without any significant difference in the duration of recovery. Preceding diarrheal illness was more common in AMAN subtype as compared to AIDP subtype (57.9% vs. 4.7%, P = 0.007). Sensory symptoms were more common in AIDP subtype than in AMAN subtype (66.6% vs. 21%, P = 0.03}. The commonest ganglioside antibody was IgM GM2. Anti GM3 antibodies were exclusively seen in children with AMAN and IgG GD1b was significantly associated with (36.7 vs. 4%; P = 0.007) AMAN subtype. IgG GT1b was identified in 50% of patients with AIDP as compared to 22.7% in patients with AMAN.

CONCLUSION

In this study, AMAN subtype accounted for a significant proportion of pediatric GBS. AMAN was associated with diarrhea and specific antiganglioside antibodies. Recovery in children with GBS was complete, irrespective of the subtype.

摘要

背景

在小儿麻痹症发病率下降后，吉兰-巴雷综合征（GBS）成为儿童弛缓性瘫痪的最常见病因。目前，还没有来自印度次大陆的文献记录儿科 GBS 的电生理模式和神经节苷脂抗体。

材料与方法

本研究纳入了因电诊断评估而就诊的 GBS 患儿，以及 2006 年 8 月至 2007 年 7 月期间在我院住院的 GBS 患儿。进行神经传导研究以确定 GBS 亚型，并使用酶联免疫吸附试验（ELISA）测量血清神经节苷脂抗体。将临床和电生理特征与神经节苷脂抗体结果相关联。

结果

43 名患儿中（男女比例为 2.1:1），97.6%存在运动无力，76.7%存在颅神经麻痹，13.9%存在自主神经功能障碍，9.3%的患儿存在呼吸肌麻痹。69.8%的患儿存在前驱疾病。GBS 亚型分布如下：急性炎症性脱髓鞘性多发性神经病（AIDP）21 例（48.8%），急性运动轴索性神经病（AMAN）19 例（44.2%），3 例（6.9%）未分类。AMAN 和 AIDP 亚型的疾病严重程度相似，两种亚型的恢复均完全，恢复时间无显著差异。与 AIDP 亚型相比，AMAN 亚型更常出现前驱腹泻（57.9% vs. 4.7%，P = 0.007）。AIDP 亚型的感觉症状比 AMAN 亚型更常见（66.6% vs. 21%，P = 0.03）。最常见的神经节苷脂抗体是 IgM GM2。抗 GM3 抗体仅见于 AMAN 患儿，而 IgG GD1b 与 AMAN 亚型显著相关（36.7% vs. 4%，P = 0.007）。与 AMAN 患儿（22.7%）相比，AIDP 患儿中 IgG GT1b 的检出率为 50%。