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对伴有皮质脊髓束受累的克罗地亚吉兰-巴雷综合征(GBS)患儿进行长期临床和电生理评估。

Long term clinical and electrophysiological assessment of Croatian children with corticospinal tract involvement in Guillain-Barré syndrome (GBS).

机构信息

Department of Pediatrics, University Hospital Zagreb, Medical School, University of Zagreb, 10000 Zagreb, Rebro, Kispatićeva 12, Zagreb, Croatia.

出版信息

Eur J Paediatr Neurol. 2010 Sep;14(5):391-9. doi: 10.1016/j.ejpn.2010.03.006. Epub 2010 Aug 3.

DOI:10.1016/j.ejpn.2010.03.006
PMID:20678946
Abstract

Guillain-Barré syndrome (GBS) is characterized by areflexia. Hyperreflexia is reported in acute motor axonal neuropathy (AMAN). We present 16 children with GBS at the age of 14 months to 13 years. All children studied fulfilled accepted diagnostic criteria for GBS. Hyperreflexia or positive Babinski sign were obtained in all children studied during follow up. Brain and spinal cord MR scans did not reveal any significant structural and morphological abnormalities of central nervous system. The children were examined clinically and electromyoneurographically 2-5 times successively during 1-8.5 years of follow-up. According to established electrodiagnostic criteria demyelinating form of GBS was most common (68%) compared to axonal (18,7%) or mixed form (12,5%). No children had antecendent Campylobacter jejuni infection. Antiganglioside antibodies were detected in 18,7% of patients associated with demyelinating or mixed (axonal/demyelinating) form. Time to nadir and recovery period of walking ability is prolonged more often in demyelinating GBS. Clinical improvement occur earlier compared to improvement of abnormal electrophysiological parameters.Outcome was excellent in 11 in the period 1 month-8.5 years. Hyperreflexia usually appeared in recovery period suggesting involvement of upper motor neurons or spinal interneurons occurring in Croatian children with both demyelinating and axonal form of GBS usually associated with milder course of disease.

摘要

格林-巴利综合征(GBS)的特征是反射消失。急性运动轴索性神经病(AMAN)则有反射亢进。我们报告了 16 例年龄在 14 个月至 13 岁的 GBS 患儿。所有研究的患儿均符合 GBS 的公认诊断标准。在随访期间,所有研究的患儿均出现反射亢进或巴宾斯基征阳性。脑和脊髓磁共振扫描未显示中枢神经系统有任何明显的结构性和形态学异常。在 1-8.5 年的随访期间,对患儿进行了 2-5 次临床和肌电图神经检查。根据既定的电诊断标准,脱髓鞘型 GBS 最为常见(68%),而轴索性(18.7%)或混合性(12.5%)。没有患儿有前期空肠弯曲菌感染。在 18.7%的患者中检测到抗神经节苷脂抗体,与脱髓鞘或混合(轴索性/脱髓鞘性)形式相关。脱髓鞘性 GBS 的运动能力的潜伏期和恢复期更长。与异常电生理参数的改善相比,临床改善更早出现。在 1 个月至 8.5 年的期间,11 例患儿的预后良好。反射亢进通常出现在恢复期,提示上运动神经元或脊髓中间神经元受累,这种情况在克罗地亚同时存在脱髓鞘和轴索性 GBS 患儿中较为常见,通常与疾病的较轻病程相关。

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