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吉兰-巴雷综合征:2000 年至 2017 年转诊至三级儿科神经肌肉中心的患者队列数据的回顾性分析:电生理发现、结局和简要文献复习。

Guillain-Barrè Syndrome-Retrospective Analysis of Data from a Cohort of Patients Referred to a Tertiary Care Pediatric Neuromuscular Center from 2000 to 2017: Electrophysiological Findings, Outcomes, and a Brief Literature Review.

机构信息

Child Neurology and Psychiatry Unit, Department of Pediatrics, AUSL-IRCCS di Reggio Emilia, 42121 Reggio Emilia, Italy.

Child Neuropsychiatry Unit, Azienda USL di Parma, 43121 Parma, Italy.

出版信息

Medicina (Kaunas). 2024 Sep 12;60(9):1490. doi: 10.3390/medicina60091490.

Abstract

Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paresis in children. The aim of this study was to describe the clinical and electrophysiological findings and outcomes of children with GBS diagnosed in our unit. Moreover, the literature on pediatric GBS cases from the past 5 years was reviewed. In this retrospective study, we reported data on 12 patients (9 male and 3 female patients; mean age: 5 y, 4 mo; range: 9 mo-11 y) clinically diagnosed at the Child Neurology Unit of the AUSL-IRCCS of Reggio Emilia, Italy, between 2000 and 2017 and a brief analysis/comparison with data from the literature. Data were collected from medical charts. In our cohort, male patients were more frequent than female ones (9 vs. 3), and upper respiratory tract infection (n = 8, 66.7%) was the most frequent triggering factor. The main clinical symptoms on admission were distal lower limbs' weakness with gait difficulties (83.3%), pain (50%), upper limbs' weakness (50%), and dysphagia for liquids (25%). Peripheral neurophysiological studies revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 66.6% of the children, acute motor and sensory axonal neuropathy (AMSAN) in 25%, and acute motor axonal neuropathy (AMAN) in 8.3%. Ten individuals (83.3%) received timely treatment with intravenous immunoglobulins (IVIG), and, out of these ten patients, 58% received concomitant treatment with IV methylprednisolone because of a progressive disease course. Complete remission was observed in the majority of individuals (91.6%) within 6 months of symptom onset. Different subtypes of GBS can affect children; however, the outcome is usually positive. Early treatment appears to be important for a favorable outcome.

摘要

格林-巴利综合征(GBS)是儿童急性弛缓性瘫痪最常见的原因。本研究旨在描述我们单位诊断的儿童 GBS 的临床和电生理表现及结局。此外,还回顾了过去 5 年有关儿科 GBS 病例的文献。在这项回顾性研究中,我们报告了 2000 年至 2017 年期间在意大利雷焦艾米利亚 AUSL-IRCCS 儿童神经病学单元临床诊断为 GBS 的 12 名患者(9 名男性和 3 名女性患者;平均年龄:5 岁,4 个月;范围:9 个月-11 岁)的数据,并与文献中的数据进行了简要分析/比较。数据从病历中收集。在我们的队列中,男性患者比女性患者更常见(9 比 3),上呼吸道感染(n = 8,66.7%)是最常见的触发因素。入院时的主要临床症状为下肢远端无力伴行走困难(83.3%)、疼痛(50%)、上肢无力(50%)和吞咽液体困难(25%)。周围神经生理研究显示,66.6%的儿童为急性炎症性脱髓鞘性多发性神经根神经病(AIDP),25%为急性运动感觉轴索性神经病(AMSAN),8.3%为急性运动轴索性神经病(AMAN)。10 名患者(83.3%)及时接受了静脉注射免疫球蛋白(IVIG)治疗,其中 58%的患者因疾病进展而同时接受了 IV 甲基强的松龙治疗。大多数患者(91.6%)在症状出现后 6 个月内完全缓解。不同类型的 GBS 可影响儿童,但结局通常较好。早期治疗似乎对预后良好很重要。

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