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儿童吉兰-巴雷综合征非典型重症变异型的新观察:诊断、疾病分类及治疗过程中仍面临的挑战

A New Observation of an Atypical and Severe Variant of the Guillain-Barre Syndrome in a Child: Remaining Challenges for Diagnosis, Nosologic Classification, and Therapeutic Course.

作者信息

Pons Linda, Manel Véronique, Ville Dorothée, Javouhey Etienne, Bordet Fabienne

机构信息

Service de réanimation pédiatrique, Hôpital Femme Mère Enfant, HCL, Lyon, France.

Service d'explorations fonctionnelles neuropédiatriques, HFME, HCL, Lyon, France.

出版信息

Child Neurol Open. 2015 Oct 26;2(4):2329048X15609053. doi: 10.1177/2329048X15609053. eCollection 2015 Oct-Dec.

Abstract

Guillain-Barré syndrome is a rare acute polyradiculoneuropathy. Several variants and unusual presentations have been described, particularly in pediatrics. In most cases, making an early diagnosis is challenging due to the treatments that consist in the rapid administration of intravenous immunoglobulin or plasma exchange. The authors present the case of a 7-year-old boy with an atypical and severe axonal Guillain-Barré syndrome, associated with . When he was admitted, febrile respiratory failure was the main focus, and then he presented signs of acute polyneuropathy with cranial nerve palsy and brief hyperreflexia. Mechanical ventilation was required for 48 days as well as 2 cycles of intravenous immunoglobulin. The authors describe all the medical challenges that the authors encountered. This case highlights the fact that respiratory distress can be the main clinical symptom in children. This delays the establishment of a correct diagnosis, even more so when neurological manifestations are abundant and unusual.

摘要

吉兰-巴雷综合征是一种罕见的急性多发性神经根神经病。已经描述了几种变异型和不寻常的表现形式,尤其是在儿科患者中。在大多数情况下,由于治疗方法包括快速静脉注射免疫球蛋白或进行血浆置换,因此早期诊断具有挑战性。作者报告了一名7岁男孩的病例,该男孩患有非典型重症轴索性吉兰-巴雷综合征,并伴有……。入院时,发热性呼吸衰竭是主要问题,随后他出现了急性多发性神经病的体征,伴有颅神经麻痹和短暂的反射亢进。机械通气持续了48天,并进行了2个周期的静脉注射免疫球蛋白治疗。作者描述了所遇到的所有医疗挑战。该病例突出了呼吸窘迫可能是儿童主要临床症状这一事实。这会延迟正确诊断的建立,当神经学表现丰富且不寻常时更是如此。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2722/5417028/9678642bf7dc/10.1177_2329048X15609053-fig1.jpg

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