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[婴儿期吉兰-巴雷综合征:神经电生理检查的重要性]

[Guillain-Barré syndrome in infancy: The importance of electroneuromyography].

作者信息

Vedrenne-Cloquet M, Maincent K, Billette de Villemeur T, Mayer M

机构信息

Service de neuropédiatrie, hôpital Armand-Trousseau, Assistance publique-Hôpitaux de Paris, 26, avenue Arnold-Netter, 75012 Paris, France.

Service de neuropédiatrie, hôpital Armand-Trousseau, Assistance publique-Hôpitaux de Paris, 26, avenue Arnold-Netter, 75012 Paris, France; Centre de référence de l'Est parisien des maladies neuromusculaires, 75012 Paris, France; DHU i2B (inflammation-immunopathologie-biothérapie), 75012 Paris, France.

出版信息

Arch Pediatr. 2016 Feb;23(2):184-7. doi: 10.1016/j.arcped.2015.10.004. Epub 2015 Nov 5.

Abstract

Guillain-Barré Syndrome (GBS) is rare in infancy, and the diagnosis of atypical forms is difficult in this age range. The main differential diagnoses include congenital neuropathy. Biological and electrophysiological investigations remain important to confirm diagnosis and start treatment quickly. We report the case of an 8-month-old boy who presented with acquired hypotonia due to progressive descending limb paralysis, predominant in the upper limbs, associated with unexplained severe neutropenia. GBS was diagnosed thanks to the association of albuminocytologic dissociation on cerebrospinal fluid and demyelinating sensomotor polyradiculoneuropathy on electroneuromyography. Only one cycle of treatment with intravenous immunoglobulins was sufficient to achieve complete recovery after 1 year. Physicians should know that atypical forms of GBS exist in infants, in order to recognize the syndrome, rule out differential diagnoses, and start treatment as soon as possible. Medical follow-up remains important before and after remission, especially in infants, to identify relapses, which might be the symptom of a genetic neuropathy or a chronic inflammatory disease.

摘要

吉兰-巴雷综合征(GBS)在婴儿期较为罕见,在这个年龄段诊断非典型形式的GBS很困难。主要的鉴别诊断包括先天性神经病。生物学和电生理检查对于确诊并迅速开始治疗仍然很重要。我们报告了一例8个月大男孩的病例,该男孩因进行性下行性肢体麻痹(以上肢为主)出现后天性肌张力减退,并伴有原因不明的严重中性粒细胞减少症。由于脑脊液中存在蛋白细胞分离以及神经电生理检查显示脱髓鞘感觉运动性多神经根神经病,该病例被诊断为GBS。仅一个疗程的静脉注射免疫球蛋白治疗就足以使其在1年后完全康复。医生应该知道婴儿中存在GBS的非典型形式,以便识别该综合征,排除鉴别诊断,并尽快开始治疗。在缓解前后进行医学随访仍然很重要,尤其是对于婴儿,以识别复发情况,复发可能是遗传性神经病或慢性炎症性疾病的症状。

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