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笨手笨脚、跌跌撞撞、摸索探寻:一名3岁男性的肌无力、跨阈步态和面部下垂症状

Bumbling, Stumbling, Fumbling: Weakness, Steppage Gait, and Facial Droop in a 3-Year-Old Male.

作者信息

Whelan Aviva J, Tolaymat Abdullah, Rainey Shane C

机构信息

University of Illinois College of Medicine at Peoria, Peoria, IL, USA.

出版信息

Glob Pediatr Health. 2019 Jul 24;6:2333794X19865858. doi: 10.1177/2333794X19865858. eCollection 2019.

DOI:10.1177/2333794X19865858
PMID:31384634
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6657125/
Abstract

A previously healthy, unimmunized, 3-year-old Caucasian boy presented to the emergency department with right-sided facial droop, clumsiness, and intermittent bilateral hip pain. Two weeks ago, he had 24 hours of self-resolving rhinorrhea and fever. Examination was significant for right facial nerve palsy, lower extremity pain, areflexia of his right lower extremity, and diminished reflexes of his left lower extremity. He was admitted for urgent magnetic resonance imaging of the brain. Cerebrospinal fluid (CSF) protein was 85 mg/dL with elevated albumin and immunoglobulin, and CSF white blood cell was 3 cells/mm. Serum immunoglobulin (Ig) M and IgG were elevated. There was concern for Guillain-Barré syndrome (GBS). He was started on intravenous IG (IVIG) and was treated for presumed infection. Weakness and gait disturbances in a child can present the clinician with a diagnostic challenge. Gait disturbance may indicate a neurological lesion anywhere from the central nervous system to the peripheral nerves, neuromuscular junction, or muscle. In the present case, the combination of peripheral facial palsy, presumed neuropathic pain, gait difficulties, and areflexia in the setting of an antecedent respiratory illness were suggestive of GBS. The cornerstone treatments involve hospitalization to facilitate continuous monitoring for serious sequelae, such as acute respiratory failure and cardiac dysrhythmia, followed by immunotherapy with IVIG or plasma exchange. Gait disturbance and weakness in a child is a diagnostic challenge. GBS is the most common cause of acute paralysis in the Western world and should remain high on the clinician's differential diagnosis. However, patients with GBS may also present nonclassically with extremity pain and cranial nerve palsies.

摘要

一名此前健康、未接种疫苗的3岁白人男孩因右侧面部下垂、行动笨拙和间歇性双侧髋关节疼痛被送往急诊科。两周前,他有过24小时自行缓解的鼻漏和发热症状。检查发现有右侧面神经麻痹、下肢疼痛、右下肢反射消失以及左下肢反射减弱。他被收治入院,接受紧急脑部磁共振成像检查。脑脊液(CSF)蛋白为85mg/dL,白蛋白和免疫球蛋白升高,脑脊液白细胞为3个/mm。血清免疫球蛋白(Ig)M和IgG升高。怀疑患有吉兰 - 巴雷综合征(GBS)。他开始接受静脉注射免疫球蛋白(IVIG)治疗,并针对疑似感染进行治疗。儿童的虚弱和步态障碍可能给临床医生带来诊断挑战。步态障碍可能表明从中枢神经系统到周围神经、神经肌肉接头或肌肉的任何部位存在神经病变。在本病例中,周围性面瘫、推测的神经性疼痛、步态困难以及在前驱呼吸道疾病背景下的反射消失,提示为GBS。关键治疗措施包括住院以促进对严重后遗症(如急性呼吸衰竭和心律失常)的持续监测,随后进行IVIG或血浆置换免疫治疗。儿童的步态障碍和虚弱是一个诊断挑战。GBS是西方世界急性瘫痪最常见的原因,应始终在临床医生的鉴别诊断中占据重要地位。然而,GBS患者也可能以非典型的肢体疼痛和颅神经麻痹表现。

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