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吉兰-巴雷综合征的咽-颈-臂变异型

Pharyngeal-Cervical-Brachial Variant of Guillain-Barré Syndrome.

作者信息

Moscona-Nissan Alberto, López-Hernández Juan Carlos, Seidman-Sorsby Alec, Cruz-Zermeño Mayte, Navalón-Calzada Andrea

机构信息

School of Medicine, Universidad Panamericana, Mexico City, MEX.

Neuromuscular Diseases, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX.

出版信息

Cureus. 2021 Oct 14;13(10):e18788. doi: 10.7759/cureus.18788. eCollection 2021 Oct.

DOI:10.7759/cureus.18788
PMID:34804654
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8592309/
Abstract

Guillain-Barré syndrome (GBS) represents the main cause of flaccid paralysis worldwide. Although most cases have a typical clinical presentation of symmetric ascending flaccid paralysis with areflexia or hyporeflexia, this disease may present as multiple clinical entities, therefore representing a diagnostic challenge for physicians, who should consider these variants when assessing neuropathies. The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with hyporeflexia or areflexia in the upper limbs. It is present in 3% of all Guillain-Barré syndrome cases and is characterized by axonal rather than demyelinating neuropathy.  We present a pharyngeal-cervical-brachial variant case in a 55-year-old male who presented to the neurological emergency department with a three-day history of progressive and continuous dysarthria, dysphagia to solids, and tongue numbness, later developing paresthesia and weakness in the upper limbs. On physical examination, slight bilateral facial weakness, limited soft palate elevation, absent gag reflex, and limited tongue lateralization were found. Additionally, weakness was found bilaterally in the upper limbs and the flexor and extensor muscles of the neck with preserved muscle strength in the lower limbs. The patient presented upper limb hyporeflexia with lower limb hyperreflexia. A lumbar puncture was performed, revealing protein levels of 35 mg/dL and no cells in the cerebrospinal fluid. Nerve conduction studies reported acute motor and sensory axonal neuropathy (AMSAN). Management of the patient consisted of IgG administration and nasogastric tube insertion.

摘要

吉兰-巴雷综合征(GBS)是全球弛缓性麻痹的主要病因。尽管大多数病例具有典型的临床表现,即对称性上行性弛缓性麻痹伴无反射或反射减退,但这种疾病可能表现为多种临床类型,因此对医生来说是一项诊断挑战,医生在评估神经病变时应考虑这些变异型。吉兰-巴雷综合征的咽颈臂(PCB)变异型的定义是迅速进展的口咽和颈臂肌无力,并伴有上肢反射减退或无反射。它占所有吉兰-巴雷综合征病例的3%,其特征是轴索性而非脱髓鞘性神经病变。我们报告一例55岁男性的咽颈臂变异型病例,该患者因进行性持续性构音障碍、固体食物吞咽困难和舌麻木3天就诊于神经急诊科,随后出现上肢感觉异常和无力。体格检查发现双侧轻度面部无力、软腭抬高受限、咽反射消失和舌侧偏受限。此外,双侧上肢及颈部屈伸肌无力,下肢肌力正常。患者上肢反射减退,下肢反射亢进。进行了腰椎穿刺,结果显示脑脊液蛋白水平为35mg/dL,无细胞。神经传导研究报告为急性运动和感觉轴索性神经病(AMSAN)。对该患者的治疗包括给予免疫球蛋白和插入鼻胃管。

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