He Nancy, Kartiko Michael C, Selim Mohammad
Internal Medicine, Creighton University School of Medicine, Omaha, USA.
Cureus. 2021 Apr 18;13(4):e14551. doi: 10.7759/cureus.14551.
Guillain-Barré Syndrome (GBS) is a rapidly evolving autoimmune inflammatory disease of the peripheral nerves. It classically presents with progressive symmetrical ascending muscle weakness and hyporeflexia. The pharyngeal-cervical-brachial (PCB) variant is a rare variant of GBS that is characterized by axonal rather than demyelinating neuropathy and presents with rapidly progressive oropharyngeal (facial palsy, dysarthria) and cervicobrachial weakness, associated upper limb weakness, and hyporeflexia. Because it is rare, the PCB variant of GBS is unfamiliar to many neurologists and it is often misdiagnosed as stroke, myasthenia gravis, or botulism. The prevalence of this variant is estimated to be about 3% of all GBS patients. We describe the only known case presentation of the PCB variant of GBS that presents with bilateral glossopharyngeal paralysis. A 39-year-old African American female presented with progressive oropharyngeal and cervicobrachial weakness along with bilateral glossopharyngeal paralysis. The patient was diagnosed based on clinical suspicion, presentation, and serum ganglioside antibodies.
吉兰-巴雷综合征(GBS)是一种周围神经迅速进展的自身免疫性炎症性疾病。其典型表现为进行性对称性上行性肌无力和反射减退。咽颈臂(PCB)变异型是GBS的一种罕见变异型,其特征为轴索性而非脱髓鞘性神经病变,表现为迅速进展的口咽(面瘫、构音障碍)和颈臂肌无力、相关上肢无力以及反射减退。由于其罕见,许多神经科医生对GBS的PCB变异型并不熟悉,它常被误诊为中风、重症肌无力或肉毒中毒。该变异型的患病率估计约占所有GBS患者的3%。我们描述了唯一已知的表现为双侧舌咽神经麻痹的GBS的PCB变异型病例。一名39岁非裔美国女性出现进行性口咽和颈臂肌无力以及双侧舌咽神经麻痹。患者根据临床怀疑、表现和血清神经节苷脂抗体得以确诊。