Pradhan Ravi R, Yadav Sant K, Yadav Shreebodh K
Internal Medicine, Tribhuvan University Institute of Medicine, Kathmandu, NPL.
Internal Medicine, Provincial Hospital, Janakpurdham, NPL.
Cureus. 2020 Feb 13;12(2):e6983. doi: 10.7759/cureus.6983.
Guillian-Barre Syndrome (GBS) typically presents as symmetrical ascending flaccid muscle weakness with areflexia, and with or without sensory symptoms. However, some patients may present atypically, and accordingly, different variants of GBS have been reported in the literature. Pharyngeal-cervical-brachial variant is one of the rare variants and is characterized by muscle weakness extending from the oropharyngeal and neck area to the proximal upper extremities. Many physicians and neurologists are unfamiliar about pharyngeal-cervical-brachial variant, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. Herein, we report a case of pharyngeal-cervical-brachial variant of GBS. To the best of our knowledge, this is the first reported case of pharyngeal-cervical-brachial variant of GBS in children from Nepal. A 14-year-old Asian male presented with weakness of bilateral upper limb, dysphagia, and nasal intonation of voice. A diagnosis of pharyngeal-cervical-brachial variant of GBS was made after excluding all other possible differentials and based on cerebrospinal fluid analysis and nerve conduction study. The patient improved following conservative management. Pharyngeal-cervical-brachial variant of GBS should always be considered in any patient presenting with symmetrical upper limb weakness and bulbar palsy. This is to ensure early diagnosis, treatment, and follow-up of the potential complications.
吉兰-巴雷综合征(GBS)通常表现为对称性上行性弛缓性肌无力伴腱反射消失,可伴有或不伴有感觉症状。然而,一些患者可能表现不典型,因此,文献中报道了GBS的不同变异型。咽颈臂型变异是罕见的变异型之一,其特征是肌无力从口咽和颈部区域延伸至近端上肢。许多内科医生和神经科医生对咽颈臂型变异并不熟悉,该型常被误诊为脑干卒中、重症肌无力或肉毒中毒。在此,我们报告一例GBS咽颈臂型变异病例。据我们所知,这是尼泊尔儿童中首例报道的GBS咽颈臂型变异病例。一名14岁亚洲男性出现双侧上肢无力、吞咽困难和声音鼻音化。在排除所有其他可能的鉴别诊断并基于脑脊液分析和神经传导研究后,诊断为GBS咽颈臂型变异。患者经保守治疗后病情好转。对于任何出现对称性上肢无力和延髓麻痹的患者,都应始终考虑GBS咽颈臂型变异。这是为了确保对潜在并发症进行早期诊断、治疗和随访。