Abstract
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.
Highlights
Guillian-Barre Syndrome (GBS) is an immune-mediated, inflammatory, demyelinating, polyradiculopathy, typically characterized by acute-onset symmetrical flaccid muscle weakness with decreased or absent deep tendon reflexes [1]
Several clinical reports have revealed the different variants of GBS
Pharyngealcervical-brachial (PCB) variant of GBS is characterized by muscle weakness involving oropharyngeal, neck, and upper extremity muscles, and was first reported by Ropper in 1986 [4,5]
Summary
Guillian-Barre Syndrome (GBS) is an immune-mediated, inflammatory, demyelinating, polyradiculopathy, typically characterized by acute-onset symmetrical flaccid muscle weakness with decreased or absent deep tendon reflexes [1]. A 14-year-old Asian male presented to our hospital with two weeks history of weakness of bilateral upper limbs, nasal intonation of voice, difficulty in swallowing and walking. Patient was in his usual state of health two weeks back when he experienced upper limb weakness, which was insidious in onset, gradually progressing for the first week, and static after; associated with tingling sensations and numbness. Differential diagnosis of intracranial mass occupying lesion, myasthenia gravis, botulism, diphtheric polyneuropathy, brainstem stroke, and PCB variant of GBS were made His routine blood and urine investigations were within normal limits. On follow-up visit after four weeks, the deep tendon reflexes recovered
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