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.
Keywords: axonal neuropathy; guillain-barré syndrome; inflammatory polyradiculoneuropathy; oropharyngeal and cervicobrachial weakness; pharyngeal-cervical-brachial variant.
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