Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a well-defined clinical condition with a heterogeneous immunological profile

The pharyngeal-cervical-brachial variant of Guillain-Barre syndrome (GBS) is rare. This variant has its own specific clinical aspects but a heterogeneous immunological profile. A 38-year-old male who presented progressive symptoms of dysphagia, dysphonia and weakness hindering movement of the upper limbs. Two weeks earlier, the patient had presented acute self-limiting diarrhoea. He displayed predominantly right-side bilateral peripheral facial paresis, and paresis of the 9th and 12th cranial nerves and upper limbs (proximal 0/5, distal 1/5), although strength in the lower limbs was not compromised; sensitivity was preserved and deep tendon reflexes were diminished (0 in the upper limbs and + in the lower extremities). At 24 hours after admission, he suffered severe respiratory distress and had to be moved to the Intensive Care Unit with invasive mechanical ventilation. An electronystagmography/electromyogram study revealed severe demyelinating damage that predominantly involved the brain, but also included a small axonal component. The most striking immunological finding was the presence of positive IgG anti-GQ1b, IgM anti-GMI and IgM anti-asialo GM1 titres. The pharyngeal-cervical-brachial variant is a clinical condition with its own clinical characteristics and well-established diagnostic criteria that allow it to be distinguished from the other variants of GBS. Our case highlights the wide clinical spectrum of acute inflammatory demyelinating polyradiculoneuropathies and the important degree of heterogeneity that exists as regards the immunological parameters.