Rarest of the rare: a case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome presenting with a demyelinating neuropathy.

BACKGROUND

Guillain-Barré syndrome (GBS) is a heterogeneous disease characterized by rapidly progressive, symmetrical limb weakness with hyporeflexia or areflexia. The rare pharyngeal-cervical-brachial (PCB) variant of GBS occurs in 3% of patients, presenting as rapidly progressive oropharyngeal and cervicobrachial weakness characterised by axonal, rather than a demyelinating neuropathy on nerve conduction studies.

CASE DESCRIPTION

A 35 year old male presented with a 5-day history of dysphagia, dysarthria, slurred speech and upper limb weakness. Physical examination showed bilateral facial nerve weakness and an impaired gag reflex. Nerve conduction study was suggestive of sensory and motor polyneuropathy of predominantly demyelinating variety. As the condition of the patient worsened, respiratory support was required and plasmapharesis was started. There was significant improvement in his condition after completion of 5 sessions of plasmapharesis.

CONCLUSION

The rare PCB variant may present with a predominantly demyelinating neuropathy. Early clinical recognition of this variant and prompt treatment with plasmapharesis is essential for preventing respiratory complications.