IVIG- responsive multiple cranial neuropathy: a pharyngo-facial variant of Guillain-Barré syndrome.

Abstract

We report a case with symptoms of facial swelling, bilateral facial paralysis, dysphagia and aspiration. On electrophysiological studies, the right facial nerve was not excitable. The left facial nerve compound muscle action potential (CMAP) amplitude was severely dispersed and latency was mildly prolonged, consistent with demyelination. Cerebrospinal fluid examinations were normal. Anti-ganglioside antibodies and tumor markers were negative. Bickerstaff brainstem encephalitis, stroke, diabetes mellitus, vasculitis, sarcoidosis, Sjögren's syndrome, Melkersson-Rosenthal Syndrome, trauma, infectious diseases, toxicity, neoplasm, facial onset sensory and motor neuronopathy (FOSMN) and other degenerative diseases were excluded. Intravenous immunoglobulin therapy resolved symptoms of lower cranial nerve dysfunction. Clinically incomplete improvement of bilateral facial paralysis was observed. We conclude that IVIg therapy may improve the symptoms of multiple cranial nerve palsies due to pharyngo-facial variant of Guillain-Barré syndrome.