Abstract
Guillain-Barré syndrome (GBS) is a heterogeneous group of acute immune-mediated polyradiculoneuropathy that typically presents with classic axonal or demyelinating sensory-motor type. However, there are variants of GBS with atypical presentation. We report a rare case of severe pharyngeal-sensory-ataxic variant of GBS associated with poor cardiac systolic function, elevated troponin, and positive anti-sulfatide IgM. The sensory symptom atypically started in the hands in an ascending pattern, which progressed to involve the trunk and face and, later, all limbs. It was associated with severe dysphagia, ataxia, and generalized areflexia but with preserved muscle strength in all extremities. Recognizing the atypical pattern of presentation and the ability to perform an accurate clinical localization are the utmost important initial steps in making the diagnosis. The patient showed complete recovery after immunoglobulin therapy.