431 - Guillain-Barré-Syndrome (GBS) an Extremely Rare Diagnosis of the Pharyngeal-Cervical-Brachial (PCB) variant: A Case Report

Case Diagnosis: Guillain-Barré-Syndrome (GBS) pharyngeal-cervical-brachial (PCB) variant

Case Description:
A 56-year-old male presented with left greater than right upper extremity weakness, areflexia, dysphagia, speech difficulties, and right-sided facial droop worsening over thirty hours, with ten days of flu symptoms. Head imaging was normal. Lumbar puncture revealed albuminocytologic disassociation, and paraneoplastic autoantibody (CASPR2+) prompting further imaging which revealed a kidney mass. He was determined to have PCB variant of GBS and was managed with intravenous immunoglobulin, plasmapheresis, and gastrostomy tube for dysphagia. Rehabilitation admission one month after initial presentation showed incomplete tetraplegia and upper worse than lower extremity weakness. He was weaned to a regular diet but required the use of a power wheelchair on discharge.

Discussions:
GBS is a rare autoimmune sequala most often preceded by infectious disease. There are four clinical presentations of GBS: Acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute sensory and motor axonal neuropathy, and Miller-Fischer syndrome (MFS). Typically, patients experience symmetric ascending weakness, with or without abnormal sensory symptoms. Cerebral spinal fluid albuminocytologic dissociation is common to all GBS variants but only occurs in 50% of patients within the first week of disease. It is thought that the PCB variant is a localized subtype of GBS with axonal rather than demyelinating neuropathy. Some believe its similarities to MFS in both symptoms (ophthalmoplegia, areflexia, ataxia) and serological profiles place both on a continuous spectrum. The PCB variant constitutes less than 1% of GBS cases, with only around 200 cases reported in the literature. Those affected tend to be middle-aged, with slight male predominance.

Conclusions: Patients with the PCB variant of GBS have predominantly upper extremity and facial weakness. Rare and debilitating, patients can have various presentations leading to disability. This brings up important new questions about the underlying immunologic response to malignancy as well as future serological profiling.