Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barre syndrome

Background: Pharyngeal-cervical-brachial weakness (PCB) is considered a variant of Guillain-Barre syndrome (GBS). Because of its rarity, there have been no studies of large numbers of patients with PCB. Objective: To clarify the nosological classification of PCB. Design: Retrospective study. Setting: Academic research. Patients: Medical records were reviewed of patients who manifested progressive weakness of the pharynx, neck, and upper limbs within 4 weeks of initial onset. Main Outcome Measures: Clinical features were analyzed, and antecedent infections and antiganglioside antibodies were investigated. Results: Diagnoses for 100 patients were "pure PCB" (n=13), PCB with preserved muscle stretch reflexes (n=8), GBS overlap (n=48), Fisher syndrome overlap (n=26), and Bickerstaff brainstem encephalitis overlap (n=5). Serological test results showed that 31.0% of antecedent infections in PCB were caused by Campylobacter jejuni. Of the antiganglioside antibodies tested, anti-GT1a IgG antibodies were positive in 51.0% of the patients. Anti-GQ1b IgG antibodies (a serological marker of Fisher syndrome and Bickerstaff brainstem encephalitis) were positive in 39.0%. The IgG antibodies to GM1, GM1b, GD1a, or GalNAc-GD1a ( serological markers of an axonal GBS subtype) were positive in 27.0%. Conclusion: This large study identified the clinical profiles of PCB. Clinical overlapping, frequent C jejuni infection, and common antiganglioside antibodies present in PCB, GBS, Fisher syndrome, and Bickerstaff brainstem encephalitis provide conclusive evidence that PCB and these conditions form a continuous spectrum.