Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome.

BACKGROUND

Pharyngeal-cervical-brachial weakness (PCB) is considered a variant of Guillain-Barré 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.