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

Arch Neurol. 2007 Oct;64(10):1519-23. doi: 10.1001/archneur.64.10.1519.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Campylobacter Infections / complications
  • Campylobacter Infections / epidemiology
  • Campylobacter jejuni
  • Child
  • Encephalitis / physiopathology
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Gangliosides / immunology
  • Guillain-Barre Syndrome / classification
  • Guillain-Barre Syndrome / complications
  • Guillain-Barre Syndrome / physiopathology*
  • Humans
  • Immunoglobulin G / analysis
  • Infections / complications
  • Infections / immunology
  • Male
  • Middle Aged
  • Muscle Weakness / etiology
  • Muscle Weakness / physiopathology*
  • Neck Muscles / physiopathology*
  • Neurologic Examination
  • Pharyngeal Muscles / physiopathology*
  • Reflex, Stretch / physiology
  • Retrospective Studies
  • Upper Extremity / physiopathology*


  • Gangliosides
  • Immunoglobulin G