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  • Title: Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome.
    Author: Nagashima T, Koga M, Odaka M, Hirata K, Yuki N.
    Journal: Arch Neurol; 2007 Oct; 64(10):1519-23. PubMed ID: 17923636.
    Abstract:
    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.
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