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Takahide Nagashima, MD, PhD; Michiaki Koga, MD, PhD; Masaaki Odaka, MD, PhD; Koichi Hirata, MD, PhD; Nobuhiro Yuki, MD, PhD

Arch Neurol. 2007;64(10):1519-1523.

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.

Author Affiliations: Department of Neurology and Research Institute for Neuroimmunological Diseases, Dokkyo Medical University School of Medicine, Tochigi, Japan.

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