title: Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome.
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.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  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.
  
  
  METHODS
  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%.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology and Research Institute for Neuroimmunological Diseases, Dokkyo Medical University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan.
authors.forename: Takahide
authors.initials: T
authors.lastname: Nagashima
authors.forename: Michiaki
authors.initials: M
authors.lastname: Koga
authors.forename: Masaaki
authors.initials: M
authors.lastname: Odaka
authors.forename: Koichi
authors.initials: K
authors.lastname: Hirata
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Yuki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002169:Campylobacter Infections / Q000150:complications / Q000453:epidemiology; D016123:Campylobacter jejuni; D002648:Child; D004660:Encephalitis / Q000503:physiopathology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005732:Gangliosides / Q000276:immunology; D020275:Guillain-Barre Syndrome / Q000145:classification / Q000150:complications / Q000503:physiopathology*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007239:Infections / Q000150:complications / Q000276:immunology; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology / Q000503:physiopathology*; D009334:Neck Muscles / Q000503:physiopathology*; D009460:Neurologic Examination; D010609:Pharyngeal Muscles / Q000503:physiopathology*; D012026:Reflex, Stretch / Q000502:physiology; D012189:Retrospective Studies; D034941:Upper Extremity / Q000503:physiopathology*
chemical_list: D005732:Gangliosides; D007074:Immunoglobulin G