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Acute bulbar palsy as a variant of Guillain-Barré syndrome

 Jong Kuk Kim  ;  Byung-Jo Kim  ;  Ha Young Shin  ;  Kyong Jin Shin  ;  Tai-Seung Nam  ;  Jeeyoung Oh  ;  Bum Chun Suh  ;  Byeol-A Yoon  ;  Hwan Tae Park  ;  So-Young Huh  ;  Seong-Il Oh  ;  Jong Seok Bae 
 NEUROLOGY, Vol.86(8) : 742-747, 2016 
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Adolescent ; Adult ; Aged ; Autoantibodies/blood ; Bulbar Palsy, Progressive/blood* ; Bulbar Palsy, Progressive/classification ; Bulbar Palsy, Progressive/diagnosis* ; Databases, Factual ; Female ; Guillain-Barre Syndrome/blood* ; Guillain-Barre Syndrome/classification ; Guillain-Barre Syndrome/diagnosis* ; Humans ; Immunoglobulin G/blood ; Male ; Middle Aged ; Young Adult
OBJECTIVE: To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barr? syndrome (GBS) and differentiate it from Miller Fisher syndrome (MFS) and pharyngeal-cervical-brachial (PCB) variants. METHODS: We analyzed cases of ABP without limb motor weakness based on a dataset containing clinical information and the results of antiganglioside antibodies assays for acute immune-mediated neuropathies. RESULTS: Eleven cases with an age at onset ranging from 18 to 65 years (mean 33.8 years) were identified as ABP-plus syndrome. All of the enrolled cases manifested with ABP as the predominant symptom, and with no limb weakness. The following features accompanied ABP in order of decreasing frequency: ophthalmoplegia (n = 9, 82%), ataxia (n = 9, 82%), and facial palsy (n = 6, 55%). An enzyme-linked immunosorbent assay study disclosed that immunoglobulin G (IgG) anti-GT1a antibodies were the most frequent (n = 11), followed by IgG anti-GQ1b antibodies (n = 6). CONCLUSIONS: We propose that ABP-plus syndrome without neck or limb weakness is a variant of GBS that is distinct from the MFS and PCB variants. The presence of IgG anti-GT1a antibodies can explain the relationships between the distinct clinical characteristics and the underlying pathomechanisms.
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1. College of Medicine (의과대학) > Dept. of Neurology (신경과학교실) > 1. Journal Papers
Yonsei Authors
Shin, Ha Young(신하영) ORCID logo https://orcid.org/0000-0002-4408-8265
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