Jong Kuk Kim1, Byung-Jo Kim1, Ha Young Shin1, Kyong Jin Shin1, Tai-Seung Nam1, Jeeyoung Oh1, Bum Chun Suh1, Byeol-A Yoon1, Hwan Tae Park1, So-Young Huh1, Seong-Il Oh1, Jong Seok Bae2. 1. From the Departments of Neurology (J.K.K., B.-A.Y.) and Physiology (H.T.P.), College of Medicine, Dong-A University, Busan; the Department of Neurology (B.-J.K.), College of Medicine, Korea University, Seoul; the Department of Neurology (H.Y.S.), College of Medicine, Yonsei University, Seoul; the Department of Neurology (K.J.S., S.-I.O.), College of Medicine, Inje University, Busan; the Department of Neurology (T.-S.N.), College of Medicine, Chonnam National University, Gwangju; the Department of Neurology (J.O.), School of Medicine, Konkuk University, Seoul; the Department of Neurology (B.C.S.), Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul; the Department of Neurology (S.-Y.H.), College of Medicine, Kosin University, Busan; and the Department of Neurology (J.S.B.), College of Medicine, Hallym University, Seoul, Korea. 2. From the Departments of Neurology (J.K.K., B.-A.Y.) and Physiology (H.T.P.), College of Medicine, Dong-A University, Busan; the Department of Neurology (B.-J.K.), College of Medicine, Korea University, Seoul; the Department of Neurology (H.Y.S.), College of Medicine, Yonsei University, Seoul; the Department of Neurology (K.J.S., S.-I.O.), College of Medicine, Inje University, Busan; the Department of Neurology (T.-S.N.), College of Medicine, Chonnam National University, Gwangju; the Department of Neurology (J.O.), School of Medicine, Konkuk University, Seoul; the Department of Neurology (B.C.S.), Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul; the Department of Neurology (S.-Y.H.), College of Medicine, Kosin University, Busan; and the Department of Neurology (J.S.B.), College of Medicine, Hallym University, Seoul, Korea. lwsbae@naver.com.
Abstract
OBJECTIVE: To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barré 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.
OBJECTIVE: To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barré 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.
Authors: Jong Kuk Kim; Yoo Hwan Kim; Byeol A Yoon; Joong Yang Cho; Sun Young Oh; Ha Young Shin; Ji Soo Kim; Kee Hong Park; Sun Young Kim; Bum Chun Suh; Hung Youl Seok; Jin Hyuk Yoo; Jong Seok Bae Journal: J Clin Neurol Date: 2018-07 Impact factor: 3.077