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.
| Original language | English |
|---|---|
| Pages (from-to) | 742-747 |
| Number of pages | 6 |
| Journal | Neurology |
| Volume | 86 |
| Issue number | 8 |
| DOIs | |
| Publication status | Published - 2016 Feb 23 |
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ASJC Scopus subject areas
- Clinical Neurology
Cite this
Acute bulbar palsy as a variant of Guillain-Barré syndrome. / Korean Inflammatory Neuropathy Consortium.
In: Neurology, Vol. 86, No. 8, 23.02.2016, p. 742-747.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Acute bulbar palsy as a variant of Guillain-Barré syndrome
AU - Korean Inflammatory Neuropathy Consortium
AU - Kim, Jong Kuk
AU - Kim, Byung Jo
AU - Shin, Ha Young
AU - Shin, Kyong Jin
AU - Nam, Tai Seung
AU - Oh, Jeeyoung
AU - Suh, Bum Chun
AU - Yoon, Byeol A.
AU - Park, Hwan Tae
AU - Huh, So Young
AU - Oh, Seong Il
AU - Bae, Jong Seok
PY - 2016/2/23
Y1 - 2016/2/23
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=84959215798&partnerID=8YFLogxK
U2 - 10.1212/WNL.0000000000002256
DO - 10.1212/WNL.0000000000002256
M3 - Article
C2 - 26718574
AN - SCOPUS:84959215798
VL - 86
SP - 742
EP - 747
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 8
ER -