Acute bulbar palsy as a variant of Guillain-Barre syndrome

作者：Kim Jong Kuk; Kim Byung Jo; Shin Ha Young; Shin Kyong Jin; Nam Tai Seung; Oh Jeeyoung; Suh Bum Chun; Yoon Byeol A; Park Hwan Tae; Huh So Young; Oh Seong Il; Bae Jong Seok^*

来源：Neurology, 2016, 86(8): 742-747.

DOI：10.1212/WNL.0000000000002256

Objective:
To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barre 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.

• 出版日期2016-2-23