Acute bulbar palsy as a variant of Guillain-Barré syndrome.

Autor(es): 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,

Resumo: 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. 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. 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). 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.

Imprenta: Neurology, v. 86, n. 8, p. 742-747, 2016

Identificador do objeto digital: 10.1212/WNL.0000000000002256

Descritores: Guillain-Barre Syndrome - Antibodies

Data de publicação: 2016