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Anti-ganglioside complex antibody profiles in a recurrent complicated case of GQ1b-seronegative miller fisher syndrome and Bickerstaff brainstem encephalitis: a case report

Authors :
Hiroto Ito
Yuki Hatanaka
Yuki Fukami
Yumiko Harada
Rei Kobayashi
Hisashi Okada
Ayumi Uchibori
Atsuro Chiba
Satoshi Okuda
Source :
BMC Neurology, Vol 18, Iss 1, Pp 1-5 (2018)
Publication Year :
2018
Publisher :
BMC, 2018.

Abstract

Abstract Background Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE) are a group of autoimmune neurological disorders (GBS spectrum disorder) that rarely recur. Recently, anti-ganglioside complex antibodies (GSC-Abs) were identified in patients with GBS spectrum disorder. However, there has been no case report describing GSC-Abs profiles in a recurrent case showing different phenotypes. Case presentation We report the case of a 33-year-old male patient with GQ1b-seronegative BBE-GBS after two prior episodes of MFS-GBS. Our patient showed ophthalmoplegia, ataxia, areflexia and a weakness of the extremities (MFS and GBS symptoms) in all episodes. In the episode reported here, our patient showed disturbed consciousness and an extensor response to cutaneous plantar stimulation was observed (BBE symptoms), with severe disability and requirement for artificial respiration management. GSC-Abs detected in previous episodes were also detected in the subsequent episodes, while new GSC-Abs emerged in each episode. Interestingly, whereas antibodies to GA1/GQ1b and GA1/GT1a, which are commonly identified in patients with GBS, MFS or BBE, appeared in all episodes, antibodies to GD1a/GD1b and GD1b/GT1b, which are predominantly associated with severe disability and the requirement for artificial respiration management in GBS, emerged for the first time in this episode. Conclusion This study reports novel phenomena about the GSC-Abs profiles and its relationship with clinical features in a case with recurrent GBS spectrum disorder, showing different phenotypes in different episodes. Further studies are required to reveal the significance of the GSC-Abs profiles in recurrent GBS spectrum disorder.

Details

Language :
English
ISSN :
14712377
Volume :
18
Issue :
1
Database :
Directory of Open Access Journals
Journal :
BMC Neurology
Publication Type :
Academic Journal
Accession number :
edsdoj.7e024b557686472fae786da1cf6c3691
Document Type :
article
Full Text :
https://doi.org/10.1186/s12883-018-1077-5