Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report
Abstract Background Fulminant Guillain-Barré syndrome (GBS) is characterized clinically by rapid progression of severe symptoms, such as the absence of brainstem reflexes, complete tetraplegia and respiratory arrest. The clinical course of fulminant GBS remains unclear. Here, we report a patient wit...
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doaj-642c157d30264b09a5e048d6df4edf612020-11-25T03:12:43ZengBMCBMC Neurology1471-23772019-06-011911410.1186/s12883-019-1376-5Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case reportYoshitsugu Nakamura0Mikiko Motoki1Takahiko Hirose2Takafumi Hosokawa3Shimon Ishida4Shigeki Arawaka5Department of Internal Medicine IV, Division of Neurology, Osaka Medical CollegeDepartment of Internal Medicine IV, Division of Neurology, Osaka Medical CollegeDepartment of Internal Medicine IV, Division of Neurology, Osaka Medical CollegeDepartment of Internal Medicine IV, Division of Neurology, Osaka Medical CollegeDepartment of Internal Medicine IV, Division of Neurology, Osaka Medical CollegeDepartment of Internal Medicine IV, Division of Neurology, Osaka Medical CollegeAbstract Background Fulminant Guillain-Barré syndrome (GBS) is characterized clinically by rapid progression of severe symptoms, such as the absence of brainstem reflexes, complete tetraplegia and respiratory arrest. The clinical course of fulminant GBS remains unclear. Here, we report a patient with fulminant GBS, who showed severe weakness of the pharyngeal-cervical-branchial (PCB) area in the recovery phase. Case presentation A 38-year-old man rapidly developed fulminant GBS. In blood examination, he was positive for a broad range of anti-ganglioside antibodies, including anti-GQ1b, GT1a, GT1b, GD1a, GD1b and GD3 IgG antibodies. We performed immunosuppressive therapies using intravenous immunoglobulin and intravenous methylprednisolone. Although disturbance of consciousness and weakness of the distal upper and lower limbs improved gradually, weakness of the oropharynx, neck, and proximal upper limbs were resistant to these therapies. Anti-GT1a IgG antibodies remained persistently positive. Consequently, mechanical ventilation and tube feeding were required for 7 and 10 months, respectively. Two years later, weakness of the proximal upper limbs and mild respiratory dysfunction remained as sequelae. Conclusion Anti-GT1a IgG antibodies are known to be detected in patients with the PCB variant of GBS. In fulminant GBS, the persistent presence of anti-GT1a IgG antibodies may be associated with occurrence of severe PCB-like weakness in the recovery phase.http://link.springer.com/article/10.1186/s12883-019-1376-5Guillain-Barré syndromePharyngeal-cervical-brachial weaknessFulminantAnti-GT1a antibodyCase report |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Yoshitsugu Nakamura Mikiko Motoki Takahiko Hirose Takafumi Hosokawa Shimon Ishida Shigeki Arawaka |
spellingShingle |
Yoshitsugu Nakamura Mikiko Motoki Takahiko Hirose Takafumi Hosokawa Shimon Ishida Shigeki Arawaka Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report BMC Neurology Guillain-Barré syndrome Pharyngeal-cervical-brachial weakness Fulminant Anti-GT1a antibody Case report |
author_facet |
Yoshitsugu Nakamura Mikiko Motoki Takahiko Hirose Takafumi Hosokawa Shimon Ishida Shigeki Arawaka |
author_sort |
Yoshitsugu Nakamura |
title |
Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report |
title_short |
Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report |
title_full |
Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report |
title_fullStr |
Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report |
title_full_unstemmed |
Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report |
title_sort |
fulminant guillain-barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report |
publisher |
BMC |
series |
BMC Neurology |
issn |
1471-2377 |
publishDate |
2019-06-01 |
description |
Abstract Background Fulminant Guillain-Barré syndrome (GBS) is characterized clinically by rapid progression of severe symptoms, such as the absence of brainstem reflexes, complete tetraplegia and respiratory arrest. The clinical course of fulminant GBS remains unclear. Here, we report a patient with fulminant GBS, who showed severe weakness of the pharyngeal-cervical-branchial (PCB) area in the recovery phase. Case presentation A 38-year-old man rapidly developed fulminant GBS. In blood examination, he was positive for a broad range of anti-ganglioside antibodies, including anti-GQ1b, GT1a, GT1b, GD1a, GD1b and GD3 IgG antibodies. We performed immunosuppressive therapies using intravenous immunoglobulin and intravenous methylprednisolone. Although disturbance of consciousness and weakness of the distal upper and lower limbs improved gradually, weakness of the oropharynx, neck, and proximal upper limbs were resistant to these therapies. Anti-GT1a IgG antibodies remained persistently positive. Consequently, mechanical ventilation and tube feeding were required for 7 and 10 months, respectively. Two years later, weakness of the proximal upper limbs and mild respiratory dysfunction remained as sequelae. Conclusion Anti-GT1a IgG antibodies are known to be detected in patients with the PCB variant of GBS. In fulminant GBS, the persistent presence of anti-GT1a IgG antibodies may be associated with occurrence of severe PCB-like weakness in the recovery phase. |
topic |
Guillain-Barré syndrome Pharyngeal-cervical-brachial weakness Fulminant Anti-GT1a antibody Case report |
url |
http://link.springer.com/article/10.1186/s12883-019-1376-5 |
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