Case Report


Locked-in on Bora Bora—Chikungunya triggered Bickerstaff’s encephalitis mimicking brain death in French Polynesia

,  ,  ,  

1 Department of Anaesthesiology and Pain Therapy, University of Bern, Bern, Switzerland

2 Rega Air Rescue, Zurich, Switzerland

3 Department of Infectiology and Hygiene, University of Zurich, Zurich, Switzerland

Address correspondence to:

Lorenz Theiler

Freiburgstrasse 4, University Hospital Bern, CH-3010 Bern,

Switzerland

Message to Corresponding Author


Article ID: 100074Z06CC2020

doi: 10.5348/100074Z06CC2020CR

Access full text article on other devices

Access PDF of article on other devices

How to cite this article

Conrad C, Staub G, Braun D, Theiler L. Lockedin on Bora Bora—Chikungunya triggered Bickerstaff’s encephalitis mimicking brain death in French Polynesia. Case Rep Int 2020;9:100074Z06CC2020.

ABSTRACT


Introduction: This is a case report about a severe neurological manifestation of a tropical disease that resulted in an overlap syndrome mimicking brain death. We are showing a Chikungunya (CHIKV)-associated encephalitis triggering an overlapping autoimmunological disease [antibody negative Bickerstaff’s brainstem encephalitis (BBE) with Miller Fisher syndrome (MFS) and Guillain–Barré syndrome (GBS)] and the resulting complications. Until recently, CHIKV was considered to cause a relatively harmless self-limiting disease with Dengue-like symptoms (DENV). This case supports the idea of likely neuroinvasiveness of CHIKV, suggesting a potential high severity of this infection. Chikungunya shows a global development and evolution from a rarely seen pathogen toward an epidemic causing disease within only a few decades.

Case Report: A 74-year-old man fell sick during his holidays in French Polynesia. He initially presented with fever and a rash, which slowly progressed to hoarseness and back pain. Within one week, the patient developed numbness of the fingertips and difficulties breathing, finally resulting in a complete irresponsive neurological state. Local physicians assumed brain death caused by a cardiac event and voted for termination of mechanical ventilatory support. However, the patient′s wife perceived responses of her husband and initiated extensive repatriation by the Swiss Air Rescue, Rega. Strikingly, the patient recovered subsequently and is wheelchair mobile today.

Conclusion: This case demonstrates how tropical airborne virus diseases become directly visible effects of global warming and globalization, as the disease recently seems to appear in Europe and America. It exemplifies how a broader knowledge of parainfectious neurologic syndromes might help to save lives by avoiding misdiagnosis or potentially fatal therapeutic decisions.

Keywords: Bickerstaff′s brainstem encephalitis, Chikungunya, Guillain–Barré syndrome, Miller Fisher syndrome

SUPPORTING INFORMATION


Acknowledgments

The patient grants written consent to publish this case and the connected data. We thank him and his family for providing the data. We thank Elsevier, for granting permission to reuse Figure 2, as well as Prof. Dr. Claeys for providing permission to adapt the data of Figure 3.

Author Contributions

Carl Conrad - Substantial contributions to conception and design, Analysis of data, Interpretation of data, Drafting the article, Final approval of the version to be published

Gabriela Staub - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Dominique Braun - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Lorenz Theiler - Substantial contributions to conception and design, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Guaranter of Submission

The corresponding author is the guarantor of submission.

Source of Support

None

Consent Statement

Written informed consent was obtained from the patient for publication of this article.

Data Availability

All relevant data are within the paper and its Supporting Information files.

Conflict of Interest

Authors declare no conflict of interest.

Copyright

© 2020 Carl Conrad et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.