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Journal of Neurology
Published in: Journal of Neurology 1/2018

01-01-2018 | Original Communication

Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series

Authors: Jonathan Douglas Santoro, Daniel V. Lazzareschi, Cynthia Jane Campen, Keith P. Van Haren

Published in: Journal of Neurology | Issue 1/2018

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Abstract

Objective

To characterize the phenotype of pediatric Bickerstaff’s brainstem encephalitis (BBE) and evaluate prognostic features in the clinical course, diagnostic studies, and treatment exposures.

Methods

We systematically reviewed PubMed, Web of Science, and SCOPUS databases as well as medical records at the Lucile Packard Children’s Hospital to identify cases of pediatric BBE. Inclusion required all of the following criteria: age ≤ 20 years, presence of somnolence or alterations in mental status at the time of presentation or developed within 7 days of presentation, ataxia, and ophthalmoplegia.

Results

We reviewed 682 manuscripts, identifying a total of 47 pediatric BBE cases. We also describe five previously unreported cases. The phenotype of these pediatric patients was similar to previously published literature. Sixty-eight percent of patients demonstrated positive anti-GQ1b antibody titers, yet the presence of these antibodies was not associated with longer times to recovery. Patients with neuroimaging abnormalities featured a longer median time to recovery, but this was not statistically significant (p = 0.124). Overall, patients treated with any form of immunotherapy (intravenous immunoglobulin, steroids, or plasmapheresis) demonstrated shorter median time to resolution of symptoms compared to supportive therapy, although this trend was not statistically significant (p = 0.277). Post-hoc t tests revealed a trend towards use of immunotherapy against supportive care alone (p = 0.174).

Conclusion

Our study identified clinical, radiologic, and treatment features that may hold prognostic value for children with BBE. The role of immunotherapy remains under investigation but may prove of utility with further, randomized controlled studies in this rare disease.
Appendix
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Metadata
Title
Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series
Authors
Jonathan Douglas Santoro
Daniel V. Lazzareschi
Cynthia Jane Campen
Keith P. Van Haren
Publication date
01-01-2018
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 1/2018
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-017-8684-8

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