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Bickerstaff's brainstem encephalitis in childhood: a literature overview

S Marino1, L Marino, F Greco

  • 1Pediatric and Pediatric Emergency Department, University Hospital "Policlinico-San Marco, Catania, Italy. ppavone@unict.it.

European Review for Medical and Pharmacological Sciences
|December 30, 2020
PubMed

Insights

Bickerstaff brainstem encephalitis in children is uncommon. Immunotherapy speeds symptom recovery, but neuroimaging changes may prolong healing time, impacting prognosis.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Infectious Diseases

Background:

  • Bickerstaff brainstem encephalitis (BBE) is a rare neurological disorder.
  • Understanding pediatric BBE is crucial for timely diagnosis and management.

Purpose of the Study:

  • To review the clinical presentation, diagnosis, and treatment of pediatric Bickerstaff brainstem encephalitis.
  • To identify factors influencing recovery and prognosis in children with BBE.

Main Methods:

  • Systematic review of pediatric BBE cases (age ≤ 18) from major databases (PubMed, Cochrane, Scopus).
  • Inclusion based on age and clinical characteristics of BBE.
  • Analysis of 236 pediatric cases from 27 articles (1990-2020).

Main Results:

  • Pediatric BBE phenotype aligns with existing literature.
  • Anti-GQ1b antibody positivity was observed in nearly half of cases but did not correlate with healing time.
  • Neuroimaging abnormalities were associated with longer recovery periods.
  • Immunotherapy (IVIg, steroids, plasmapheresis) led to faster symptom resolution compared to supportive care.

Conclusions:

  • Bickerstaff brainstem encephalitis is rare, with prognosis influenced by clinical presentation, comorbidities, investigations, and treatment timing.
  • Early immunotherapy appears beneficial for symptom resolution in pediatric BBE.
  • Neuroimaging findings are important prognostic indicators for recovery duration.
Abstract

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