Bickerstaff's brainstem encephalitis (BBE) in childhood: rapid resolution after intravenous immunoglobulins treatment

P Pavone1, A Le Pira, F Greco

  • 1Unit of Pediatrics and Pediatric Emergency, University Hospital, "Vittorio Emanuele Polyclinic", Catania, Italy. ppavone@unict.it.

Insights

Bickerstaff

Area of Science:

  • Neurology
  • Pediatrics
  • Immunology

Background:

  • Bickerstaff's brainstem encephalitis (BBE) is a rare neurological disorder.
  • It often follows an upper respiratory tract infection and is underdiagnosed in children.

Observation:

  • Three pediatric patients presented with acute onset of lower limb weakness after initial recovery from infection.
  • Symptoms progressed to cranial nerve involvement, consciousness impairment, and coma in one case.

Findings:

  • Intravenous immunoglobulin (IVIG) treatment led to rapid and progressive clinical improvement in all three children.
  • BBE shares neuro-immune pathogenesis with Fisher syndrome and Guillain-Barré syndrome, suggesting a spectrum of disease.

Implications:

  • Early diagnosis and prompt IVIG treatment are crucial for favorable outcomes in pediatric BBE.
  • Understanding BBE as part of a broader neuro-immune spectrum can improve diagnostic and therapeutic approaches.

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