A Probable Case of Recurrent Bickerstaff Brainstem Encephalitis With Fulminant Course in a Pediatric Patient

Bickerstaff brainstem encephalitis is a rare, often postinfectious, syndrome characterized by the clinical triad of ophthalmoplegia, ataxia, and altered consciousness. Here, we present a probable case of recurrent, pediatric Bickerstaff encephalitis, whereby the patient acutely developed loss of consciousness and eventually brain death despite optimal management.

A 3-year-old male patient initially presented to the emergency department with progressive ataxia, following history of upper respiratory tract infection. He deteriorated within 12 hours of hospitalization, requiring cardiopulmonary resuscitation. The patient had decreased consciousness thereafter, showing minimal signs of brain activity. He was then deemed to be suffering a second episode of Bickerstaff encephalitis, the first being a year prior, and intravenous immunoglobulins were administered immediately. Magnetic resonance imaging of the brain on day 2 of admission showed signs of diffuse, bilateral encephalitis in the brainstem, thalami, and basal ganglia. Brain death was confirmed on day 11 of admission, following 2 brain death examinations.

Although Bickerstaff brainstem encephalitis tends to involve the brainstem, outcomes of brain death have been rarely reported, even more so in the pediatric age group. This case report sheds light on, possibly, the first reported fatality of Bickerstaff encephalitis among children.