A Rare Case of Bickerstaff Encephalitis in Childhood: A Case Report

Abstract

Background: Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune disease and approximately 74 cases have been reported in the literature, mostly in childhood. Methods: We reported this case report according to the CARE guidelines. Results: A 13-year-old female presented with a 4-day history of persistent fever and hallucinations. She rapidly developed nystagmus associated with blurred vision with ataxic gait. She also developed altered mental status, blepharoptosis, diplopia and extrinsic ocular motility. An EEG showed asymmetric brain electrical activity with slow and spiky abnormalities in the left cerebral hemisphere. Lumbar puncture showed mild pleocytosis with lymphocytic predominance, elevated protein, with normal glucose. Anti-GM1 and anti-GM2 antibodies were positive. She was administered intravenous immunoglobulin therapy due to a suspicion of BBE, showing rapid improvement in mental status. Conclusions: BBE is a diagnosis of exclusion and should be considered especially in pediatric age.