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Bickerstaff's Brainstem Encephalitis

Synonyms

Bickerstaff's encephalitis; Bickerstaff's syndrome

Definition

Bickerstaff's brainstem encephalitis (BBE) is an uncommon central nervous system (CNS) disease characterized by acute external opthtalmoplegia, cerebellar ataxia with disturbances of consciousness and pyramidal signs. Its etiology remains unknown but it is postulated that BBE has an autoimmune origin because of an antecedent illness and frequently the presence of anti-GQ1b antibodies. BBE is usually a monophasic disease with a generally good outcome.

Characteristics

Clinical Presentation

The first cases of descending brainstem syndrome were described in 1951 by Bickerstaff and Cloake under the title "Mesencephalitis and rhombencephalitis" [1]. In 1957 Bickerstaff defined the disease as "brain-stem encephalitis" [2]. The condition has been called Bickerstaff's brainstem encephalitis since 1978 when Bickerstaff wrote a review for the Handbook of Clinical Neurology under