Disease. Bickerstaff brainstem encephalitis (BBE) is a rare, autoimmune disease of the peripheral and central nervous system (i.e., brainstem). BBE is considered a variant of other immune-mediated polyneuropathies, such as Guillain Barré syndrome (GBS) and Miller Fisher syndrome (MFS).

What causes Bickerstaff encephalitis?

BBE has been reported to occur following infection with several agents including cytomegalovirus, Campylobacter jejuni, and Mycoplasma pneumonie. Although the exact pathological mechanism is not fully understood, BBE is associated with the presence of the antiganglioside antibody, anti-GQ1b.

What is Bickerstaff brainstem encephalitis?

Bickerstaff's brainstem encephalitis (BBE) is a rare condition that must be suspected in patients presenting with ataxia, ophthalmoplegia and central nervous system involvement, which may take the form of pseudobulbar affect, decreased consciousness and/or pyramidal signs.