Q: What is Bickerstaff's brainstem encephalitis (BBE)?

Answer: Bickerstaff's brainstem encephalitis (BBE), is a variant of Guillain–Barré syndrome. It is characterized by acute onset of

• ophthalmoplegia,


• ataxia,


• disturbance of consciousness,


• hyperreflexia or Babinski's sign

The course of the disease can be monophasic or remitting-relapsing but despite severe initial presentation usually it has a good prognosis. MRI plays a critical role in the diagnosis of BBE, characterized by large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla.