Bickerstaff encephalitis is a rare immune-mediated condition believed to be one of a number of conditions sharing a similar immunological mechanism, and collectively termed anti-GQ1b IgG antibody syndrome.
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Clinical presentation
Bickerstaff encephalitis is often seen following varicella zoster virus and cytomegalovirus infections 1.
Patients typically present acutely with cerebellar ataxia and external ophthalmoplegia. They may also demonstrate drowsiness, hemisensory disturbance as well as brisk reflexes and extensor plantar response 2.
Radiographic features
MRI
It transiently involves the brainstem and basal ganglia and is characterized on MRI by regions of high T2 signal with little - if any - enhancement. Typically these regions also demonstrate some minor restricted diffusion 1.
Treatment and prognosis
Although symptoms are severe, the condition is usually self-limiting with a good prognosis 2.
History and etymology
Bickerstaff encephalitis was first described by Edwin Robert Bickerstaff (1920-2007), a renowned British neurologist 3.