Bickerstaff Brainstem Encephalitis

Bickerstaff brainstem encephalitis is an autoimmune disorder associated with anti-GQ1b antibodies, characterized by acute onset ophthalmoplegia, ataxia, and altered consciousness. Pathophysiology involves immune-mediated demyelination of the brainstem and peripheral nerves. Patients may also exhibit hyperreflexia and long-tract signs. Diagnosis is clinical, supported by cerebrospinal fluid analysis showing elevated protein and MRI demonstrating brainstem hyperintensities. Management involves immunotherapy with corticosteroids, intravenous immunoglobulin (IVIG), or plasma exchange; prognosis is generally favorable with most patients achieving substantial recovery over weeks to months.