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Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune neurological disease characterized by a triad of acute ophthalmoplegia, ataxia, and altered consciousness, with positive serum IgG anti-GQ1b and negative anti–N-methyl-d-aspartate (NMDA) receptor antibodies.
This video shows an unusual manifestation of the disorder in a previously healthy 20-year-old woman.
On day 1, the patient presented with bilateral ptosis, severe external ophthalmoplegia in all extraocular eye muscles, and facial grimacing and sniffing motions.
By day 5, she developed slow and restless movements in her lower limbs and rapid neck twisting.
On day 7, abnormal movements appeared in her upper limbs, and her consciousness disturbance and ophthalmoplegia improved.
The abnormal movements are unusual for BBE and are more characteristic of anti-NMDA encephalitis. The patient returned to normal after treatment with intravenous immunoglobulin and methylprednisolone.
Click the Related Article link for full case details and discussion.
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