Bickerstaff Brainstem Encephalitis with a Decorticate-like Posture, Involuntary Movements, Impaired Visual Acuity,...

The acute immune-mediated disorder, Bickerstaff brainstem encephalitis (BBE), is characterized by consciousness disturbance, external ophthalmoplegia, and ataxia. BBE is usually readily diagnosed and patients recover well, but rare features may sometimes obscure the diagnosis, and some patients develop prolonged weakness. We herein describe a Japanese patient with BBE who presented with uncharacteristic features of a decorticate-like posture, involuntary movements, and impaired visual acuity, entered a coma requiring intubation, and later required orthosis-assisted ambulation for 10 months. Despite these uncharacteristic features, the typical triad and other BBE characteristic features can be valuable for making an early diagnosis. BBE with a coma or intubation may therefore occasionally lead to prolonged weakness.