Reply: Bickerstaff Brainstem Encephalitis With Exceptionally Fast Recovery

We thank Dr. Wu for his interest in our article1 and his thoughtful and detailed comments. Dr. Wu suggests that a small ischemic brainstem stroke was the likely explanation for our patient's presentation. We detail below why we believe that this patient had Bickerstaff brainstem encephalitis (BBE) and not an ischemic brainstem event. Our patient was a young woman without any known ischemic risk factors, making ischemic event a cause of her presentation very unlikely. While MRI-negative brainstem strokes can occur, they are not common, and our patient had high-resolution imaging of the brainstem with diffusion-weighted sequencing performed within 3–4 days after the onset of her symptoms. This study did not demonstrate any restricted diffusion in the brainstem, making an ischemic event an explanation for her presentation very improbable. In addition, she experienced an antecedent viral illness, sleepiness, and hyperreflexia, features that are highly suggestive of BBE. A small, MRI-negative lesion in the right paramedian pontine tegmentum, as suggested by Dr. Wu, would not produce sleepiness or hyperreflexia. Most importantly, the patient had high titers of anti-GQ1b antibodies, which are strongly associated with BBE, and fulfilled all criteria for clinically definite BBE: presence of typical clinical triad of external ophthalmoplegia, ataxia, and impaired level of consciousness. She also had positive anti-GQ1b antibody titers. High titers of this antibody are highly specific for an antiganglioside syndrome.2,3 Bickerstaff,4 in his original description of 8 patients with brainstem encephalitis, which was subsequently named BBB, described one individual whose symptoms resolved within 1 week of onset, similar to the patient we described. Given the young age of our patient and absence of any known microvascular risk factors, along with her history of an antecedent infection and fulfillment of the criteria for clinically definite BBE coupled with anti-GQ1b antibody titers, the diagnosis was certainly BBE rather than a small, MRI-negative ischemic brainstem stroke.

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