Etiology matters for neuroprognostication: A multimodal electrophysiological investigation in a case of Bickerstaff's brainstem encephalitis

We report the case of a 19-year-old patient with an acute-onset non-traumatic coma. Brain MRI scan was normal, CSF showed mild pleocytosis and moderately elevated protein, and continuous EEG-monitoring was compatible with spindle-coma. Cortical somatosensory evoked potentials (SSEPs) and middle-latency auditory evoked potentials (MLAEPs) were bilaterally absent, and brainstem auditory evoked potentials suggested a brainstem dysfunction. Serum anti-GQ1b and anti-GT1a IgG antibodies positivity suggested Bickerstaff's brainstem encephalitis (BBE). The clinical and functional outcomes were favorable and normal cortical SSEPs/MLAEPs reappeared in a few weeks. Based on this report, in cases of unexplained MRI-negative coma with neurophysiological evidence of brainstem dysfunction, BBE should be eliminated before considering withdrawal of life-sustaining therapy (WLST). (c) 2022 Elsevier Masson SAS. All rights reserved.

Automated summary

We present a case of a 19-year-old patient with acute-onset non-traumatic coma. The MRI was normal, but neurophysiological examinations indicated brainstem dysfunction. The patient was diagnosed with Bickerstaff's brainstem encephalitis (BBE) based on positive serum antibodies. The patient had favorable clinical and functional outcomes after treatment.