Electroneuromyography and Neuromuscular Diseases Unit, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, Bron, France.
Electroneuromyography and Neuromuscular Diseases Unit, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, Bron, France; Institut NeuroMyoGène, Université Lyon1 - CNRS UMR 5310 - INSERM U1217, Lyon, France.
Neurophysiol Clin. 2022 Oct;52(5):398-403. doi: 10.1016/j.neucli.2022.09.004. Epub 2022 Oct 7.
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).
我们报告了一例 19 岁患者突发非外伤性昏迷。脑 MRI 扫描正常，CSF 显示轻度白细胞增多和中度升高的蛋白，连续 EEG 监测与纺锤昏迷相符。皮质体感诱发电位（SSEP）和中潜伏期听觉诱发电位（MLAEP）双侧缺失，脑干听觉诱发电位提示脑干功能障碍。血清抗 GQ1b 和抗 GT1a IgG 抗体阳性提示 Bickerstaff 脑干脑炎（BBE）。临床和功能结局良好，数周后正常皮质 SSEP/MLAEP 再次出现。基于此报告，对于原因不明的 MRI 阴性昏迷且神经生理学证据提示脑干功能障碍的病例，在考虑停止生命支持治疗（WLST）之前，应排除 BBE。
