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Alain Rougé,1,2 Jérémie Lemarié,1,2 Sébastien Gibot,1,2 Pierre Edouard Bollaert1,2 1Medical Intensive Care Unit, Hôpital Central, University Hospital of Nancy, Nancy, France; 2INSERM UMRS-1116, Faculty of Medicine, University of Lorraine, Nancy, France Abstract: A 47-year-old man was admitted to the intensive care unit a few hours after ­presenting to emergency department with acute diplopia and dysphonia. Swallowing disorders and respiratory muscular weakness quickly required invasive ventilation. On day 3, the patient was in a “brain-death”-like state with deep coma and absent brainstem reflexes. Electroencephalogram ruled out brain death diagnosis as a paradoxical sleep trace was recorded. Cerebrospinal fluid analysis, electrophysiologic studies, and a recent history of diarrhea led to the diagnosis of Campylobacter jejuni-related fulminant Guillain-Barré syndrome (GBS) mimicking brain death. The outcome was favorable after long Intensive Care Unit and inpatient rehabilitation stays, despite persistent disability at 9 years follow-up. This case and the associated literature review of 34 previously reported fulminant GBS patients emphasize the importance of electrophysiological investigations during clinical brain-death states with no definite cause. Fulminant GBS has a worse outcome than “standard” GBS with higher rates of severe disability (about 50%). Long-term physiotherapy and specific rehabilitation programs appear essential to improve recovery. Keywords: fulminant Guillain-Barré syndrome, brain death, electroencephalogram, C. jejuni, long-term follow