A case of fulminant Guillain-Barré syndrome presenting as brain death

Abstract

Brain death as a presentation of fulminant Guillain Barré syndrome is rare with only twenty cases reported in the literature. This condition should be treated immediately despite high mortality as survivors recover with early treatment and supportive care. A 15-year-old boy with a history of recovery from Guillain Barré syndrome nine months ago, presented with coma, respiratory paralysis, fixed and dilated pupils. CT angiogram of the brain confirmed adequate cerebral perfusion, nerve conduction studies detected sensory motor axonal polyneuropathy and stool biofire was positive for Campylobacter jejuni. His prolonged hospital stay on life support systems was complicated with dysautonomia, shock, respiratory failure and ventilator associated pneumonia. Prompt treatment with endotracheal intubation, ventilation, intravenous immunoglobulins, supportive therapy and physiotherapy enabled the patient to recover and gradually regain motor power. At day 90, he was on tracheostomy and standing with support. Reviewing the literature, there are sporadic case reports of fulminant Guillain Barre syndrome recovering with rigorous and prolonged treatment. Clinicians should be vigilant about this variant as timely treatment could save lives and instill hope in distressed families. Dysautonomia, cranial nerve involvement, ventilator dependency and sepsis contribute to high mortality. This case report is unique as it highlights the trials and tribulations of management of this rare variant of Guillain Barre syndrome with emphasis on the necessity to maintain life sustaining measures and prolonged treatment in affected patients. The clinical course and treatment response was documented daily till the patient was on the road to recovery.