Guillain-Barré syndrome and acute flaccid paralysis: Case

Clinical case of a 6-year-old boy with Guillain-Barré syndrome after Campylobacter jejuni gastroenteritis, covering the ascending areflexic presentation, the falling forced vital capacity and bulbar weakness triggering intensive care, the first-line intravenous immunoglobulin 2 g per kg over two to five days, the exclusion of acute flaccid myelitis, the intensive care course with ventilation and autonomic monitoring, and the paediatric recovery and rehabilitation.

paediatric emergency long case3 min readVerification in progress

Prompt

A previously well 6-year-old boy presents with progressive leg weakness over 4 days. Two weeks earlier he had a diarrhoeal illness. He is now unable to walk. Examination shows symmetrical flaccid weakness of the legs, absent knee and ankle jerks, no sensory level, and normal sphincter tone. His weight is 22 kilograms. His bedside forced vital capacity is 18 mL per kilogram, down from 25 mL per kilogram six hours ago. He has a weak cough with pooled secretions.

Summary and immediate impression

This boy presents a textbook case of Guillain-Barré syndrome. The tempo is subacute over days, with a Campylobacter-like diarrhoeal illness two weeks earlier, progressive symmetrical ascending flaccid weakness, and early areflexia without a sensory level. The falling forced vital capacity and the bulbar weakness are the immediate threats, and they drive the decision to escalate to intensive care. The diagnosis is made on the clinical picture, and the disease-modifying treatment is intravenous immunoglobulin. [1]

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References6Show ledger

[1]Willison HJ, Jacobs BC, van Doorn PA Guillain-Barré syndrome. Lancet, 2016.PMID 26948435
[2]Fokke C, van den Berg B, Drenthen J, et al Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain, 2014.PMID 24163275
[4]van der Meché FG, Schmitz PI A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. N Engl J Med, 1992.PMID 1552913
[7]Hughes RAC, Swan AV, Raphaël JC, et al Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain, 2007.PMID 17337484
[10]Korinthenberg R, Trollmann R, Felderhoff-Müser U, et al Diagnosis and treatment of Guillain-Barré Syndrome in childhood and adolescence: An evidence- and consensus-based guideline. Eur J Paediatr Neurol, 2020.PMID 31941581
[12]Hu MH, Chen CM, Lin KL, et al Risk factors of respiratory failure in children with Guillain-Barré syndrome. Pediatr Neonatol, 2012.PMID 23084721