Paeds Vivas · neurology-neurodisability-and-neuromuscular
Guillain-Barré syndrome and acute flaccid paralysis: Viva
Branching clinical structured oral on paediatric Guillain-Barré syndrome and acute flaccid paralysis covering the Brighton diagnostic criteria, the albuminocytological dissociation, the subtypes AIDP, AMAN, AMSAN, and the Miller Fisher syndrome with their antibody associations, the molecular mimicry mechanism, the first-line intravenous immunoglobulin dose of 2 g per kg over two to five days and plasma exchange as alternative, the lack of benefit of corticosteroids, the respiratory monitoring with forced vital capacity thresholds, the acute flaccid paralysis differential including acute flaccid myelitis, and the Erasmus prognostic scores.
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Branch 1: Making the diagnosis
A strong candidate recognises this as Guillain-Barré syndrome and applies the Brighton criteria. The diagnosis turns on the tempo, the symmetry, and the reflex pattern rather than on a single test. The child has a rapid onset of progressive, symmetrical, ascending flaccid weakness over days, with early areflexia and no sensory level, which is the classic clinical picture. The antecedent diarrhoeal illness points to Campylobacter jejuni and supports the diagnosis. A lumbar puncture for albuminocytological dissociation and neurophysiology to distinguish the demyelinating from the axonal subtypes are sent, but a normal early cerebrospinal fluid does not exclude the diagnosis, and treatment is started on the clinical picture. [2]
References7ShowHide
- [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
- [8]van Koningsveld R, Steyerberg EW, Hughes RAC, et al A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet Neurol, 2007.PMID 17537676
- [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