Pharyngeal-Cervical-Brachial Variant Guillain-Barre Syndrome in a Child

Abstract

The pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is uncommon but well recognized in the adult literature. Patients have weakness in a pharyngeal-cervical-brachial distribution with relative lower limb sparing. We describe a 12-year-old boy with predominantly pharyngeal-cervical-brachial weakness and subsequent respiratory failure. Owing to prominent bulbar symptoms, he was initially misdiagnosed as having epiglottitis. This case illustrates that the clinical spectrum of Guillain-Barré syndrome in children includes the pharyngeal-cervical-brachial variant, which is distinct from Miller-Fisher syndrome. Atypical Guillain-Barré syndrome should be considered in the differential diagnosis of a child presenting with bulbar palsy and/or respiratory failure.

Reversible conduction failure in pharyngeal‐cervical‐brachial variant of guillain‐barré syndrome

“…They expand the clinical profile and provide evidence that PCB and other GBS clinical variants form a continuous spectrum 2. In PCB, electrophysiological studies have been variably reported to be consistent with either demyelination or primary axonal degeneration 3–6…”

Variante faringo-cérvico-braquial da síndrome de Guillain-Barré: uma causa rara de disfunção bulbar aguda em crianças

“…CSF examination of our patient was normal, but this is not uncommon in GBS, especially in the early stages. 7 The diagnosis of PCB variant was supported by the clinical course, neurologic examination, neurophysiologic findings and the presence of antiganglioside antibodies in the serum (anti-GT1a, GD1a, GQ1b). Administration of IVIG and plasmapheresis are the main therapeutic options.…”

“…The PCB variant was first described by Ropper et al 5 and has rarely been described in children. 6,7 The authors present a case of a child who was diagnosed with PCB variant of GBS. This case illustrates that this is a rare cause of acute bulbar dysfunction and respiratory failure that needs a timely diagnosis.…”

The “Child in the Barrel Syndrome” – Severe Pharyngeal-cervical-brachial Variant of Guillain-Barre Syndrome in a Toddler

“…Such a presentation is characteristic, but if PCB is not considered, the diagnosis may be delayed. Problems with early diagnosis were underlined by MacLennan et al, whose 12-year-old patient developed acute bulbar weakness with respiratory distress but was initially misdiagnosed with epiglottitis [5] . Recent publications stress the continuity and overlap in the clinical spectrum between PCB and MFS [9] .…”