Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children

• Autor(a) principal: Coelho, Joana
• Data de Publicação: 2014
• Outros Autores: Azevedo, Sara, Furtado, Fátima, Abecasis, Francisco, Quintas, Sofia, Levy, António
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Scientia Medica (Porto Alegre. Online)
• Texto Completo: https://revistaseletronicas.pucrs.br/scientiamedica/article/view/18310
• topic: BULBAR PALSY; PROGRESSIVE; CHILDHOOD; GUILLAIN-BARRE SYNDROME; PARALISIA BULBAR PROGRESSIVA; CRIANÇA; SÍNDROME DE GUILLAIN- BARRÉ

AIMS: To report a case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, which is characterized by rapidly progressive bulbar palsy with upper limb, neck and oropharyngeal involvement. It is a rare disorder in childhood and most cases have been described in adolescents.CASE DESCRIPTION: A seven year-old-boy presented with dysarthria, hoarseness, dysphagia, facial diplegia and bilateral progressive upper limb weakness. These symptoms started two weeks after a gastrointestinal infection. Nerve conduction studies were compatible with an acute demyelinating polyneuropathy in the upper extremities. Anti-ganglioside antibodies in the serum (anti-GT1a, GD1a, GQ1b) were positive and Campylobacter jejuni was isolated from stools. The patient was treated with intravenous immunoglobulin and needed ventilatory support during the first 12 days of admission. He was discharged at day 15 showing improvement of his neurological deficits. He fully recovered after eleven months of follow-up.CONCLUSIONS: Although pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is uncommon in children, it should be considered in a child with acute bulbar dysfunction because a timely diagnosis allows the early institution of therapeutic measures that can be lifesaving.