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

Joana Coelho, Sara Azevedo, Fátima Furtado, Francisco Abecasis, Sofia Quintas, António Levy


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.



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Jones HR Jr. Guillain-Barré syndrome: perspectives with infants and children. Semin Pediatr Neurol. 2000;7(2):91-102.

Nagashima T, Koga M, Odaka M, Hirat K, Yuki N. Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Arch Neurol. 2007;64(10):1519-23.

Van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis and treatment of Guillain- Barré syndrome. Lancet Neurol. 2008;7(10):939-50.

Herguner MO, Tepe T, Altunbasak S, Baytok V. A rare form of Guillain- Barré syndrome: pharyngeal-cervical-brachial variant. The Turkish J of Pediatrics. 2008;50(1):91-3.

Ropper AH. Unusual clinical variants and signs in Guillain-Barré syndrome. Arch Neurol. 1986;43(11):1150-52.

Asbury AK. New concepts of Guillain-Barré syndrome. J Child Neurol. 2000;15(3):183-91.

MacLennan SC, Fahey MC, Lawson JA. Pharyngeal-cervical-brachial variant Guillain- Barré syndrome in a child. J Child Neurol. 2004;19(8):626-7.

Koga M, Yoshino H, Morimatsu M, Yuki N. Anti-GT1a IgG in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 2002;72(6):767-71.

Murakami N, Tomita Y, Koga M et al. An adolescent with pharygeal-cervical-brachial variant of Guillain-Barré syndrome after cytomegalovirus infection. Brain and Development. 2006;28(4):269-71.

Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2012;11(7):CD002063.

Vajsar J, Fehlings D, Stephens D. Long-term outcome in children with Guillain-Barré syndrome. J Pediatr. 2003;142(3):305-9.



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