Abstract
AIMS: To report a case of pharyngeal-cervical-brachial variant of Guillain-Barre 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-Barre 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.
Highlights
Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in healthy children
Conclusions: 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
pharyngealcervical-brachial variant (PCB) variant of GBS was first described in 1986 by Ropper.[5]. The diagnosis of this variant was made based on specific clinical features, such as an acute progression of oropharyngeal, neck, shoulder, upper limbs and diaphragm weakness, bulbar palsy and preserved muscle strength in the lower limbs.[2,5]
Summary
Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children. Joana Coelho[1], Sara Azevedo[1], Fátima Furtado[2], Francisco Abecasis[3], Sofia Quintas[4], António Levy[4]
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