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DOI: 10.1055/s-0029-1202768
© Georg Thieme Verlag KG Stuttgart · New York
The “Child in the Barrel Syndrome” – Severe Pharyngeal-cervical-brachial Variant of Guillain-Barre Syndrome in a Toddler
Publication History
received 31.10.2008
accepted 09.02.2009
Publication Date:
30 June 2009 (online)
![](https://www.thieme-connect.de/media/neuropediatrics/200806/lookinside/thumbnails/10.1055-s-0029-1202768-1.jpg)
Abstract
One week after a flu-like prodrome, an 18-month-old boy developed acute severe, symmetrical, painless weakness and wasting of the shoulder girdle and upper limbs, drooling, dysphagia, dysarthria, atrophy and fasciculations of the tongue. Milder paresis involved the mimic muscles and the neck extensors. The legs were intact with brisk reflexes. The flail immobile upper limbs produced the appearance that the boy was restrained in a narrow barrel. Electrodiagnostic findings suggested demyelinating motor neuropathy sparing the legs. CSF (45 days after onset) was normal. Initial recovery was observed but 70 days after onset the child suffered severe relapse and died from respiratory arrest. This is another rare case of the pharyngeal-cervical-brachial variant of Guillain-Barre syndrome in infancy with an unusual relapsing course leading to a fatal outcome
Key words
Guillain-Barre syndrome - pharyngeal-cervical-brachial variant - polyneuropathy - nerve conduction studies - EMG
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Correspondence
R. T. Rousseff
Department of Clinical Neurophysiology
Ibn-Sina Hospital
PoB 25427
13115 Kuwait
Phone: +965/663 799 70
Fax: +965/248 492 26
Email: rossentrousseff@yahoo.co.uk