Neuropediatrics 2008; 39(6): 354-356
DOI: 10.1055/s-0029-1202768
Short Communication

© 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

R. T. Rousseff 1 , A. J. Khuraibet 1 , D. Neubauer 2
  • 1Department of Clinical Neurophysiology, Ibn-Sina Hospital, Kuwait, Kuwait
  • 2Department of Pediatric Neurology, University Children's Hospital, University of Ljubljana, Ljubljana, Slovenia
Further Information

Publication History

received 31.10.2008

accepted 09.02.2009

Publication Date:
30 June 2009 (online)

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

References

  • 1 Buompadre MC, Gáñez LA, Miranda M. et al . Variantes inusuales del síndrome de Guillain-Barré en la infancia.  Rev Neurol. 2006;  42 85-90
  • 2 Hergüner MÖ, Tepe T, Altunbaşak S. et al . A rare form of Guillain-Barré syndrome: pharyngeal-cervical-brachial variant.  Turk J Pediatr. 2008;  50 91-93
  • 3 Koga M, Yuki N, Hirata K. Antiganglioside antibody in patients with Guillain-Barré syndrome who show bulbar palsy as an initial symptom.  J Neurol Neurosurg Psychiatry. 1999;  66 513-516
  • 4 Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study.  Neuropediatrics. 2007;  38 10-17
  • 5 MacLennan SC, Fahey MC, Lawson JA. Pharyngeal-cervical-brachial variant Guillain-Barré syndrome in a child.  J Child Neurol. 2004;  19 626-627
  • 6 Miura Y, Susuki K, Yuki N. et al . Guillain-Barré syndrome presenting pharyngeal-cervical-brachial weakness in the recovery phase.  Eur Neurol. 2002;  48 53-54
  • 7 Mogale KD, Antony JH, Ryan MM. The pharyngeal-cervical-brachial form of Guillain-Barré syndrome in childhood.  Pediatr Neurol. 2005;  33 285-288
  • 8 Nagashima T, Koga M, Odaka M. et al . Clinical correlates of serum anti-GT1a IgG antibodies.  J Neurol Sci. 2004;  219 139-145
  • 9 Nagashima T, Koga M, Odaka M. et al . Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome.  Arch Neurol. 2007;  64 1519-1523
  • 10 Ropper AH. Unusual clinical variants and signs in Guillain-Barré syndrome.  Arch Neurol. 1986;  43 1150-1152
  • 11 Schessl J, Funakoshi K, Susuki K. et al . Anti-GT1a IgG antibodies in a child with severe Guillain-Barre syndrome.  Pediatr Neurol. 2006;  35 277-279
  • 12 Tankisi H, Pugdahl K, Fuglsang-Frederiksen A. et al . Pathophysiology inferred from electrodiagnostic nerve tests and classification of neuropathies. Suggested guidelines.  Clin Neurophysiol. 2005;  116 1571-1580

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

    >