Childhood Guillain-Barré Syndrome- Comparing Intravenous Immunoglobulin Treatment with Supportive Care
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Objectives: Guillain-Barré Syndrome (GBS) is an acute ascending flaccid paralysis that is often
preceded by a mild bacterial or viral infection. Management options include supportive care,
physiotherapy, intravenous immunoglobulin (IVIG) and plasmapheresis. Our aim was to compare
IVIG treatment and supportive care to supportive care alone in cases of moderate to severe GBS
in children less than 16 years of age. Methods: Using specific keywords, the Cochrane Library and
PubMed were searched. Eight relevant articles were found and appraised. The studies compared
specific outcome criteria including mortality, days taken to regain independent locomotion, days
taken to improve by one disability grade on Hughes’ Functional Scale, days of hospitalisation and
need for mechanical ventilation. Results: Four articles concluded definite benefits from the use of
IVIG in GBS and two articles concluded that there was no difference in outcome with IVIG. One
study showed statistically significant benefits with IVIG regarding death and need for mechanical
ventilation. No studies showed statistically significant differences regarding recovery times or days
of hospitalisation. Conclusion: The articles concurred with the data on GBS in adults, that timely
use of IVIG reduces mortality and morbidity in a paediatric setting. However, IVIG use made no
difference in the incidence of long-term sequelae of GBS. Sample sizes were small. Larger studies
are needed in order to fully explore the benefits of IVIG with regard to these outcome criteria.
Logistically and ethically, this would be difficult and no randomised controlled trials have been done
to date for this reason. Acute relapse was a new phenomenon, which had not been previously
noted in the pre-IVIG era and warrants further investigation.
References
Guillain-Barré Syndrome and related diseases: Review
of clinical features and antibody specificities. J
Neurosci Res 2005;[epublication ahead of print].
2. Kumar P and Clark M, eds. Clinical medicine. 5th ed.
United Kingdom: Bath Press Ltd., 2002.
3. Healthwise Medical Database. Guillain-Barré
Syndrome. (Accessed at
http://www.caminomedical.org.)
4. A collection of resources on Guillain-Barré, MillerFisher and other related syndromes. (Accessed at
http://www.gbs.org.)
5. Dalakas MC. Intravenous immunoglobulin in the
treatment of autoimmune neuromuscular diseases:
present status and practical therapeutic guidelines.
Muscle Nerve 1999;22(11):1479-97.
6. Misbah SA, Chapel HM. Adverse effects of
intravenous immunoglobulin. Drug Saf 1993;9(4):254-
62.
7. Van Doorn PA. Guillain-Barré Syndrome.
(http://www.orpha.net, Updated Sep 2004.)
8. McLean S, Oon SF, Conran S, et al. Childhood
Guillain-Barré Syndrome: Comparing intravenous
immunoglobulin treatment with supportive care.
Presentation by S McLean, June 2003, Adelaide and
Meath Hospital, Incorporating the National Children’s
Hospital, Tallaght, Dublin, Ireland.
9. Sharrack B, Hughes RAC, Soudain S, Dunn G. The
psychometric properties of clinical rating scales used in
multiple sclerosis. Brain 1999;122(1):141-59.
10. Koul R, Chacko A, Ahmed R, et al. Ten year
prospective study (Clinical Spectrum) of childhood
Guillain-Barré Syndrome in the Arabian Peninsula:
Comparison of outcome in patients in pre- and postintravenous immunoglobulin eras. J Child Neurol
2003;18(11):767-71.
11. Vajsar J, Fehlings D, Stephens D. Long term
outcome in children with Guillain-Barré Syndrome.
J Paediatr 2003;142(3):305-9.
12. Shanbag P, Amirtharaj C, Pathak A. Intravenous
immunoglobulins in severe Guillain-Barré Syndrome in
childhood. Indian J Pediatr 2003;70(7):541-3.
13. Zafeiriou DI, Kontopoulos EE, Katzos GS,
Gombakis NP, Kanakoudi FG. Single dose
immunoglobulin therapy for childhood Guillain-Barré
Syndrome. Brain Dev 1997;19:323-5.
14. Graf WD, Katz JS, Eder DN, et al. Outcome in
severe paediatric Guillain-Barre syndrome after
immunotherapy or supportive care. Neurology 1999;
52:1494.
15. Yata J, Nihei K, Ohya T, et al. Study group for
pediatric Guillain-Barré Syndrome. High dose
immunoglobulin therapy for Guillain-Barré Syndrome
in Japanese children. Pediatr Int 2003;45(5):543-9.
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