Clinical and Laboratory Features, Treatment and Prognosis in Children with Guillian-Barre Syndrome

A retrospective study of 42 cases of acute flaccid paralysis (AFP) in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014), was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS). GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (77%), 5 with acute motor axonal neuropathy (AMAN) (16%) and 2 with аcute motor-sensory axonal neuropathy (AMSAN) in a total of cases (7%). Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51%) compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg) and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per course. The treatment has shown to be effective for the majority of patients, but three children was resistant to the intravenous immunoglobulin. An important feature of pediatric GBS is a nonthreatening prognosis at the point of discharge, with the length of hospitalization numbering in with a median of 28 [20,5; 38] days.

1. Olivé J.M., Castillo C., Castro R.G., de Quadros C.A. Epidemiologic Study of Guillain-Barré Syndrome in Children < 15 Years of Age in Latin America //J Infect Dis. 1997 Feb;175 Suppl 1:S160—4.

2. Marx A., Glass J.D., Sutter R.W. Differential diagnosis of acute flaccid paralysis and its role in poliomyelitis surveillance // Epidemiol Rev 2000;22:298–316.

3. Пирадов М.А., Супонева Н.А. Синдром Гийена-Барре: диагностика и лечение. — М.: МЕДпресс, 2011. — 208 с. Piradov M.A., Suponeva N.A. [Gullian-Barre syndrome: diagnostic and treatment]. — M., 2011. — 208 s. (In Russ.)

4. Hughes RA, Cornblath DR. Guillain-Barré syndrome // Lancet. 2005. Nov 5; 366(9497):1653—66.

5. Супонева Н.А., Мочалова Е.Г., Гришина Д.А., Пирадов М.А. Особенности течения СГБ в России: анализ 186 случаев // Нервно-мышечные болезни, 2014;1:37 — 46. Suponeva N.A., Mochalova E.G., Grishina D.A., Piradov M.A. [Some aspects of GBS in Russia: analysis of 186 cases]. — 2014;1:37 — 46. (In Russ.)

6. Fokke C, Van den berg B, Drenthen J et-al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria // Brain. 2014;137 (Pt): 33—43.

7. Супонева Н.А., Пирадов М.А.,Никитин С.С. и др. Патогенетическая и прогностическая роль аутоантител к гликозидам периферических нервов при синдроме Гийена-Барре // Анн неврол 2013; 7(1):4—11.

8. Suponeva N.A., Piradov M.A., Nikitin S.S. et al. [Pathogenic and prognostic role of autoantibody to peripheral nerves’s glycosides]. // Ann. Nevr. 2013; 7(1):4-11.

9. Van den Berg B, Walgaatd C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10:469–482.

10. Hughes R.A.C., Swan A.V., Raphaе? J.S. et al. Immunotherapy for Gullain-Barre syndrome: a systematic review // Brain 2007; 130:2245—57.

11. Tasdemir H.A., Dilber C., Kanber Y., Uysal S. Intravenous immunoglobulin for Guillain-Barré syndrome: how effective? // J Child Neurol. 2006 Nov; 21(11):972—4.

12. Raphael J.C., Chevret S., Hughes R.A.C., Annane D. Plasma exchange for Guillain Barré syndrome // Cochrane Database of Systematic Reviews 2009, Issue 2: CD001798.

13. Hadden R.D.M., Cornblath D.R., Hughes R.A.C., Zielasek J., Hartung H.P., Toyka K. et al. Electrophysiological classification of Guillain-Barré Syndrome: clinical associations and outcome // Ann Neurol, 1998; 44:780—788.

14. Hughes R.A.C., Cornblath D.R. Guillain-Barré syndrome // Lancet Neurol, 2005; 366:1653—1666.

15. Van Doorn P.A., Kuitwaard K., Walgaard C., van Koningsveld R., Ruts L., Jacobs B.C. IVIG Treatment and Prognosis in Guillain–Barré Syndrome // Journal of Clinical Immunology. 010; 30 (Suppl 1): 74—78.

16. Постановление Главного государственного санитарного врача РФ от 13.03.2013 N 11 «О Плане действий по поддержанию свободного от полиомиелита статуса Российской Федерации на 2013—2015 гг.» [Postanovlenie glavnogo sanitarnogo vracha RF]. 13.03.2013 N 11. (In Russ.)

17. Asbury A.K., Cornblath D.R. Assessment of current diagnostic criteria for Guillain-Barre syndrome // Ann. Neurol. 1990. V. 27. S. 21—24.

18. Biou D., Benoist J.F., Nguyen-Thi C., Huong X., Morel P., Marchand M. Cerebrospinal fluid protein concentrations in children: age-related values in patients without disorders of the central nervous system // Clin Chem. 2000 Mar; 46(3):399-403.

19. Wong M., Schlaggar B.L., Buller R.S., Storch G.A., Landt M. Cerebrospinal fluid protein concentration in pediatric patients: defining clinically relevant reference values // Arch Pediatr Adolesc Med. 2000 Aug; 154(8):827-31.

20. Elovaara I., Apostolski S., van Doorn P., Gilhus N.E., Hietaharju A., Honkaniemi J., van Schaik I.N., Scolding N., Soelberg Sorensen P., Udd B. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases // Eur J Neurol. 2008 Sep;15(9):893-908. Erratum in: Eur J Neurol. 2009 Apr;16(4):547.

21. Korinthenberg R., Monting J.S. Natural history and treatment effects in Guillain-Barré syndrome: a multi centre study // Arch Dis Child 1996; 74:281—287.

22. Ma Y.M., Liu T.K., Wong V. Guillain-Barre syndrome in southern Chinese children: 32 year experience in Hong Kong // Pediatr Int (2010) 52:13—19.

23. Hicks C.W., Kay B., Worley S.E., Moodley M. A clinical picture of Guillain-Barré syndrome in children in the United States // J Child Neurol. 2010 Dec; 25(12):1504—10.

24. Ryan M.M. Guillain-Barre syndrome in childhood // J Paediatr. Child Health. 2005; 41:237—241.

25. Lee J.H., Sung I.Y., Rew I.S. Clinical presentation and prognosis of childhood Guillain-Barre syndrome // J Paediatr Child Health. 2008; 44:449—454.

26. Plasma Exchange/Sandoglobulin Guillain–Barré Syndrome Trial Group Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain–Barré syndrome // Lancet. 1997; 349:225—230.