Pediatric Guillain Barre syndrome: clinical, electrophysiological and prognostic features

Öz

Objective: In this study, it was aimed to investigate the clinical, electrophysiological, laboratory and radiological findings and outcomes of patients who were followed up with the diagnosis of Guillain Barre Syndrome. Materials and Methods: Twenty-nine patients diagnosed with Guillain Barre Syndrome and followed for at least one year, between 2011 and 2019 were enrolled. The clinical, laboratory and electrophysiological findings, treatment and follow-up results were analyzed retrospectively. Hughes scale was used for the functional evaluation of the patients. Results: Eighteen of the patients were male (male/female ratio: 1.64). The age range was 1.4-16.4 (median 9.1) years. Preceding infection was found in 21 patients (72.4%). At the time of admission, 26 patients’ (89.7%) deep tendon reflexes were not elicited or decreased. According to their electrophysiological characteristics, eight patients were demyelinating, eleven patients were acute motor axonal, four patients were acute sensorimotor axonal and six patients were Miller Fisher syndrome. All patients received intravenous immunoglobulin therapy and one patient needed plasmapheresis as well. Eight patients recovered with sequelae during the follow-up, and two patients had recurrence. Conclusion: Axonal forms, which are reported more frequently in developing countries, were also higher in our patient group. It was concluded that the presence of deep tendon reflexes as normal or increased is not sufficient to exclude the diagnosis of Guillain Barre Syndrome, and especially axonal forms can be considered in patients with acute flask weakness. Although functional improvement is common in children, it should be known that chronic complaints and recurrences may occur in the long term.

Anahtar Kelimeler

• abnormal deep tendon reflex,
• child
• Guillain-Barré Syndrome
• prognosis

Pediatrik Guillain Barre Sendromu: Klinik, Elektrofizyolojik ve Prognostik Özellikler

Öz

Amaç: Bu çalışmada Guillain Barre Sendromu tanısı ile izlenen hastaların klinik, elektrofizyolojik, laboratuvar ve radyolojik bulgularının ve izlem sonuçlarının araştırılması amaçlanmıştır. Gereç ve Yöntem: 2011-2019 yılları arasında kliniğimizde Guillain Barre Sendromu tanısı almış ve en az bir yıl süre ile izlenmiş 29 hasta değerlendirildi. Hastaların demografik, klinik, laboratuvar, radyolojik ve elektrofizyolojik bulguları, tedavi ve izlem sonuçları retrospektif olarak incelendi. Hastaların fonksiyonel değerlendirmesinde Hughes skalası kullanıldı. Bulgular: Hastaların 18’i erkekti (erkek/kadın oranı: 1,64). Yaş aralığı 1,4-16,4 (ortanca 9,1) yıldı. Hastaların 21’inde (%72,4) enfeksiyon öyküsü vardı. Başvuru sırasında 26 hastanın (%89,7) derin tendon refleksleri alınamıyor veya azalmıştı. Elektrofizyolojik özelliklerine göre sekiz hasta demiyelinizan, on bir hasta akut motor aksonal, dört hasta akut sensorimotor aksonal, altı hasta Miller Fisher sendromu idi. Tüm hastalar intravenöz immünglobulin tedavisi almış, bir hastada ek olarak plazmaferez uygulanmıştı. İzlemde sekiz hastada sekelli iyileşme görülmüş, iki hastada ise hastalık tekrarlamıştı. Sonuç: Gelişmekte olan ülkelerde daha sık olarak bildirilen aksonal formlar bizim hasta grubumuzda da daha fazlaydı. Derin tendon reflekslerinin normal ya da artmış olarak bulunmasının akut flask güçsüzlük ile gelen hastalarda Guillain Barre Sendromu tanısının dışlanması için yeterli olmadığı, özellikle aksonal formların düşünülebileceği kanısına varılmıştır. Çocuklarda fonksiyonel iyileşme sık olsa da uzun dönemde kronik şikâyetlerin devam edebileceği ve rekürrensler olabileceği bilinmeli, hastalar dikkatle izlenmeye devam edilmelidir.

Anahtar Kelimeler

• anormal derin tendon refleksi
• çocuk
• Guillain-Barré Sendromu

Kaynakça

1. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology. 2011;36:123-33.
2. van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol. 2008;7:939-50.
3. Yuki N, Hartung H-P. Guillain-Barré syndrome. The New England journal of medicine. 2012;366:2294-304.
4. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet (London, England). 2016;388:717-27.
5. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990;27 (Suppl S1):S21-4.
6. Cai F, Zhang J. Study of nerve conduction and late responses in normal Chinese infants, children, and adults. J Child Neurol. 1997;12:13-8.
7. Hughes RA, Newsom-Davis JM, Perkin GD, Pierce JM. Controlled trial prednisolone in acute polyneuropathy. Lancet (London, England). 1978;2:750-3.
8. Kleyweg RP, van der Meché FG, Schmitz PI. Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barré syndrome. Muscle Nerve. 1991;14:1103-9.

9. Ropper AH. The Guillain-Barré syndrome. The New England journal of medicine. 1992;326:1130-6.
10. Doets AY, Verboon C, van den Berg B, Harbo T, Cornblath DR, Willison HJ, et al. Regional variation of Guillain-Barré syndrome. Brain. 2018;141:2866-77.
11. Lin JJ, Hsia SH, Wang HS, Lyu RK, Chou ML, Hung PC, et al. Clinical variants of Guillain-Barré syndrome in children. Pediatr Neurol. 2012;47:91-6.
12. Tekgul H, Serdaroglu G, Tutuncuoglu S. Outcome of axonal and demyelinating forms of Guillain-Barré syndrome in children. Pediatr Neurol. 2003;28:295-9.
13. Webb AJ, Brain SA, Wood R, Rinaldi S, Turner MR. Seasonal variation in Guillain-Barré syndrome: a systematic review, meta-analysis and Oxfordshire cohort study. J Neurol Neurosurg Psychiatry. 2015;86:1196-201.
14. Kılıç B, Güngör S, Özgör B. Clinical, electrophysiological findings and evaluation of prognosis of patients with Guillain-Barré syndrome. Turk J Pediatr. 2019;61:200-8.
15. Karalok ZS, Taskin BD, Yanginlar ZB, Gurkas E, Guven A, Degerliyurt A, et al. Guillain-Barré syndrome in children: subtypes and outcome. Childs Nerv Syst. 2018;34:2291-7.
16. Jacobs BC, Rothbarth PH, van der Meché FG, Herbrink P, Schmitz PI, de Klerk MA, et al. The spectrum of antecedent infections in Guillain-Barré syndrome: a case-control study. Neurology. 1998;51:1110-5.
17. Donofrio PD. Guillain-Barré Syndrome. Continuum (Minneap Minn). 2017;23(5, Peripheral Nerve and Motor Neuron Disorders):1295-309.
18. Uncini A, Notturno F, Kuwabara S. Hyper-reflexia in Guillain-Barre syndrome: systematic review. J Neurol Neurosurg Psychiatry. 2020;91:278-84.
19. Kuwabara S, Ogawara K, Koga M, Mori M, Hattori T, Yuki N. Hyperreflexia in Guillain-Barre syndrome: relation with acute motor axonal neuropathy and anti-GM1 antibody. J Neurol Neurosurg Psychiatry. 1999;67:180-4.
20. Burke D, Wissel J, Donnan GA. Pathophysiology of spasticity in stroke. Neurology. 2013;80(Suppl 2):S20-6.
21. Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study. Neuropediatrics. 2007;38:10-7.
22. Nachamkin I, Arzarte Barbosa P, Ung H, Lobato C, Gonzalez Rivera A, Rodriguez P, et al. Patterns of Guillain-Barre syndrome in children: results from a Mexican population. Neurology. 2007;69:1665-71.
23. Konuskan B, Okuyaz Ç, Taşdelen B, Hız Kurul S, Anlar B. Electrophysiological Subtypes and Prognostic Factors of Childhood Guillain-Barré Syndrome. Archives of neuropsychiatry. 2018;55:199-204.
24. Guillain G, Barré JA, Strohl A. [Radiculoneuritis syndrome with hyperalbuminosis of cerebrospinal fluid without cellular reaction. Notes on clinical features and graphs of tendon reflexes. 1916]. Ann Med Interne (Paris). 1999;150:24-32.
25. Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain. 2014;137:33-43.
26. Yikilmaz A, Doganay S, Gumus H, Per H, Kumandas S, Coskun A. Magnetic resonance imaging of childhood Guillain-Barre syndrome. Childs Nerv Syst. 2010;26:1103-8.
27. Gorson KC, Ropper AH, Muriello MA, Blair R. Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome. Neurology. 1996;47:813-7.
28. Maloney JA, Mirsky DM, Messacar K, Dominguez SR, Schreiner T, Stence NV. MRI findings in children with acute flaccid paralysis and cranial nerve dysfunction occurring during the 2014 enterovirus D68 outbreak. AJNR Am J Neuroradiol. 2015;36:245-50.
29. Mulkey SB, Glasier CM, El-Nabbout B, Walters WD, Ionita C, McCarthy MH, et al. Nerve root enhancement on spinal MRI in pediatric Guillain-Barré syndrome. Pediatr Neurol. 2010;43:263-9.
30. Zuccoli G, Panigrahy A, Bailey A, Fitz C. Redefining the Guillain-Barré spectrum in children: neuroimaging findings of cranial nerve involvement. AJNR Am J Neuroradiol. 2011;32:639-42.
31. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019;15:671-83.
32. Kalra V, Sankhyan N, Sharma S, Gulati S, Choudhry R, Dhawan B. Outcome in childhood Guillain-Barré syndrome. The Indian Journal of Pediatrics. 2009;76:795.
33. Roodbol J, de Wit MC, Aarsen FK, Catsman-Berrevoets CE, Jacobs BC. Long-term outcome of Guillain-Barré syndrome in children. J Peripher Nerv Syst. 2014;19:121-6.
34. Kuitwaard K, van Koningsveld R, Ruts L, Jacobs BC, van Doorn PA. Recurrent Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 2009;80:56-9.
35. Ropper AH WE, Truax BT. Guillain-Barré syndrome. Contemporary Neurology Series. Philadelphia: FA Davis, 1991: 132.
36. Hadden RD, Cornblath DR, Hughes RA, Zielasek J, Hartung HP, Toyka KV, et al. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Ann Neurol. 1998;44:780-8.
37. Asbury AK. New Concepts of Guillain-Barré Syndrome. J Child Neurol. 2000;15:183-91.
38. Yadav S, Jain P, Sharma S, Kumar V, Aneja S. Guillain-Barre syndrome in North Indian children: Clinical and serial electrophysiological features. Neurol India. 2019;67:724-7.
39. Burns TM. Guillain-Barré syndrome. Semin Neurol. 2008;28:152-67.