Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis
- Monique M Ryan, FRACP
Monique M Ryan, FRACP
- University of Melbourne
- Head, Department of Neurology
- Royal Children's Hospital Melbourne
- Section Editors
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
- Sheldon L Kaplan, MD
Sheldon L Kaplan, MD
- Editor-in-Chief — Pediatrics
- Section Editor — Pediatric Infectious Diseases
- Professor and Vice Chairman for Clinical Affairs
- Baylor College of Medicine
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré syndrome (GBS), after the authors of early descriptions of the disease. Historically, GBS was considered a single disorder, but it is now known to be a heterogeneous syndrome with several variant forms. Most often, GBS presents as an acute monophasic paralyzing illness provoked by a preceding infection. In addition to the demyelinating form, which is the most common type, axonal forms of GBS are well-recognized.
This topic will review the epidemiology, clinical features, and diagnosis of GBS in children. Other aspects of GBS are discussed separately. (See "Guillain-Barré syndrome: Pathogenesis" and "Guillain-Barré syndrome in children: Treatment and prognosis".)
The pathogenesis of Guillain-Barré syndrome (GBS) is discussed here briefly and reviewed in detail elsewhere. (See "Guillain-Barré syndrome: Pathogenesis".)
One proposed mechanism for GBS is that an antecedent infection evokes an immune response, which in turn cross-reacts with peripheral nerve components because of the sharing of cross-reactive epitopes (molecular mimicry). The end result is an acute polyneuropathy. This immune response can be directed towards the myelin or the axon of peripheral nerve. The main lesions of GBS are acute inflammatory demyelinating polyradiculoneuropathy and, particularly in patients with Campylobacter-associated disease, acute axonal degeneration. (See "Guillain-Barré syndrome: Pathogenesis", section on 'Molecular mimicry' and "Guillain-Barré syndrome: Pathogenesis", section on 'Pathology'.)
Antecedent infections are common with GBS, and are thought to trigger the immune response that leads to acute polyneuropathy. Approximately two-thirds of patients give a history of an antecedent respiratory tract or gastrointestinal infection. Campylobacter infection is the most commonly identified precipitant of GBS and can be demonstrated in as many as 30 percent of cases. Other precipitants include cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, and influenza-like illnesses. GBS can also occur in association with human immunodeficiency virus (HIV) infection, predominantly in those who are not profoundly immunocompromised. A small percentage of patients develop GBS after another triggering event such as immunization, surgery, trauma, or bone-marrow transplantation. (See "Guillain-Barré syndrome: Pathogenesis", section on 'Antecedent events'.)
- Verity C, Stellitano L, Winstone AM, et al. Guillain-Barré syndrome and H1N1 influenza vaccine in UK children. Lancet 2011; 378:1545.
- Bardage C, Persson I, Ortqvist A, et al. Neurological and autoimmune disorders after vaccination against pandemic influenza A (H1N1) with a monovalent adjuvanted vaccine: population based cohort study in Stockholm, Sweden. BMJ 2011; 343:d5956.
- Lee GM, Greene SK, Weintraub ES, et al. H1N1 and seasonal influenza vaccine safety in the vaccine safety datalink project. Am J Prev Med 2011; 41:121.
- Jones HR Jr. Guillain-Barré syndrome: perspectives with infants and children. Semin Pediatr Neurol 2000; 7:91.
- Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012; 366:2294.
- Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011; 36:123.
- Morris AM, Elliott EJ, D'Souza RM, et al. Acute flaccid paralysis in Australian children. J Paediatr Child Health 2003; 39:22.
- Carroll JE, Jedziniak M, Guggenheim MA. Guillain-Barré syndrome. Another cause of the "floppy infant". Am J Dis Child 1977; 131:699.
- Buchwald B, de Baets M, Luijckx GJ, Toyka KV. Neonatal Guillain-Barré syndrome: blocking antibodies transmitted from mother to child. Neurology 1999; 53:1246.
- Hahn AF. Guillain-Barré syndrome. Lancet 1998; 352:635.
- Ropper AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326:1130.
- Evans OB, Vedanarayanan V. Guillain-Barré syndrome. Pediatr Rev 1997; 18:10.
- Jones HR. Childhood Guillain-Barré syndrome: clinical presentation, diagnosis, and therapy. J Child Neurol 1996; 11:4.
- Pier DB, Hallbergson A, Peters JM. Guillain-Barré syndrome in a child with pain: lessons learned from a late diagnosis. Acta Paediatr 2010; 99:1589.
- Roodbol J, de Wit MC, Walgaard C, et al. Recognizing Guillain-Barre syndrome in preschool children. Neurology 2011; 76:807.
- Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study. Neuropediatrics 2007; 38:10.
- Nguyen DK, Agenarioti-Bélanger S, Vanasse M. Pain and the Guillain-Barré syndrome in children under 6 years old. J Pediatr 1999; 134:773.
- Hund EF, Borel CO, Cornblath DR, et al. Intensive management and treatment of severe Guillain-Barré syndrome. Crit Care Med 1993; 21:433.
- Maier H, Schmidbauer M, Pfausler B, et al. Central nervous system pathology in patients with the Guillain-Barré syndrome. Brain 1997; 120 ( Pt 3):451.
- Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol 1990; 27 Suppl:S21.
- Kleyweg RP, van der Meché FG, Loonen MC, et al. The natural history of the Guillain-Barré syndrome in 18 children and 50 adults. J Neurol Neurosurg Psychiatry 1989; 52:853.
- Cole GF, Matthew DJ. Prognosis in severe Guillain-Barré syndrome. Arch Dis Child 1987; 62:288.
- Briscoe DM, McMenamin JB, O'Donohoe NV. Prognosis in Guillain-Barré syndrome. Arch Dis Child 1987; 62:733.
- Wong V. A neurophysiological study in children with Miller Fisher syndrome and Guillain-Barre syndrome. Brain Dev 1997; 19:197.
- Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group. Neurology 1985; 35:1096.
- Lamont PJ, Johnston HM, Berdoukas VA. Plasmapheresis in children with Guillain-Barré syndrome. Neurology 1991; 41:1928.
- Epstein MA, Sladky JT. The role of plasmapheresis in childhood Guillain-Barré syndrome. Ann Neurol 1990; 28:65.
- Lin JJ, Hsia SH, Wang HS, et al. Clinical variants of Guillain-Barré syndrome in children. Pediatr Neurol 2012; 47:91.
- Arcila-Londono X, Lewis RA. Guillain-Barré syndrome. Semin Neurol 2012; 32:179.
- McKhann GM, Cornblath DR, Griffin JW, et al. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neurol 1993; 33:333.
- Visser LH, Van der Meché FG, Van Doorn PA, et al. Guillain-Barré syndrome without sensory loss (acute motor neuropathy). A subgroup with specific clinical, electrodiagnostic and laboratory features. Dutch Guillain-Barré Study Group. Brain 1995; 118 ( Pt 4):841.
- Ho TW, Mishu B, Li CY, et al. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain 1995; 118 ( Pt 3):597.
- Reisin RC, Cersósimo R, García Alvarez M, et al. Acute "axonal" Guillain-Barré syndrome in childhood. Muscle Nerve 1993; 16:1310.
- Paradiso G, Tripoli J, Galicchio S, Fejerman N. Epidemiological, clinical, and electrodiagnostic findings in childhood Guillain-Barré syndrome: a reappraisal. Ann Neurol 1999; 46:701.
- Nagasawa K, Kuwabara S, Misawa S, et al. Electrophysiological subtypes and prognosis of childhood Guillain-Barré syndrome in Japan. Muscle Nerve 2006; 33:766.
- Nachamkin I, Arzarte Barbosa P, Ung H, et al. Patterns of Guillain-Barre syndrome in children: results from a Mexican population. Neurology 2007; 69:1665.
- Ho TW, Li CY, Cornblath DR, et al. Patterns of recovery in the Guillain-Barre syndromes. Neurology 1997; 48:695.
- Feasby TE, Gilbert JJ, Brown WF, et al. An acute axonal form of Guillain-Barré polyneuropathy. Brain 1986; 109 ( Pt 6):1115.
- Feasby TE, Hahn AF, Brown WF, et al. Severe axonal degeneration in acute Guillain-Barré syndrome: evidence of two different mechanisms? J Neurol Sci 1993; 116:185.
- FISHER M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255:57.
- Marks HG, Augustyn P, Allen RJ. Fisher's syndrome in children. Pediatrics 1977; 60:726.
- Winer JB. Bickerstaff's encephalitis and the Miller Fisher syndrome. J Neurol Neurosurg Psychiatry 2001; 71:433.
- Odaka M, Yuki N, Hirata K. Anti-GQ1b IgG antibody syndrome: clinical and immunological range. J Neurol Neurosurg Psychiatry 2001; 70:50.
- Shahrizaila N, Yuki N. Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry 2013; 84:576.
- Visser LH, van der Meché FG, Meulstee J, et al. Cytomegalovirus infection and Guillain-Barré syndrome: the clinical, electrophysiologic, and prognostic features. Dutch Guillain-Barré Study Group. Neurology 1996; 47:668.
- Morosini A, Burke C, Emechete B. Polyneuritis cranialis with contrast enhancement of cranial nerves on magnetic resonance imaging. J Paediatr Child Health 2003; 39:69.
- Polo A, Manganotti P, Zanette G, De Grandis D. Polyneuritis cranialis: clinical and electrophysiological findings. J Neurol Neurosurg Psychiatry 1992; 55:398.
- Ropper AH. Unusual clinical variants and signs in Guillain-Barré syndrome. Arch Neurol 1986; 43:1150.
- Mogale KD, Antony JH, Ryan MM. The pharyngeal-cervical-brachial form of Guillain-Barré syndrome in childhood. Pediatr Neurol 2005; 33:285.
- Rousseff RT, Khuraibet AJ, Neubauer D. The "Child in the Barrel syndrome"--severe pharyngeal-cervical-brachial variant of Guillain-Barre Syndrome in a toddler. Neuropediatrics 2008; 39:354.
- Ropper AH. Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness. Arch Neurol 1994; 51:671.
- Nass R, Chutorian A. Dysaesthesias and dysautonomia: a self-limited syndrome of painful dysaesthesias and autonomic dysfunction in childhood. J Neurol Neurosurg Psychiatry 1982; 45:162.
- Mericle RA, Triggs WJ. Treatment of acute pandysautonomia with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 1997; 62:529.
- Wicklein EM, Pfeiffer G, Yuki N, et al. Prominent sensory ataxia in Guillain-Barré syndrome associated with IgG anti-GD1b antibody. J Neurol Sci 1997; 151:227.
- van den Berg B, Fokke C, Drenthen J, et al. Paraparetic Guillain-Barré syndrome. Neurology 2014; 82:1984.
- Ropper AH, Wijdicks EFM, Truax BT. Guillain-Barré syndrome, FA Davis, Philadelphia 1991. p.57.
- Nishimoto Y, Odaka M, Hirata K, Yuki N. Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination. J Neuroimmunol 2004; 148:200.
- Delanoe C, Sebire G, Landrieu P, et al. Acute inflammatory demyelinating polyradiculopathy in children: clinical and electrodiagnostic studies. Ann Neurol 1998; 44:350.
- Bradshaw DY, Jones HR Jr. Guillain-Barré syndrome in children: clinical course, electrodiagnosis, and prognosis. Muscle Nerve 1992; 15:500.
- Korinthenberg R, Mönting JS. Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study. Arch Dis Child 1996; 74:281.
- Coşkun A, Kumandaş S, Paç A, et al. Childhood Guillain-Barré syndrome. MR imaging in diagnosis and follow-up. Acta Radiol 2003; 44:230.
- Yikilmaz A, Doganay S, Gumus H, et al. Magnetic resonance imaging of childhood Guillain-Barre syndrome. Childs Nerv Syst 2010; 26:1103.
- Mulkey SB, Glasier CM, El-Nabbout B, et al. Nerve root enhancement on spinal MRI in pediatric Guillain-Barré syndrome. Pediatr Neurol 2010; 43:263.
- 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.
- Chiba A, Kusunoki S, Obata H, et al. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology 1993; 43:1911.
- Willison HJ, Veitch J, Paterson G, Kennedy PG. Miller Fisher syndrome is associated with serum antibodies to GQ1b ganglioside. J Neurol Neurosurg Psychiatry 1993; 56:204.
- Ruts L, Drenthen J, Jacobs BC, et al. Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study. Neurology 2010; 74:1680.
- CLINICAL FEATURES
- Physical examination
- Clinical course
- SUBTYPES OF GUILLAIN-BARRÉ SYNDROME
- Acute inflammatory demyelinating polyneuropathy
- Acute motor axonal neuropathy
- Acute motor-sensory axonal neuropathy
- Miller Fisher syndrome
- Bickerstaff encephalitis
- Polyneuritis cranialis
- Pharyngeal-cervical-brachial weakness
- Other variants
- Cerebrospinal fluid
- Electrodiagnostic studies
- Magnetic resonance imaging
- DIFFERENTIAL DIAGNOSIS
- Chronic inflammatory demyelinating polyneuropathy
- Other polyneuropathies
- Spinal cord disorders
- Neuromuscular junction disorders
- Muscle disorders
- INFORMATION FOR PATIENTS