Pediatric Guillain-Barre Syndrome Clinical Presentation
- Author: Marc P DiFazio, MD; Chief Editor: Amy Kao, MD more...
Patients with Guillain-Barré syndrome (GBS) present with complaints of weakness and/or unsteadiness (ataxia). Weakness is a hallmark of GBS. The weakness typically starts in the legs and ascends to the arms (hence, the description progressive ascending flaccid paralysis). This progression may occur over hours to days to weeks. The weakness is usually symmetric.
Pain and dysesthesias also are noted, particularly in children. Pain may be the initial manifestation in almost half of affected children. The nonspecific nature of the symptoms may distract from the actual diagnosis, for exampe, a pediatric patient presenting with prolonged and unexplained abdominal pain.
Often, onset of these symptoms is within 2-4 weeks of an illness or immunization. The preceding illness often involves fever, muscle pains, diarrhea or upper respiratory infection.
Urinary retention is also noted early in the course of 10-15% of children with GBS. At the peak of illness, about half the pediatric patients with GBS may have associated autonomic dysfunction and cranial nerve (CN) involvement, and about 10-12% require a mechanical ventilator. In those with CN involvement, the facial nerve is most commonly affected, resulting in bilateral facial weakness.
Subtypes of GBS
GBS peripheral nerve damage can be classified histopathologically into 2 main types: demyelinating forms and axonal-degenerating forms. Motor nerves are more susceptible to disease than sensory ones. In 1995, GBS was subdivided into 4 distinct forms based on histopathological and neurophysiological basis: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and Miller-Fisher syndrome (MFS).[25, 26]
The clinical spectrum of GBS, which includes individual variation and variable severity of presentation, comprises the following:
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) - This accounts for 80-90% of GBS cases in Europe and North America. It is characterized by an immune-mediated attack on myelin with infiltration of lymphocytes and macrophages with segmental stripping of myelin. Motor and sensory fibers are usually affected simultaneously, producing corresponding deficits. Electrophysiology shows slow nerve conduction velocity and prolonged F waves.
Acute motor axonal neuropathy (AMAN) - This form of neuropathy is most commonly seen in China and Japan (50-60% of cases), as apposed to Western countries (10-20% of cases). In this form, axonal degeneration occurs by immune attack within 1-2 weeks after infection. Specific antibodies to axonal membranes of motor fibers attack the nodes of Ranvier. This, in turn, activates complement and intrusion of macrophages into periaxonal space, resulting in destruction of axons. C jejuni is the most common preceding infection, and antiganglioside antibodies are usually found in this type. Electrophysiology shows reduction in muscle action potentials with relatively preserved motor nerve conduction velocity and normal sensory nerve action potentials and F waves. [25, 27]
Acute motor and sensory axonal neuropathy (AMSAN) - This type is rare and resembles AMAN except sensory nerves are also affected. This type is associated with a severe course and poor prognosis.
Miller-Fisher syndrome (MFS) - The involvement of CNs is very distinct in this form of GBS. Ocular motor nerves (oculomotor, trochlear, and abducens) are affected and produce a triad of ophthalmoplegia, ataxia, and areflexia. Electrophysiology is normal. The characteristic autoantibodies are against gangliosides GQ1b and GT1a. GQ1b plays a key role in the pathogenesis of MFS. 
Polyneuritis cranialis - This is an acute onset of multiple CN palsies (usually bilateral CN VII with sparing of CNs I and II), elevated cerebrospinal fluid protein, and slowed nerve conduction velocity with uncomplicated recovery.
Pharyngo-cervical-brachial syndrome - This variant form of GBS is characterized by localized and regional involvement of autonomic and motor nerves in the pharyngeal-cervical-brachial distribution. The diagnosis of this condition is based on clinical, laboratory, and neurophysiological findings and the exclusion of other conditions mimicking this disorder.
Acute sensory neuropathy of childhood
Acute pandysautonomia - Besides the above main forms of GBS, acute pandysautonomia is also a common subtype with which the autonomic nervous system is involved. Parasympathetic and sympathetic involvement is seen along with sensory or motor nerve involvement.
On physical examination, an ascending motor weakness is noted along with areflexia in the classic form. Areflexia is a hallmark of GBS. Occasionally, some of the more proximal reflexes still may be elicited during the early phase of the disease. Of clinical value is documenting reflexes in serial exams; the progression from normoreflexia/hyporeflexia to areflexia is consistent with acute features of GBS.
Occasionally, autonomic instability (26%), ataxia (23%), dysesthesias (20%), and cranial nerve findings (35-50%), predominantly facial palsy, are noted. These latter findings are probably more frequent in children than in adults with this syndrome.
Leg weakness (ie, foot drop) is usually noticed first and weakness eventually involves the calves and thighs. Later, respiratory muscles and upper extremities show involvement. Some children may become non-ambulatory. Weakness also may involve the respiratory muscles, and some children need respiratory support during the course of the disease. Mechanical ventilation is used until respiratory muscle function returns.
The autonomic neuropathy involves both the sympathetic and parasympathetic systems. Manifestations include orthostatic hypotension, hypertension, pupillary dysfunction, sweating abnormalities, and sinus tachycardia.
Complications of GBS
The most common serious complications are weakness of the respiratory muscles and autonomic instability. Pneumonia, adult respiratory distress syndrome, septicemia, pressure sores, pulmonary embolus, ileus, constipation, gastritis and dysesthesias are also important potential complications. Nephropathy has been reported in pediatric patients.
Features that would put the diagnosis in doubt include (1) marked persistent weakness, (2) bowel and bladder dysfunction at onset, (3) persistent bladder or bowel dysfunction, (4) mononuclear leukocytosis in the cerebrospinal fluid (>50 cells/µL), and (5) a sharp sensory level
Features that rule out the diagnosis include (1) a current history of hexacarbon abuse; (2) abnormal porphyria metabolism; (3) recent diphtheria infection; and (4) evidence of polio, botulism, toxic neuropathy, tic paralysis, or organophosphate poisoning.
Features required for diagnosis are (1) progressive weakness of more than one extremity, (2) hyporeflexia or areflexia, (3) elevated cerebrospinal fluid protein (>45 mg/dL) after 1 week following onset of symptoms, and (4) slow conduction velocity or prolonged F wave on electrophysiology testing.
Kuwabara S. Guillain-Barré syndrome: epidemiology, pathophysiology and management. Drugs. 2004. 64(6):597-610. [Medline].
Lee JH, Sung IY, Rew IS. Clinical presentation and prognosis of childhood Guillain-Barré syndrome. J Paediatr Child Health. 2008 Jul-Aug. 44(7-8):449-54. [Medline].
Kimoto K, Koga M, Odaka M, Hirata K, Takahashi M, Li J, et al. Relationship of bacterial strains to clinical syndromes of Campylobacter-associated neuropathies. Neurology. 2006 Nov 28. 67(10):1837-43. [Medline].
Souayah N, Nasar A, Suri MF, Qureshi AI. Guillain-Barré syndrome after vaccination in United States: data from the Centers for Disease Control and Prevention/Food and Drug Administration Vaccine Adverse Event Reporting System (1990-2005). J Clin Neuromuscul Dis. 2009 Sep. 11(1):1-6. [Medline].
CDC. Estimating Deaths from Seasonal Influenza in the United States. [Full Text].
Preliminary results: surveillance for Guillain-Barré syndrome after receipt of influenza A (H1N1) 2009 monovalent vaccine - United States, 2009-2010. MMWR Morb Mortal Wkly Rep. 2010 Jun 4. 59(21):657-61. [Medline].
World Health Organization. Programmes and Projects - Global Alert and Response (GAR). Pandemic (H1N1) 2009 briefing notes. World Health Organization. Available at http://www.who.int/csr/disease/swineflu/notes/briefing_20091119/en/. Accessed: January 8, 2012.
Tremblay ME, Closon A, D'Anjou G, Bussières JF. Guillain-Barré syndrome following H1N1 immunization in a pediatric patient. Ann Pharmacother. 2010 Jul-Aug. 44(7-8):1330-3. [Medline].
da Silveira CM, Salisbury DM, de Quadros CA. Measles vaccination and Guillain-Barré syndrome. Lancet. 1997 Jan 4. 349(9044):14-6. [Medline].
Slade BA, Leidel L, Vellozzi C, Woo EJ, Hua W, Sutherland A, et al. Postlicensure safety surveillance for quadrivalent human papillomavirus recombinant vaccine. JAMA. 2009 Aug 19. 302(7):750-7. [Medline].
Smith MJ. Meningococcal tetravalent conjugate vaccine. Expert Opin Biol Ther. 2008 Dec. 8(12):1941-6. [Medline].
Landaverde JM, Danovaro-Holliday MC, Trumbo SP, Pacis-Tirso CL, Ruiz-Matus C. Guillain-Barré syndrome in children aged 1111111111111111J Infect Dis</i>. 2010 Mar. 201(5):746-50. [Medline].
Griffin JW, Li CY, Ho TW, Tian M, Gao CY, Xue P, et al. Pathology of the motor-sensory axonal Guillain-Barré syndrome. Ann Neurol. 1996 Jan. 39(1):17-28. [Medline].
Griffin JW, Li CY, Ho TW, Xue P, Macko C, Gao CY, et al. Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain. 1995 Jun. 118 ( Pt 3):577-95. [Medline].
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 Oct 23. 69(17):1665-71. [Medline].
Kalra V, Chaudhry R, Dua T, Dhawan B, Sahu JK, Mridula B. Association of Campylobacter jejuni infection with childhood Guillain-Barré syndrome: a case-control study. J Child Neurol. 2009 Jun. 24(6):664-8. [Medline].
Barzegar M, Alizadeh A, Toopchizadeh V, Dastgiri S, Majidi J. Association of Campylobacter jejuni infection and GuillainBarré syndrome: a cohort study in the northwest of Iran. Turk J Pediatr. 2008 Sep-Oct. 50(5):443-8. [Medline].
Islam Z, Jacobs BC, Islam MB, Mohammad QD, Diorditsa S, Endtz HP. High incidence of Guillain-Barre syndrome in children, Bangladesh. Emerg Infect Dis. 2011 Jul. 17(7):1317-8. [Medline].
Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study. Neuropediatrics. 2007 Feb. 38(1):10-7. [Medline].
Shafqat S, Khealani BA, Awan F, Abedin SE. Guillain-Barré syndrome in Pakistan: similarity of demyelinating and axonal variants. Eur J Neurol. 2006 Jun. 13(6):662-5. [Medline].
Kalra V, Sankhyan N, Sharma S, Gulati S, Choudhry R, Dhawan B. Outcome in childhood Guillain-Barré syndrome. Indian J Pediatr. 2009 Aug. 76(8):795-9. [Medline].
Roodbol J, de Wit MC, Walgaard C, de Hoog M, Catsman-Berrevoets CE, Jacobs BC. Recognizing Guillain-Barre syndrome in preschool children. Neurology. 2011 Mar 1. 76(9):807-10. [Medline].
al-Qudah AA, Shahar E, Logan WJ, Murphy EG. Neonatal Guillain-Barré syndrome. Pediatr Neurol. 1988 Jul-Aug. 4(4):255-6. [Medline].
Kieseier BC, Kiefer R, Gold R, Hemmer B, Willison HJ, Hartung HP. Advances in understanding and treatment of immune-mediated disorders of the peripheral nervous system. Muscle Nerve. 2004 Aug. 30(2):131-56. [Medline].
Hughes RA. The concept and classification of Guillain-Barré syndrome and related disorders. Rev Neurol (Paris). 1995 May. 151(5):291-4. [Medline].
Schwerer B. Antibodies against gangliosides: a link between preceding infection and immunopathogenesis of Guillain-Barré syndrome. Microbes Infect. 2002 Mar. 4(3):373-84. [Medline].
Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with Guillain-Barré syndrome. Pediatr Nephrol. 2004 Jan. 19(1):105-6. [Medline].
Heiner JD, Ball VL. A child with benign acute childhood myositis after influenza. J Emerg Med. 2010 Sep. 39(3):316-9. [Medline].
Lacroix LE, Galetto A, Haenggeli CA, Gervaix A. Delayed recognition of Guillain-Barré syndrome in a child: a misleading respiratory distress. J Emerg Med. 2010 Jun. 38(5):e59-61. [Medline].
Gorson KC, Ropper AH, Muriello MA, Blair R. Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome. Neurology. 1996 Sep. 47(3):813-7. [Medline].
Nishimoto Y, Susuki K, Yuki N. Serologic marker of acute motor axonal neuropathy in childhood. Pediatr Neurol. 2008 Jul. 39(1):67-70. [Medline].
Schessl J, Koga M, Funakoshi K, Kirschner J, Muellges W, Weishaupt A, et al. Prospective study on anti-ganglioside antibodies in childhood Guillain-Barré syndrome. Arch Dis Child. 2007 Jan. 92(1):48-52. [Medline]. [Full Text].
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 Jun. 19(2):121-6. [Medline].
Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003 Sep 23. 61(6):736-40. [Medline].
Yata J, Nihei K, Ohya T, Hirano Y, Momoi M, Maekawa K, et al. High-dose immunoglobulin therapy for Guillain-Barré syndrome in Japanese children. Pediatr Int. 2003 Oct. 45(5):543-9. [Medline].
Korinthenberg R, Schessl J, Kirschner J, Mönting JS. Intravenously administered immunoglobulin in the treatment of childhood Guillain-Barré syndrome: a randomized trial. Pediatrics. 2005 Jul. 116(1):8-14. [Medline].
Baranwal AK, Ravi RN, Singh R. Exchange transfusion: a low-cost alternative for severe childhood Guillain-Barré syndrome. J Child Neurol. 2006 Nov. 21(11):960-5. [Medline].
Shahar E. Current therapeutic options in severe Guillain-Barré syndrome. Clin Neuropharmacol. 2006 Jan-Feb. 29(1):45-51. [Medline].