Guillain-Barre Syndrome

Guillain Barre

 

Aw, the Winter Season in the Ped ED… how we all love the viral pathology! Let us recall, however, that we must be vigilant in the face of the seemingly constant waves of children with fevers and rhinorrhea. We already know that we should not say, “It’s Just a Virus.” While it is good to give appropriate anticipatory guidance, it is also important to be vigilant for potential complications of “simple” viral illnesses also (Pneumonia, Acute Otitis Media, Myocarditis). Let us review another potentially scary complication: Guillain-Barre Syndrome.

 

Guillain-Barre: Basics

  • Guillain-Barre Syndrome (GBS) is the most common cause of acute flaccid paralysis in children.
    • Incidence – 0.5 – 1.5 per 100,000 children/year in the US. [Sladky, 2004]
    • ~15% of children require mechanical ventilation. [Sladky, 2004]
    • Can be fatal in ~10% of patients [Lee, 2008]
  • GBS is thought to be an autoimmune disorder: [Ryan, 2013]
    • Antibodies attack motor and/or sensory peripheral nerves
    • Likely due to molecular mimicry.
      • Infections – EBV, CMV, Mycoplasma, Campylobacter jejuni
      • Immunizations
        • No clear causal relationship, but several cases describe an association.
        • 17% of cases in one study had recent immunization. [Hicks, 2010]
      • Surgery
  • Variable involvement of different types of peripheral nerves leads to several (9) subtypes.
    • Acute Inflammatory Demyelinating Polyneuropathy
      • “Classic form”
      • Accounts for ~75% of cases in the West
      • Typically recover more quickly than those w/ AMAN.
    • Acute Motor Axonal Neuropathy (AMAN)
      • No sensory deficits
      • Associated with epidemics following C. jejuni enteritis.
    • Miller-Fisher Syndrome
      • Uncommon
      • Triad of Ataxia, Areflexia, and Ophthalmoplegia
      • Bulbar weakness can be present
      • Affects adults more than children.

 

Guillain-Barre: Presentation

  • GBS typically occurs 2-4 weeks after a preceding illness.
    • 50-70% have GI or respiratory illness [Yuki, 2012]
  • Presenting Symptoms:
    • Weakness
      • 49% of cases [Hicks, 2010]
      • Limb weakness is both distal and proximal.
      • Limb weakness is one of the most common symptoms at onset. [Lee, 2008]
      • Up to 60% become unable to ambulate during illness.
    • Distal paresthesias
      • 54% of cases [Hicks, 2010]
      • Neuropathic pain affects children prominently
    • Myalgias
      • 49% of cases [Hicks, 2010]
    • Hyoreflexia or areflexia
      • Present in 94% of cases [Hicks, 2010]
      • Always check reflexes!!
    • Cranial Nerve Involvement
      • Bulbar palsies, Facial weakness, Optic nerve palsies [Teksam, 2015]
      • 46% of cases [Hicks, 2010]
    • Autonomic dysfunction
      • Blood pressure instability, sinus tachycardia
      • Abnormal sweating, pupillary abnormalities
      • Common and under-recognized in children!
  • Average number of days between symptom onset and peak of disease was ~8 days, but ranges from 2 to 27 days.

 

Guillain-Barre: Evaluation

  • Clinical Examination
    • Thorough neurologic examination
    • Don’t overlook reflexes.
    • Assess motor strength carefully.
    • Can be very difficult to diagnose in children and is often missed initially. [Bloch, 2013; Orlik, 2014]
  • Lumbar puncture
    • Albuminocytological dissociation (increased protein level without pleocytosis) is associated with decreased time to peak of disease. [Hicks, 2010]
    • Does not need to be done emergently.
    • May actually need to be repeated, if the initial LP was done after only a few days of symptoms and results were normal.
  • Radiologic investigations
    • Gadolinium enhanced MRI of the spine [Mulkey, 2010]
      • May show enhancement of the peripheral nerve roots and caudal equina.
      • Not specific to the disorder, but in the right clinical setting, can be helpful.
  • Nerve conduction studies
    • ~80% of studies are abnormal [Hicks, 2010]
    • May be normal early on in disease course.

 

Guillain-Barre: Treatment

  • Supportive Therapies
    • Monitoring respiratory status
    • Monitoring autonomic complications
    • Pain management
    • Prevention of immobility complications
  • IVIG
    • Often preferred in childhood
    • Total dose of 2 grams/kg over 2-5 days [Ryan, 2013]
  • Plasmapheresis
    • Useful for those patients > 10 kgs. [Ryan, 2013]

 

Moral of the Morsel

  • Fight against complacency for seemingly minor complaints.
    • That child who is limping or who’s family states is “weak” may appear well and actually have no significant pathology, but before you reach that conclusion, be thorough.
  • Strive to remain vigilant for presentations of potentially devastating conditions.
    • Ask about recent viral illnesses or immunizations.
    • Perform thorough neurologic exam, including reflexes.
    • Search for subtle weakness – make the child stand up from seated position without using hands.
  • It is with a keen, vigilant mind (not a million tests) that you will be able to make a the difference in the child’s life.

 

References

Teksam O1, Keser AG, Konuskan B, Haliloglu G, Oguz KK, Yalnizoglu D. Acute Abducens Nerve Paralysis in the Pediatric Emergency Department: Analysis of 14 Patients. Pediatr Emerg Care. 2015 Mar 24. PMID: 25806679. [PubMed] [Read by QxMD]

Varkal MA1, Uzunhan TA1, Aydınlı N1, Ekici B1, Çalışkan M1, Özmen M1. Pediatric Guillain-Barré syndrome: Indicators for a severe course. Ann Indian Acad Neurol. 2015 Jan-Mar;18(1):24-8. PMID: 25745306. [PubMed] [Read by QxMD]

Orlik K1, Griffin GD2. Guillain-Barré in a 10-month-old: diagnostic challenges in a pediatric emergency. Am J Emerg Med. 2014 Jan;32(1):110. PMID: 24051012. [PubMed] [Read by QxMD]

Ryan MM1. Pediatric Guillain-Barré syndrome. Curr Opin Pediatr. 2013 Dec;25(6):689-93. PMID: 24240288. [PubMed] [Read by QxMD]

Bloch SA1, Akhavan M, Avarello J. Weakness and the inability to ambulate in a 14-month-old female: a case report and concise review of guillain-barre syndrome. Case Rep Emerg Med. 2013;2013:953612. PMID: 23431480. [PubMed] [Read by QxMD]

Hu MH1, Chen CM, Lin KL, Wang HS, Hsia SH, Chou ML, Hung PC, Wu CT. Risk factors of respiratory failure in children with Guillain-Barré syndrome. Pediatr Neonatol. 2012 Oct;53(5):295-9. PMID: 23084721. [PubMed] [Read by QxMD]

Yuki N1, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2012 Jun 14;366(24):2294-304. PMID: 22694000. [PubMed] [Read by QxMD]
Zuccoli G1, Panigrahy A, Bailey A, Fitz C. Redefining the Guillain-Barré spectrum in children: neuroimaging findings of cranial nerve involvement. AJNR Am J Neuroradiol. 2011 Apr;32(4):639-42. PMID: 21292802. [PubMed] [Read by QxMD]

Hicks CW1, Kay B, Worley SE, Moodley M. A clinical picture of Guillain-Barré syndrome in children in the United States. J Child Neurol. 2010 Dec;25(12):1504-10. PMID: 20823034. [PubMed] [Read by QxMD]

Mulkey SB1, Glasier CM, El-Nabbout B, Walters WD, Ionita C, McCarthy MH, Sharp GB, Shbarou RM. Nerve root enhancement on spinal MRI in pediatric Guillain-Barré syndrome. Pediatr Neurol. 2010 Oct;43(4):263-9. PMID: 20837305. [PubMed] [Read by QxMD]

Lee JH1, 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. PMID: 18557809. [PubMed] [Read by QxMD]

Sladky JT1. Guillain-Barré syndrome in children. J Child Neurol. 2004 Mar;19(3):191-200. PMID: 15119480. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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2 Responses

  1. January 24, 2016

    […] lexicons. One simple reason is that viruses can lead to significant illness also (ex, myocarditis, Guillain-Barre). While ordering a million tests and admitting everyone is not the answer, remaining vigilant can […]

  2. December 30, 2016

    […] is due to a viral illness, but remember viral illnesses can become complicated (see, Myocarditis, Guillaine-Barre). So, take care not to be dismissive. Additionally, while you are doing your best to avoid the […]

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