eMedicine Specialties > Neurology > Pediatric Neurology

Guillain-Barre Syndrome in Childhood: Treatment & Medication

Author: Brian S Tseng, MD, PhD, Assistant Professor, Department of Neurology, Division of Pediatric Neurology, Harvard Medical School, Massachusetts General Hospital
Coauthor(s): Jennifer A Markowitz, MD, Fellow in Neuromuscular Disease, Massachusetts General Hospital and Brigham and Women's Hospital
Contributor Information and Disclosures

Updated: Sep 18, 2008

Treatment

Medical Care

To date, treatment for Guillain-Barré syndrome (GBS) has been aimed primarily at immunomodulation. In pediatrics, the most effective form of therapy is generally considered to be intravenous immunoglobulin (IVIG). Each batch of IVIG is made of human plasma derived from pools of 3,000-10,000 donors.

  • IVIG has been used in multiple studies to treat the symptoms. It seems helpful in reducing the severity of the disease as well as the duration of symptoms. However, the long-term outcome may not be affected. Several regimens have been used. The optimal dose and dosage schedules for IVIG have not been rigorously determined in childhood GBS. Only one prospective, randomized treatment trial in childhood GBS is published. 
    • One possible regimen includes daily administration of IVIG for 5 days at a dose of 0.4 g/kg/d, which can lead to improvements 2-3 days after the start of therapy. IVIG can be given by way of a peripheral intravenous route.
    • Some authors use 2 g/kg of IVIG given as a single dose or 1 g/kg/d over 2 days in children who are showing rapid signs of deterioration.
  • Plasmapheresis: Studies in children using both historical and case controls indicate that plasmapheresis may decrease the severity and shorten the duration of GBS. 
    • Between 4 and 5 plasmapheresis treatments may be performed over 7-10 days, as described in standard protocols.
    • Potential complications include autonomic instability, hypercalcemia, and bleeding due to depletion of clotting factors.
    • Results of plasmapheresis and IVIG are similar, with possibly fewer side effects seen with IVIG.
    • It stands to reason that plasmapheresis should not typically follow IVIG administration.
    • Plasmapheresis may be offered in some pediatric centers but is limited to larger children. In most institutions, children weighing less than 10-15 kg may not be considered for volume exchange therapy and central line vascular access dictates intensive care hospitalization. These features distinguish plasmapheresis from IVIG, which can be given to smaller children. Also, IVIG can be administered to patients by peripheral IV in specialized ambulatory clinic settings, advanced home nursing programs, and at ward level hospital settings.
  • Although steroids previously were used to treat GBS, current data suggest that they provide no to little benefit.

Consultations

  • Consultation with a neurologist should be considered to confirm the diagnosis. Intensivists may need to be involved quickly if critical care (cardiorespiratory) issues are suspected.
  • Patients who need a prolonged time for recovery may benefit from consultation with a rehabilitation medicine specialist.

Activity

  • When stabilized, activity with physical and/or occupational therapy should be encouraged. If motor deficits are profound, prevention of decubitus ulcers is highly important.
  • In addition to the weakness, autonomic symptoms (eg, orthostatic hypotension) may also restrict activity and should be monitored.  

Medication

The goals of pharmacotherapy are to reduce morbidity and prevent complications. Intravenous immunoglobulin (IVIG) is the predominant choice in childhood Guillain-Barré syndrome (GBS). DVT prophylaxis should be targeted and gastritis stress symptoms may benefit from H2 blockers (eg, ranitidine).

Blood products

IVIG is an effective treatment of autoimmune neuropathies in general. It can reduce duration of hospitalization as well as need or duration for mechanical ventilation.


IVIG (Gammagard, Gamimune)

Features relevant to efficacy may include neutralization of circulating myelin antibodies through anti-idiotypic antibodies; down-regulation of proinflammatory cytokines, including IFN-gamma; blockade of Fc receptors on macrophages; suppression of inducer T and B cells and augmentation of suppressor T cells; blockade of complement cascade; promotion of remyelination; 10% increase in CSF IgG.

Adult

2 g/kg IV over 2-5 d

Pediatric

Possible regimen includes 0.4 g/kg/d IV for 5 d; other authors use 2 g/kg once or 1 g/kg/d over 2 d

Documented hypersensitivity to product; severe systemic response to immune globulin (human); IgA deficiency; anti-IgE/IgG antibodies

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Screen patients before IVIG administration; IgA deficiency occurs in about 1 out of 1000 people; exercise extreme caution in patients with congestive heart failure or renal insufficiency; rare reports exist of noncardiogenic pulmonary edema, acute renal failure, aseptic meningitis, hemolytic anemia, and thrombotic events
Adverse reactions are usually minor and occur in no more than 10% of patients, which include mild-to-moderate headache, chills, chest discomfort, fatigue, fever, nausea, wheezing, dizziness, rashes, pains, and tenderness at injection site
Consider checking serum IgA before IVIG and using IgA-depleted IVIG (G-Gard-SD) if indicated; IVIG may increase serum viscosity and thromboembolic events
Adverse effects include migraine attacks, 10% increased risk of aseptic meningitis; increased risk of urticaria, pruritus, or petechiae 2-5 d postinfusion and lasting as long as 1 mo; increased risk of renal tubular necrosis in older patients, diabetic patients, volume-depleted patients, and patients with preexisting kidney disease
Can lead to changes in lab values including elevated antiviral or antibacterial antibody titers for 1 mo; 6-fold increased ESR for 2-3 wk; apparent hyponatremia

More on Guillain-Barre Syndrome in Childhood

Overview: Guillain-Barre Syndrome in Childhood
Differential Diagnoses & Workup: Guillain-Barre Syndrome in Childhood
Treatment & Medication: Guillain-Barre Syndrome in Childhood
Follow-up: Guillain-Barre Syndrome in Childhood
References
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Further Reading

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Keywords

Guillain-Barre syndrome in children, Guillain-Barre syndrome, Guillain-Barré-Strohl syndrome, acute inflammatory demyelinating polyneuropathy, acute inflammatory demyelinating polyradiculopathy, AIDP, acute febrile polyneuritis, GBS, acute motor axonal neuropathy, AMAN

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Contributor Information and Disclosures

Author

Brian S Tseng, MD, PhD, Assistant Professor, Department of Neurology, Division of Pediatric Neurology, Harvard Medical School, Massachusetts General Hospital
Brian S Tseng, MD, PhD is a member of the following medical societies: Child Neurology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Jennifer A Markowitz, MD, Fellow in Neuromuscular Disease, Massachusetts General Hospital and Brigham and Women's Hospital
Jennifer A Markowitz, MD is a member of the following medical societies: Child Neurology Society
Disclosure: Nothing to disclose.

Medical Editor

Robert Stanley Rust Jr, MD, MA, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology
Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Neil A Busis, MD, Chief, Division of Neurology, Department of Medicine, Head, Clinical Neurophysiology Laboratory, University of Pittsburgh Medical Center-Shadyside
Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Neurology, Department of Pediatrics, Division of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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