Pediatric Guillain-Barre Syndrome Treatment & Management

  • Author: Marc P DiFazio, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Jan 26, 2012
 

Approach Considerations

To date, treatment for Guillain-Barré syndrome (GBS) has been aimed primarily at immunomodulation.

In pediatrics, the most effective form of therapy is intravenous immunoglobulin (IVIG). Each batch of IVIG is made of human plasma derived from pools of 3,000-10,000 donors. Plasmapheresis may also be used.

Corticosteroids were previously used to treat GBS, but current data indicate they provide little benefit.

Go to Guillain-Barre Syndrome and Emergent Management of Guillain-Barre Syndrome for complete information on these topics.

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Intravenous Immune Globulin

IVIG has been shown to be safe and effective in the treatment of pediatric GBS.[34, 35] Although only one prospective, randomized treatment trial in childhood GBS has been published,[36] multiple studies have shown that IVIG 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. 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. Although, in a small, randomized trial, the outcomes between the 2 treatment regimens were equivalent, treatment-related fluctuation (deterioration after receiving IVIG) occurred more often in children who received the 2-day course of IVIG.[36]

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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.

The availability of plasmapheresis is generally limited to major referral centers that have the requisite equipment and trained personnel.[37] Central line vascular access dictates intensive care hospitalization. In addition, plasmapheresis is limited to larger children; in most institutions, children weighing less than 10-15 kg may not be considered for volume exchange therapy. These features distinguish plasmapheresis from IVIG, which can be given to smaller children and can be administered via peripheral IV in specialized ambulatory clinic settings, advanced home nursing programs, and at ward level hospital settings.

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Exchange Transfusion

When high cost limits the use of IVIG or unavailability limits the use of plasmapheresis, exchange transfusion can be used as an alternative therapy for severe disease in children.[38]

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Complications of Monitoring and Treatment

Careful attention should be paid to multiple issues that may require intervention and specialist consultation. Temperature, blood pressure, heart rate, respiratory capacity, and urine output of the patient should be monitored. In pediatric patients, monitoring for dysautonomia is paramount.

Among the concerns of Guillain-Barré syndrome (GBS) comorbidities are cardiorespiratory function, nutrition, urinary retention, decubitus ulcers, constipation, gastritis, dysesthesias/pain, mood and anxiety issues, iatrogenic infectious complications, and contractures in patients who are severely ill or who have a particularly prolonged course.

During the acute phase of the illness, orthostatic hypotension and urinary retention also may cause significant problems. In addition to the weakness, autonomic symptoms (eg, orthostatic hypotension) may also restrict activity and should be monitored.

Respiratory status and signs of dysautonomia

During the acute phase of the disease, close attention should be paid to respiratory status and signs of dysautonomia. Intubation and mechanical ventilation should be considered when vital capacity falls below 15 mL/kg body weight or arterial pressure of oxygen falls below 70 mm Hg (or the patient has significant fatigue).

In cooperative children older than 5 years, respiratory function measurements, such as vital capacity or maximal inspiratory force (MIF), can be valuable. MIFs are also known as negative inspiratory force (NIF). MIFs are normally greater than -40 mL water pressure; thus, the more negative, the better MIF. MIFs less than -20 mL water pressure can be an indication of poor inspiratory ability and respiratory distress.

MIFs provide objective data to follow and compare. This measure is unfortunately difficult to monitor in young children (< 5 y) and in uncooperative children. Experienced pediatric respiratory therapists can be very valuable in these measures.

Experienced pulmonary care is vital if neuromuscular weakness is affecting pulmonary function. Possible interventions include continuous positive airway pressure (CPAP), bilateral positive airway pressure (BiPAP), mechanical ventilation, or cough-assist devices.

Blood gases are not helpful in assessing neuromuscular respiratory failure, as they do not become abnormal until there is no longer any respiratory reserve. Other means of assessing respiratory function, such as respiratory rate and dyspnea on lying supine, are far more valuable early indictors of respiratory dysfunction.

Chest radiographs can be obtained to look for signs of infection. Cardiac monitoring is essential to detect any signs of cardiovascular instability and treat any arrhythmia.

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Consultations

Consultation with a pediatric neurologist should be considered to confirm the diagnosis. Intensivists may need to be involved quickly if critical care (cardiorespiratory) issues are suspected.

Once the patient’s condition has stabilized, patients should benefit from consultation with a rehabilitation medicine specialist, especially if it appears that recovery will be prolonged. Physical therapy, occupational therapy, and orthotics are also helpful.

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Contributor Information and Disclosures
Author

Marc P DiFazio, MD  Associate Professor, Department of Neurology, Uniformed Services University of the Health Sciences; Director, Pediatric Subspecialty Services, Shady Grove Adventist Hospital for Children

Marc P DiFazio, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Cerebral Palsy and Developmental Medicine, American Academy of Neurology, Child Neurology Society, and Movement Disorders Society

Disclosure: Nothing to disclose.

Coauthor(s)

Nitin C Patel, MD, MPH  Professor of Clinical Neurology and Child Health, Department of Child Health, Chief for Developmental Pediatrics and Child Neurology, Specialist in Pediatrics/Neurology, University of Missouri Hospital and Clinics at Columbia

Nitin C Patel, MD, MPH is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Mita N Patel  University of Missouri-Columbia School of Medicine

Disclosure: Nothing to disclose.

Sameer Chhibber, MD, FRCPC  Neuromuscular Fellow, Department of Neurology, Brigham and Women's Hospital and Massachusetts General Hospital, Harvard Medical School

Disclosure: Nothing to disclose.

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.

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center

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.

Additional Contributors

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.

Jennifer A Markowitz, MD Attending Physician, Department of Neurology, Children's Hospital Boston

Jennifer A Markowitz, MD is a member of the following medical societies: Child Neurology Society

Disclosure: Nothing to disclose.

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 School of Medicine; 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.

Sarah Sheikh, MBBCh, MSc, MRCP Neuromuscular Fellow, Department of Neurology, Brigham and Women's Hospital

Sarah Sheikh, MBBCh, MSc, MRCP is a member of the following medical societies: American Academy of Neurology, Massachusetts Medical Society, and Royal College of Physicians of the UK

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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