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Guillain-Barré syndrome in children: Treatment and prognosis

Author: Monique M Ryan, FRACP


Section Editors: Douglas R Nordli, Jr, MD, Adrienne G Randolph, MD, MSc, Jeremy M Shefner, MD, PhD
Deputy Editor: John F Dashe, MD, PhD

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Dec 2017. | This topic last updated: Sep 25, 2017.

INTRODUCTION — The acute immune-mediated polyneuropathies are classified under the eponym Guillain-
Barré syndrome (GBS). Typically, GBS presents as an acute monophasic paralyzing illness provoked by a
preceding infection. The main modalities of therapy for GBS are intravenous immune globulin and plasma
exchange. Even before initiating therapy, common questions that arise include whether the patient needs
intensive care, assisted ventilation, or other supportive measures.

This topic will discuss the management and prognosis of GBS in children. Other aspects of GBS in children are
discussed separately. (See "Guillain-Barré syndrome: Pathogenesis" and "Guillain-Barré syndrome in children:
Epidemiology, clinical features, and diagnosis".)

CLINICAL FEATURES AND DIAGNOSIS — The clinical features and diagnosis of GBS are reviewed here
briefly and discussed in detail separately. (See "Guillain-Barré syndrome in children: Epidemiology, clinical
features, and diagnosis".)

Acute inflammatory demyelinating polyneuropathy (AIDP) is the prototype of GBS, and is the most common form
in North America, Europe and most of the developed world. Acute motor axonal neuropathy (AMAN) is a pure
motor form of GBS. It occurs mainly in northern China, but is also a common variant of GBS in other locations,
including Japan, Mexico, and South America. The clinical presentation is similar in the two types. In most cases,
neurologic symptoms develop two to four weeks after what initially appears to be a benign febrile respiratory or
gastrointestinal infection. The predominant symptoms of GBS at presentation in children are pain and gait
difficulty. Lower extremity symmetric weakness may ascend over hours to days to involve the arms, and the
muscles of respiration in severe cases. In those with cranial neuropathy, the facial nerve is most commonly
affected, resulting in bilateral facial weakness. Autonomic dysfunction occurs in approximately one-half of
children with GBS. Physical examination reveals symmetric weakness with diminished or absent reflexes. Most
patients reach the nadir of their function within two to four weeks, followed by a slow return of function over the
course of weeks to months. (See "Guillain-Barré syndrome in children: Epidemiology, clinical features, and
diagnosis", section on 'Clinical features'.)

Electrophysiologic studies are the most specific and sensitive tests for diagnosis of GBS. Characteristic findings
from lumbar puncture include a normal opening pressure and cerebrospinal fluid with fewer than 10 cells
(typically mononuclear) and an elevated protein concentration (>45 mg/dL). Spinal MRI with administration of
gadolinium frequently shows enhancement of the spinal nerve roots and cauda equina during the first weeks
after the onset of symptoms. (See "Guillain-Barré syndrome in children: Epidemiology, clinical features, and
diagnosis", section on 'Diagnosis'.)

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MONITORING AND SUPPORTIVE CARE — During the initial phase of GBS, all patients require close
monitoring of motor, autonomic (ie, blood pressure, heart rate and sphincter function), and respiratory function.
Serial pulmonary function testing should be performed routinely. In most cases, pulmonary function monitoring
(ie, vital capacity and maximum inspiratory pressure) should be performed every four hours at the bedside. All
children, particularly those who are too young to cooperate with pulmonary testing, should be closely monitored
and observed for fatigue and other clinical signs of impending respiratory muscle failure, as discussed below.

Patients should be electively intubated if clinical evaluation or pulmonary function tests suggest impending
respiratory failure. Vigilance is essential since respiratory deterioration can occur rapidly. (See 'Need for assisted
ventilation' below.)

Mortality is often due to complications such as nosocomial infection, acute respiratory arrest, deep venous
thrombosis with pulmonary embolism, and pneumothorax. Outcomes can be optimized with meticulous care in
the intensive care unit to identify patients progressing to respiratory failure early and to prevent hospital-acquired
complications.

Need for intensive care — Children with any of the following problems should be admitted urgently to a
pediatric intensive care unit [1]:

● Flaccid quadriparesis

● Rapidly progressive weakness

● Reduced vital capacity (≤20 mL/kg)

● Bulbar palsy

● Significant autonomic instability

Less severely affected patients can be managed in intermediate care units if available, and mildly affected
patients whose trajectory is stable can be managed on the general ward, preferably with cardiac telemetry, along
with monitoring of blood pressure and vital capacity every four hours. Hospitalization is continued until the child's
condition has clearly stabilized.

Need for assisted ventilation — Approximately 10 to 20 percent of children with GBS require mechanical
ventilation for respiratory failure [2,3]. The need for tracheal intubation should be anticipated so that it can be
performed as an elective procedure [4].

Children with a vital capacity approximately one-half the normal value for age or ≤20 mL/kg of body weight
generally progress to require ventilatory support. In a study of patients with GBS that included some children,
serial measurements of pulmonary function were most helpful for detecting the risk of developing respiratory
failure [5]. Progression to requiring mechanical ventilation was more likely in patients with rapidly increasing
weakness, bulbar dysfunction, bilateral facial weakness, or dysautonomia.

The following parameters warn of impending respiratory arrest and are an indication for urgent intubation [5]:

● Vital capacity ≤20 mL/kg

● Maximum inspiratory pressure less negative than -30 cmH2O (ie, between -30 and 0 cmH2O)

● Maximum expiratory pressure ≤40 cmH2O

● Tidal volume <5 mL/kg

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Pulmonary function testing is difficult in children who cannot cooperate, typically those younger than six years of
age. These patients should be closely monitored and observed for fatigue and other clinical signs of impending
respiratory muscle failure. These signs include the following:

● A sustained increase of pCO2 to ≥50 mmHg (normally 35 to 40 mmHg)

● An increasing respiratory rate

● Increasing oxygen requirement and increasing alveolar to arterial oxygen difference (normally 5 to 10
mmHg)

● An increased use of accessory muscles (eg, sternocleidomastoid use, flaring of the alae nasi, intercostal
retractions) and decreased or paradoxical diaphragm movements; these reflect decreased chest wall
movement and low lung volumes

● Sweating about the head and neck, wide pulse pressure, and bounding pulses; these portend respiratory
failure with CO2 retention

Children have less metabolic and muscle reserve than adults. They can deteriorate quite rapidly and become
apneic or develop alveolar hypoventilation "right under your nose." Generally, it is wise to have a pediatric critical
care specialist involved early in the clinical course.

Sedation and neuromuscular blockade should be avoided in ventilated patients because they obscure the course
of the illness. Providing scrupulous airway care and chest physiotherapy reduces the risk of pneumonia.
Tracheostomy may be needed if prolonged ventilation is required.

Autonomic dysfunction — Autonomic dysfunction is a well-recognized feature of GBS and is a significant


source of mortality [4,6]. Consequently, close monitoring of blood pressure, fluid status, and cardiac rhythm are
essential to the management of these patients. Care must also be taken when vasoactive or sedative drugs are
used, because GBS-related dysautonomia may exaggerate the hypotensive responses to these drugs.

Other supportive measures — Nutritional needs should be addressed early in the disease course. Orogastric
tube feeding, gastrostomy, or parental nutrition is often necessary.

The patient's position should be changed frequently for comfort and to avoid skin breakdown. Intermittent
pressure leg boots are used in the intensive care setting to prevent deep vein thrombosis.

Physical therapy, occupational therapy, and social services should be involved early. Providing the patient with a
method of communication is important if normal speech is not possible. Pencil and paper, a "magic slate," or a
communication board can be given to those old enough to write.

IMMUNOTHERAPIES — The main modalities of therapy for GBS are intravenous immune globulin (IVIG) and
plasma exchange (also called plasmapheresis).

While acknowledging the limitations of data for children, we suggest treatment with IVIG or plasma exchange for
children with severe GBS, in general agreement with guidelines from the American Academy of Neurology (AAN)
[7,8]. IVIG is preferred to plasma exchange in children because of the relative safety and ease of administration,
although there are no reliable data suggesting that one or the other is superior.

IVIG and plasma exchange for children with GBS should be reserved for those with any of the following
indications:

● Progressing weakness

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● Worsening respiratory status or need for mechanical ventilation

● Significant bulbar weakness

● Inability to walk unaided

IVIG and plasma exchange are not recommended for ambulatory children with GBS who have mild,
nonprogressive disease or for children whose symptoms have stabilized. Children who have rapid progression
followed by stabilization of symptoms within the first or second week of GBS onset may still be considered
candidates for treatment by some child neurologists.

Time to treatment onset may be important in children although data are lacking. In adults, IVIG is recommended
by the AAN for patients who are unable to walk unaided if treatment is started within two or possibly four weeks
of onset. Similarly, plasma exchange is recommended for adult patients who are unable to walk unaided if
treatment is started within four weeks of symptom onset [7].

Glucocorticoids are not beneficial for GBS and have no role in its treatment [9,10].

Intravenous immune globulin — Reports of the use of intravenous immune globulin (IVIG) in children with
GBS are limited, and no large randomized controlled trials exist. Nevertheless, data from the available small
open-label randomized trials in children suggest that IVIG shortens the time to recovery compared with
supportive care alone [11-13]. Similarly, most observational studies show that IVIG hastens recovery in children
[14-18]. While these trials and studies in children have not proven that IVIG leads to improvement in overall
prognosis, their results are consistent with the larger randomized trials showing a beneficial effect of IVIG
treatment for GBS in adults. (See "Guillain-Barré syndrome in adults: Treatment and prognosis".)

The mechanism of improvement with IVIG is uncertain, but it is thought to involve suppression of inflammatory
and immune-mediated processes. This is discussed separately. (See "Overview of intravenous immune globulin
(IVIG) therapy", section on 'Suppression of inflammatory/autoimmune processes'.)

Methodologic differences between reports make it difficult to generalize about when children with GBS begin to
improve after IVIG treatment. Nevertheless, muscle strength generally begins to improve in most children within
14 days after initiation of IVIG therapy and most are walking within three months [16-18]. Early transient relapse
after IVIG treatment has been reported [12,19-21].

The total dose of IVIG for the treatment of GBS in children is 2 g/kg, given as 1 g/kg for two days or 400 mg/kg
for five days. This dose is empiric and is based upon treatment of patients with immune deficiency disorders. The
dosage schedule is also empiric as little is known of the pharmacokinetics of serum IgG [22]. Most centers give a
total of 2 g/kg of IVIG over two days, although one small trial suggested a slightly greater risk of relapse with this
regimen as opposed to a five-day course [12].

Typically, patients are given only a single course of IVIG treatment. One study in adults found that a second
course of IVIG treatment may benefit patients who have a limited response to the initial course [23]. Variations in
treatment response may relate to the wide variation in serum IgG levels achieved after standard doses of IVIG
[22]. A study investigating the value of additional doses of IVIG in severe or treatment-refractory adult GBS is
now underway [24]. In practice many pediatric centers would consider an additional course of IVIG or plasma
exchange in severe cases where no recovery is seen within two weeks of presentation, although a prolonged
recovery is to be expected in axonal GBS and there is very limited evidence supporting this approach [23].

Complications and side effects of IVIG treatment are those related to transfusion reactions and are discussed
elsewhere. (See "Immunologic transfusion reactions".)

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IVIG is preferred to plasma exchange in children because of the relative safety and ease of administration,
although it has not been shown to have better results.

Plasma exchange — Plasma exchange is recommended for those patients who have rapidly progressing
weakness, worsening respiratory status, are unable to walk unaided, require mechanical ventilation, or have
significant bulbar weakness. As a result of the cost, risk, and discomfort to the patient, plasma exchange
generally is not used for ambulatory patients with mild disease or for patients whose symptoms have stabilized.

Large, multicenter trials have established the effectiveness of plasma exchange in adult patients with severe
GBS. The mechanism is thought to be removal of antibodies directed against nerves from the circulation.
Increased muscle strength, earlier improvement, and a lower requirement for mechanical ventilation have been
demonstrated, as discussed separately. (See "Guillain-Barré syndrome in adults: Treatment and prognosis",
section on 'Plasma exchange'.)

Experience with plasma exchange in children with GBS is limited. In an updated (2012) meta-analysis of six trials
and 649 patients with GBS that included a small number of children who were all ≥10 years of age, treatment
with plasma exchange was superior to supportive care [25]. Even patients with mild disease treated with two
exchanges had improved outcomes when compared with controls who did not undergo plasma exchange.
Plasma exchange was most effective when started within seven days of symptom onset. Compared with
supportive care, patients treated with plasma exchange had a slightly higher risk of relapse in the first year after
treatment but nevertheless were more likely at one year to have full recovery of muscle strength.

In general, results of plasma exchange treatment in pediatric patients appear to be similar to those in adults, with
a shortened disease course and reduced incidence of respiratory failure. The main benefit seems to be a
decrease in the interval from maximum weakness to recovery of independent walking [26].

Plasma exchange generally is considered most effective if begun acutely, but may still be beneficial if started
within four weeks of initial symptoms. Approximately 10 percent of patients relapse within 10 days after
treatment.

Plasma exchange requires special equipment and trained personnel and can be performed only at centers with
expertise in the treatment of children. Because of technical considerations, this procedure is more challenging in
children younger than two years of age. The procedure usually consists of four to six double-volume exchanges
performed on alternate days over one week. Immunoglobulin levels may be decreased by 30 to 40 percent after
plasma exchange.

The frequency and types of complications from therapeutic apheresis depend on the overall condition of the
patient, the number of plasma exchanges, the replacement fluid, and the venous access device. For all
therapeutic apheresis, patients may be at risk for citrate-induced hypocalcemia, metabolic alkalosis, or
complications related to the vascular catheter. Potential early symptoms of hypocalcemia include perioral and
distal extremity paresthesias or numbness; severe reactions can include tetany, prolongation of the QT interval,
arrhythmias, or hypotension. (See "Therapeutic apheresis (plasma exchange or cytapheresis): Complications".)

Younger children most often require placement of a central venous catheter. Potential complications of central
venous catheters include infection, pain, nerve damage, thrombosis, perforation, dissecting hematomas, air
embolism, or arteriovenous fistulas. Many of these complications can also occur if peripheral veins are used.

IVIG versus plasma exchange — There are few comparative trials of plasma exchange and intravenous
immune globulin (IVIG) in childhood GBS. In a randomized trial from Egypt that enrolled 41 children with very
severe GBS necessitating ventilatory support, there was no significant difference between treatment with plasma
exchange versus IVIG for length of stay in the pediatric intensive care unit or for ability to walk unaided at four
weeks after discharge [27]. However, the duration of mechanical ventilation was significantly shorter for the
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group treated with plasma exchange (median 11 days, versus 13 days for IVIG). This finding requires
confirmation in larger, more rigorous trials.

PROGNOSIS — In general, the prognosis of GBS in children is better than in adults [28-32]. In various reports,
the following observations were noted:

● Mortality was 3 to 4 percent, and usually was secondary to respiratory failure or cardiac complications [2,28].

● An excellent long-term recovery (eg, symptom-free or no disability despite residual symptoms) was
observed in 85 to 92 percent of children [10,33,34].

● Approximately 88 percent or more of children were ambulatory within six months after onset, and nearly all
walked within one year [10,34-36].

● In a study of 52 children with a median follow-up of 11 years, sequelae of GBS were reported by 65 percent
of patients; the most common residual complaints were paresthesia, unsteady gait in the dark, painful hands
or feet, and severe fatigue. Long-term neurologic deficits included sensory loss, limb weakness, and
areflexia in 14, 8, and 5 percent, respectively [34].

● Early relapses occur in about 4 percent of cases of childhood GBS, and are generally responsive to
immunomodulatory treatment [29]. Occasionally children go on to have recurrent weakness and are
ultimately diagnosed with chronic inflammatory demyelinating polyneuropathy [28,29]. (See "Chronic
inflammatory demyelinating polyneuropathy: Etiology, clinical features, and diagnosis".)

● Recurrence of GBS was reported in 2 to 5 percent [34].

Evidence is limited, but outcomes may be less favorable in the following groups [28,32,36-39]:

● Very young (<2 years)

● Very weak at presentation

● Cranial nerves involved

● Quadriparetic on day 10

● Require ventilator support

● Have inexcitable motor nerves on nerve conduction studies

The long-term outcome of GBS does not appear to differ substantially among the GBS subtypes (see "Guillain-
Barré syndrome in children: Epidemiology, clinical features, and diagnosis", section on 'Subtypes of Guillain-
Barré syndrome') [34,35,40]. In an observational study of Japanese children with GBS, outcomes were generally
favorable in both acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy
(AMAN) subtypes, although delayed recovery was more frequent with AMAN [35]. All 11 children with AIDP and
12 of 15 (80 percent) with AMAN regained the ability to walk independently at six months; 14 of 15 children (93
percent) with AMAN walked independently at two years. Differences in clinical recovery between children with
AIDP and AMAN were not statistically significant.

SUMMARY AND RECOMMENDATIONS

● During the initial phase of Guillain-Barré syndrome (GBS), all patients require close monitoring of motor,
autonomic (ie, blood pressure, heart rate and sphincter function), and respiratory function. In most cases,
vital capacity and maximum inspiratory pressure should be assessed every four hours at the bedside. All

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children, particularly those too young to cooperate with pulmonary testing, should be closely monitored and
observed for fatigue and other clinical signs of impending respiratory muscle failure. (See 'Monitoring and
supportive care' above.)

● Children with GBS who have flaccid quadriparesis, rapidly progressive weakness, reduced vital capacity
(≤20 mL/kg), bulbar palsy, or autonomic instability should be admitted emergently to a pediatric intensive
care unit. (See 'Need for intensive care' above.)

● Approximately 10 to 20 percent of children with GBS require mechanical ventilation for respiratory failure.
Parameters that warn of impending respiratory arrest and are an indication for elective intubation include a
vital capacity ≤20 mL/kg, a maximum inspiratory pressure less negative than -30 cmH2O (ie, between -30
and 0 cmH2O), a maximum expiratory pressure ≤40 cmH2O, or a tidal volume <5 mL/kg. Clinical signs of
impending respiratory muscle failure include a sustained increase of pCO2 to ≥50 mmHg, increasing
respiratory rate, an increased use of accessory muscles and decreased or paradoxical diaphragm
movements, sweating about the head and neck, wide pulse pressure, and bounding pulses. (See 'Need for
assisted ventilation' above.)

● Autonomic dysfunction is a well-recognized feature of GBS and is a significant source of mortality.


Therefore, management requires continued close monitoring of blood pressure, fluid status, and cardiac
rhythm. (See 'Autonomic dysfunction' above.)

● We suggest treatment with intravenous immune globulin (IVIG) or plasma exchange for children with severe
GBS (Grade 1B), as defined by the presence of one or more of the following characteristics (see
'Immunotherapies' above):

• Progressing weakness

• Worsening respiratory status or need for mechanical ventilation

• Significant bulbar weakness

• Inability to walk unaided

We prefer IVIG to plasma exchange for the treatment of children because of its relative safety and ease of
administration, although it has not been shown to have better results. Neither treatment is recommended for
ambulatory children with GBS who have mild disease or for children whose symptoms have stabilized.
However, children who have rapid progression followed by stabilization of symptoms within the first or
second week of GBS onset may still be considered candidates for treatment. (See 'Immunotherapies'
above.)

● Data regarding the prognosis of GBS in children are limited, but suggest a mortality rate of 3 to 4 percent,
and an excellent long-term recovery (eg, symptom-free or no disability despite residual symptoms) in 90
percent or more of children. (See 'Prognosis' above.)

ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Robert P Cruse, DO, who
contributed to earlier versions of this topic review.

Use of UpToDate is subject to the Subscription and License Agreement.

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Topic 6204 Version 15.0

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Contributor Disclosures
Monique M Ryan, FRACP Nothing to disclose Douglas R Nordli, Jr, MD Nothing to disclose Adrienne G
Randolph, MD, MSc Grant/Research/Clinical Trial Support: Genentech [lipid biomarkers in influenza critical
illness]. Consultant/Advisory Boards: Asahi Kasei Pharma [Sepsis with coagulopathy (ART-13)]; Ferring, Inc
[Septic shock (Selepressin)]; Therabron, Inc [Acute respiratory failure in children (Recombinant clara cell
protein)]; Genentech [recombinant antibody to Influenza A]; La Jolla Pharmaceuticals [Angiotensin 2 in pediatric
refractory shock]. Jeremy M Shefner, MD, PhD Grant/Research Support: Biogen Idec; Cytokinetics; Neuraltus
[ALS]; Avanir [ALS (Nuedexta)]. Consultant/Advisory Boards: Biogen Idec; Cytokinetics; Ionis; Neuraltus;
Mitsubishi Tanabe [ALS]; Edaravone [ALS (MT Pharma)]. John F Dashe, MD, PhD Nothing to disclose

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform to
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