This is the current release of the guideline.

Guillain-Barre syndrome

Management
Treatment

Neurology

Physicians

To provide an evidence-based statement to guide physicians in the management of Guillain-Barre syndrome (GBS)

Adults and children with Guillain-Barre syndrome (GBS)

Immunotherapy:

1. Plasma exchange (PE)
2. Intravenous immunoglobulin (IVIg)

Note: The guideline developers considered, but did not recommend the following treatments:

• Combination treatments
• Steroids

Note: The guideline developer considered the following treatment but found insufficient evidence to recommend its use:

• Immunoabsorption

• Recovery from Guillain-Barre syndrome (GBS), measured using a disability scale
• Cost-effectiveness
• Adverse effects, measured using relative risk

Hand-searches of Published Literature (Primary Sources)
Hand-searches of Published Literature (Secondary Sources)
Searches of Electronic Databases

A search of MEDLINE from 1966 and of the Cochrane library was performed in March 2002. "Polyradiculoneuritis" was limited by "human" and cross-referenced with "therapy." The search results were reviewed for each question by at least two members of the practice parameter group and supplemented from the reference lists in the articles retrieved and the personal reference lists of the members of the practice parameter group.

Not stated

Weighting According to a Rating Scheme (Scheme Given)

Class of Evidence for Therapy

Class I: High quality randomized controlled trials (RCTs).

Class II: Prospective matched group cohort studies or randomized controlled trials lacking adequate randomization concealment or blinding or potentially liable to attrition or outcome ascertainment bias.

Class III: Other studies such as natural history studies.

Class IV: Uncontrolled studies, case series, or expert opinion.

Review of Published Meta-Analyses
Systematic Review

Not stated

Not stated

Strength of Recommendations

A = established as effective, ineffective, or harmful or as useful/predictive or not useful/predictive.

B = probably useful/predictive or not useful/predictive for the given condition in the specified population.

C = possibly effective, ineffective, or harmful or as useful/predictive or not useful/predictive.

U = data inadequate or conflicting. Treatment, test or predictor unproven.

The costs in the United States related to Guillain-Barre syndrome (GBS) have been estimated as $110,000 for direct health care and $360,000 in lost productivity per patient.

In one study comparing plasma exchange (PE) with supportive therapy in Scandinavia, the cost of plasma exchange was more than offset by the savings in health care costs as a result of shorter hospital stay. Similar conclusions have been reached in the United Kingdom. For patients with moderately severe Guillain-Barre syndrome, one study calculated that four plasma exchanges are more cost-effective than two.

External Peer Review
Internal Peer Review

Draft guidelines were reviewed for accuracy, quality, and thoroughness by the American Academy of Neurology (AAN) members, topic experts, and pertinent physician organizations.

Final guidelines were approved by the Quality Standards Subcommittee on November 9, 2002, the Practice Committee on April 2, 2003, and the American Academy of Neurology Board of Directors June 20, 2003. They were published in Neurology 2003; 61:736-740.

Definitions of the ratings of recommendations (A, B, C, U) and the class of evidence for therapy (Class I-IV) are provided at the end of the "Major Recommendations" field.

Does initial immunotherapy hasten recovery?

Plasma exchange (PE)

1. PE is recommended in nonambulant patients within 4 weeks of onset (Level A recommendation, Class II evidence) and for ambulant patients within 2 weeks of onset (Level B recommendation, limited Class II evidence).
2. The effects of PE and intravenous immunoglobulin (IVIg) are equivalent (see below).
3. There is insufficient evidence to recommend the use of cerebrospinal fluid (CSF) filtration (Level U recommendation, limited Class II evidence).

Immunoabsorption

1. The evidence is insufficient to recommend the use of immunoabsorption (Level U recommendation, Class IV evidence).

Intravenous immunoglobulin (IVIg)

1. IVIg is recommended for patients with Guillain-Barre syndrome (GBS) who require aid to walk within 2 (Level A recommendation) or 4 weeks from the onset of neuropathic symptoms (Level B recommendation derived from Class II evidence concerning PE started within the first 4 weeks and Class I evidence concerning the comparisons between PE and IVIg started within the first 2 weeks).
2. The effects of IVIg and PE are equivalent.

Combination treatments

1. Sequential treatment with PE followed by IVIg (Level A recommendation, Class I evidence) or immunoabsorption followed by IVIg (Level U recommendation, Class IV evidence) is not recommended.

Steroids

1. Corticosteroids are not recommended for the treatment of patients with GBS (Level A recommendation, Class I evidence).

Are there special issues in the management of children with GBS?

1. PE or IVIg are treatment options for children with severe GBS (Level B recommendation derived from Class II evidence in adults).

Definitions:

Class of Evidence for Therapy

Class I: High quality randomized controlled trials (RCTs).

Class II: Prospective matched group cohort studies or randomized controlled trials lacking adequate randomization concealment or blinding or potentially liable to attrition or outcome ascertainment bias.

Class III: Other studies such as natural history studies.

Class IV: Uncontrolled studies, case series or expert opinion.

Strength of the Recommendations

A = established as effective, ineffective, or harmful or as useful/predictive or not useful/predictive.

B = probably useful/predictive or not useful/predictive for the given condition in the specified population.

C = possibly effective, ineffective, or harmful or as useful/predictive or not useful/predictive.

U = data inadequate or conflicting. Treatment, test, or predictor unproven.

None provided

The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations").

• These guidelines may assist physicians in making appropriate clinical decisions regarding immunotherapy for patients with Guillain-Barre syndrome.
• Evidence suggests that plasma exchange (PE) hastens recovery in nonambulant patients with Guillain-Barre syndrome who seek treatment within 4 weeks of the onset of neuropathic symptoms. Plasma exchange also hastens recovery in ambulant patients who are examined within 2 weeks, but the evidence is limited to one trial.
• When started within 2 weeks from the onset, intravenous immunoglobulin (IVIg) has equivalent efficacy to plasma exchange in hastening recovery for patients with Guillain-Barre syndrome who require aid to walk.

Adverse events from therapy

In a Dutch trial, pneumonia, atelectasis, thrombosis, and hemodynamic difficulties occurred more often with plasma exchange (PE) than with intravenous immunoglobulin (IVIg). Sixteen out of 73 patients (22%) had multiple complications with plasma exchange compared with 5 of 74 (7%) with intravenous immunoglobulin. In the largest trial, adverse events occurred in 8 of 121 patients (7%) in the plasma exchange group (hypotension, septicemia, pneumonia, malaise, abnormal clotting, and hypocalcaemia) and in 6 of 130 (5%) patients in the intravenous immunoglobulin group (vomiting, meningism, renal failure, myocardial infarction, and infusion site erythema).

This statement is provided as an educational service of the American Academy of Neurology. It is based on an assessment of current scientific and clinical information. It is not intended to include all possible, proper methods of care for a particular neurologic problem or all legitimate criteria for choosing to use a specific procedure. Neither is it intended to exclude any reasonable alternative methodologies. The American Academy of Neurology recognizes that specific patient-care decisions are the prerogative of the patient and the physician caring for the patient, based on all of the circumstances involved.

An implementation strategy was not provided.

Getting Better
Living with Illness

Effectiveness
Timeliness

Not applicable: The guideline was not adapted from another source.

2003 Sep 23

American Academy of Neurology - Medical Specialty Society

American Academy of Neurology (AAN)

Quality Standards Subcommittee of the American Academy of Neurology

Quality Standards Subcommittee Members: Gary Franklin, MD, MPH (Co-Chair); Catherine Zahn, MD (Co-Chair); Milton Alter, MD, PhD; Stephen Ashwal, MD; Richard M. Dubinsky, MD; Jacqueline French, MD; Michael Glantz, MD; Gary Gronseth, MD; Deborah Hirtz, MD; Robert G. Miller, MD; James Stevens, MD; and William J. Weiner, MD

Not stated

This is the current release of the guideline.

This summary was completed by ECRI on February 12, 2004.

This NGC summary is based on the original guideline, which is copyrighted by the American Academy of Neurology.