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## Complete Summary

#### GUIDELINE TITLE

Practice parameter: immunotherapy for Guillain-Barre syndrome: report of
the Quality Standards Subcommittee of the American Academy of Neurology.

#### BIBLIOGRAPHIC SOURCE(S)

Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF,
Meythaler JM, Miller RG, Sladky JT, Stevens JC. Practice parameter:
immunotherapy for Guillain-Barre syndrome: report of the Quality
Standards Subcommittee of the American Academy of Neurology.
Neurology 2003 Sep 23;61(6):736-40. \[46 references\]
[PubMed](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=14504313)

#### GUIDELINE STATUS

This is the current release of the guideline.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Guillain-Barre syndrome

#### GUIDELINE CATEGORY

Management\
Treatment

#### CLINICAL SPECIALTY

Neurology

#### INTENDED USERS

Physicians

#### GUIDELINE OBJECTIVE(S)

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

#### TARGET POPULATION

Adults and children with Guillain-Barre syndrome (GBS)

#### INTERVENTIONS AND PRACTICES CONSIDERED

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

#### MAJOR OUTCOMES CONSIDERED

-   Recovery from Guillain-Barre syndrome (GBS), measured using a
    disability scale

-   Cost-effectiveness

-   Adverse effects, measured using relative risk

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

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

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

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.

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

**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.

#### METHODS USED TO ANALYZE THE EVIDENCE

Review of Published Meta-Analyses\
Systematic Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not stated

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Not stated

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

**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.

#### COST ANALYSIS

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.

#### METHOD OF GUIDELINE VALIDATION

External Peer Review\
Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

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.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

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?]{.underline}**

**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?]{.underline}**

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

**[Definitions]{.underline}**:

**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.

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

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

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

-   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.

#### POTENTIAL HARMS

**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).

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

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.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Patient Resources\
Quick Reference Guides/Physician Guides\
Slide Presentation

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness\
Timeliness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF,
Meythaler JM, Miller RG, Sladky JT, Stevens JC. Practice parameter:
immunotherapy for Guillain-Barre syndrome: report of the Quality
Standards Subcommittee of the American Academy of Neurology.
Neurology 2003 Sep 23;61(6):736-40. \[46 references\]
[PubMed](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=14504313)

#### ADAPTATION

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

#### DATE RELEASED

2003 Sep 23

#### GUIDELINE DEVELOPER(S)

American Academy of Neurology - Medical Specialty Society

#### SOURCE(S) OF FUNDING

American Academy of Neurology (AAN)

#### GUIDELINE COMMITTEE

Quality Standards Subcommittee of the American Academy of Neurology

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*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

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: A list of American Academy of Neurology (AAN)
guidelines, along with a link to a Portable Document Format (PDF) file
for this guideline, is available at the [AAN Web
site](http://aan.com/go/practice/guidelines).

Print copies: Available from the AAN Member Services Center, (800)
879-1960, or from AAN, 1080 Montreal Avenue, St. Paul, MN 55116.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following is available:

-   AAN guideline summary for clinicians: immunotherapy for
    Guillain-Barre syndrome. St. Paul (MN): American Academy of
    Neurology, 2003 Sep. Electronic copies: Available from the [American
    Academy of Neurology (AAN) Web
    site](http://www.aan.com/professionals/practice/pdfs/gbs_guide_aan_mem.pdf).
    See the related QualityTool summary on the [Health Care Innovations
    Exchange Web site](http://innovations.ahrq.gov/content.aspx?id=982).

-   Practice parameter: immunotherapy for Guillain-Barre syndrome slide
    presentation. St. Paul (MN): American Academy of Neurology, 2003
    Sep. Electronic copies: Available from the [AAN Web
    site](http://www.aan.com/professionals/practice/guidelines/pwr_pnt/GBS_Edu_tool_V3.ppt).
    See the related QualityTool summary on the [Health Care Innovations
    Exchange Web site](http://innovations.ahrq.gov/content.aspx?id=977)

-   AAN guideline development process \[online\]. St. Paul (MN):
    American Academy of Neurology. Electronic copies: Available from the
    [American Academy of Neurology Web
    site](http://aan.com/go/practice/guidelines/development).

#### PATIENT RESOURCES

The following is available:

-   AAN guideline summary for patients and their families: treatment for
    Guillain-Barre syndrome. St. Paul (MN): American Academy of
    Neurology, 2003 Sep. Electronic copies: Available from the [American
    Academy of Neurology (AAN) Web
    site](http://www.aan.com/professionals/practice/pdfs/gbs_guide_pat_care.pdf).
    See the related QualityTool summary on the [Health Care Innovations
    Exchange Web site](http://innovations.ahrq.gov/content.aspx?id=980).

Please note: This patient information is intended to provide health
professionals with information to share with their patients to help them
better understand their health and their diagnosed disorders. By
providing access to this patient information, it is not the intention of
NGC to provide specific medical advice for particular patients. Rather
we urge patients and their representatives to review this material and
then to consult with a licensed health professional for evaluation of
treatment options suitable for them as well as for diagnosis and answers
to their personal medical questions. This patient information has been
derived and prepared from a guideline for health care professionals
included on NGC by the authors or publishers of that original guideline.
The patient information is not reviewed by NGC to establish whether or
not it accurately reflects the original guideline\'s content.

#### NGC STATUS

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

#### COPYRIGHT STATEMENT

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

### DISCLAIMER

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Date Modified: 11/10/2008