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## Complete Summary

#### GUIDELINE TITLE

European Federation of Neurological Societies/Peripheral Nerve Society
Guideline on management of paraproteinemic demyelinating neuropathies.
Report of a joint task force of the European Federation of Neurological
Societies and the Peripheral Nerve Society.

#### BIBLIOGRAPHIC SOURCE(S)

Joint Task Force of the EFNS and the PNS. European Federation of
Neurological Societies/Peripheral Nerve Society guideline on management
of paraproteinemic demyelinating neuropathies. Report of a joint task
force of the European Federation of Neurological Societies and the
Peripheral Nerve Soc. J Peripher Nerv Syst 2006 Mar;11(1):9-19. \[60
references\]
[PubMed](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16519778)

#### GUIDELINE STATUS

This is the current release of the guideline.

### \*\* REGULATORY ALERT \*\*

#### FDA WARNING/REGULATORY ALERT

**Note from the National Guideline Clearinghouse**: This guideline
references a drug(s) for which important revised regulatory and/or
warning information has been released.

-   [September 11, 2008, Rituxan
    (Rituximab)](http://www.fda.gov/medwatch/safety/2008/safety08.htm#Rituxan):
    Genentech informed healthcare professionals of revisions to
    prescribing information for Rituxan regarding a case of progressive
    multifocal leukoencephalopathy (PML) leading to death in a patient
    with rheumatoid arthritis who received Rituxan in a long-term safety
    extension clinical study.

### COMPLETE SUMMARY CONTENT

 \*\* REGULATORY ALERT \*\*\
 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)

Paraproteinemic demyelinating neuropathy (PDN)

**Note**: Axonal neuropathies with a paraprotein are not part of the
scope of these guidelines but are mentioned briefly.

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation\
Management\
Treatment

#### CLINICAL SPECIALTY

Family Practice\
Internal Medicine\
Neurology\
Oncology

#### INTENDED USERS

Physicians

#### GUIDELINE OBJECTIVE(S)

To construct clinically useful guidelines for the diagnosis,
investigation, and treatment of patients with both a demyelinating
neuropathy and a paraprotein (paraproteinemic demyelinating neuropathy
\[PDN\]), based on the available evidence and, where evidence was not
available, consensus

#### TARGET POPULATION

Patients presenting with paraproteinemic demyelinating neuropathy (PDN)

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis/Evaluation**

1.  Investigation of a paraprotein including:

    -   Serum immunofixation electrophoresis (SIFE)

    -   Physical examination and assessment of signs and symptoms

    -   Full blood count, renal and liver function, erythrocyte
        sedimentation rate, C-reactive protein, uric acid

    -   Total immunoglobulin G (IgG), IgA, IgM concentrations

    -   Radiologic x-ray skeletal survey (skull, pelvis, spine, ribs,
        long bones)

    -   Ultrasound or computed tomography of abdomen and chest

**Management/Treatment**

1.  IgM paraproteinemic demyelinating neuropathy (PDN)

    -   Withholding immunosuppressive or immunomodulatory treatment and
        providing symptomatic treatment for tremor and paresthesiae in
        patients without significant disability

    -   Intravenous immunoglobulin (IVIg)

    -   Plasma exchange (PE)

    -   Immunosuppressive treatment in patients with significant
        disability

2.  IgG and IgA PDN

    -   Therapeutic approach is similar to the approach in chronic
        inflammatory demyelinating polyradiculoneuropathy (CIDP)

3.  POEMS (signs of polyneuropathy, organomegaly, endocrinopathy, M
    band, and skin changes)

    -   Consultation with hemato-oncologist

    -   Local radiation or surgery

    -   Melphalan with or without corticosteroids

#### MAJOR OUTCOMES CONSIDERED

-   Sensitivity of diagnostic tests

-   Effectiveness of treatment

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

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

The Task Force members searched MEDLINE from 1980 onwards on July 24,
2004, for articles on paraproteinemic demyelinating neuropathy (PDN) and
\"diagnosis\" or \"treatment\" or \"guideline\" and used the personal
databases of Task Force members. They also searched the Cochrane Library
in September 2004.

#### 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

**Evidence Classification Scheme for a Diagnostic Measure**

**Class I**: A prospective study in a broad spectrum of persons with the
suspected condition, using a \"gold standard\" for case definition,
where the test is applied in a blinded evaluation, and enabling the
assessment of appropriate tests of diagnostic accuracy

**Class II**: A prospective study of a narrow spectrum of persons with
the suspected condition, or a well-designed retrospective study of a
broad spectrum of persons with an established condition (by \"gold
standard\") compared to a broad spectrum of controls, where test is
applied in a blinded evaluation, and enabling the assessment of
appropriate tests of diagnostic accuracy

**Class III**: Evidence provided by a retrospective study where either
persons with the established condition or controls are of a narrow
spectrum, and where test is applied in a blinded evaluation

**Class IV**: Any design where test is not applied in blinded evaluation
OR evidence provided by expert opinion alone or in descriptive case
series (without controls)

**Evidence Classification Scheme for a Therapeutic Intervention**

**Class I**: An adequately powered prospective, randomized, controlled
clinical trial with masked outcome assessment in a representative
population or an adequately powered systematic review of prospective
randomized controlled clinical trials with masked outcome assessment in
representative populations. The following are required:

a.  Randomization concealment

b.  Primary outcome(s) is/are clearly defined

c.  Exclusion/inclusion criteria are clearly defined

d.  Adequate accounting for dropouts and crossovers with numbers
    sufficiently low to have minimal potential for bias

e.  Relevant baseline characteristics are presented and substantially
    equivalent among treatment groups or there is appropriate
    statistical adjustment for differences

**Class II**: Prospective matched-group cohort study in a representative
population with masked outcome assessment that meets a--e above or a
randomized, controlled trial in a representative population that lacks
one criteria a--e

**Class III**: All other controlled trials (including well-defined
natural history controls or patients serving as own controls) in a
representative population, where outcome assessment is independent of
patient treatment

**Class IV**: Evidence from uncontrolled studies, case series, case
reports, or expert opinion

#### METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review

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

Not stated

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

This guideline has been produced by the Task Force members of the
European Federation of Neurological Societies (EFNS) who are also
members of the Peripheral Nerve Society (PNS). Additional non-European
members of the Task Force were appointed on the recommendation of the
Board of Directors of the PNS.

**Methods for Reaching Consensus**

Pairs of Task Force members prepared draft statements about
classification, investigation, and treatment which were considered at a
meeting in September 2004. Evidence was classified as classes I to IV,
and recommendations were classified as levels A to C (see the \"Rating
Scheme for the Strength of the Evidence\" and the \"Rating Scheme for
the Strength of the Recommendations\" fields). When only class IV
evidence was available but consensus could be reached, the Task Force
has offered advice as good practice points. The statements were collated
into a single document that was revised iteratively until consensus was
reached.

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

**Rating of Recommendations for a Diagnostic Measure**

**Level A rating** (established as useful/predictive or not
useful/predictive) requires at least one convincing class I study or at
least two consistent, convincing class II studies.

**Level B rating** (established as probably useful/predictive or not
useful/predictive) requires at least one convincing class II study or
overwhelming class III evidence.

**Level C rating** (established as possibly useful/predictive or not
useful/predictive) requires at least two convincing class III studies.

**Rating of Recommendations for a Therapeutic Intervention**

**Level A rating** (established as effective, ineffective, or harmful)
requires at least one convincing class I study or at least two
consistent, convincing class II studies.

**Level B rating** (probably effective, ineffective, or harmful)
requires at least one convincing class II study or overwhelming class
III evidence.

**Level C rating** (possibly effective, ineffective, or harmful)
requires at least two convincing class III studies.

**Good Practice Point (GPP)** When only class IV evidence was available
but consensus could be reached, the Task Force offered advice as good
practice points.

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

The guidelines were validated according to the European Federation of
Neurological Societies (EFNS) criteria (Hughes RAC, Barnes MP, Baron J,
Brainin M \[2001\]. Guidance for the preparation of neurological
management guidelines by EFNS scientific task forces. *Eur J Neurol*
8:549-550).

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

The levels of evidence (class I-IV) supporting the recommendations and
ratings of recommendations (A-C, Good Practice Point \[GPP\]) are
defined at the end of the \"Major Recommendations\" field.

**Investigation and Classification of the Paraproteins**

The Table below suggests investigations to be considered in all patients
with a paraprotein. Serum immunofixation electrophoresis (SIFE) should
be performed in all cases of known paraprotein to define the heavy- and
light-chain types, in all acquired demyelinating neuropathies, and if a
paraprotein is suspected but not detected by standard serum protein
electrophoresis (SPEP).

+-----------------------------------------------------------------------+
| **Table. Investigation of a Paraprotein**                             |
+=======================================================================+
| The following should be considered in all patients with a             |
| paraprotein:                                                          |
|                                                                       |
| 1.  Serum immunofixation electrophoresis                              |
|                                                                       |
| 2.  Physical examination for peripheral lymphadenopathy,              |
|     hepatosplenomegaly, macroglossia, and signs of polyneuropathy,    |
|     organomegaly, endocrinopathy, M band, and skin changes (POEMS     |
|     syndrome) (see section \"Other neuropathy syndromes associated    |
|     with paraproteinemia\" in the original guideline document)        |
|                                                                       |
| 3.  Full blood count, renal and liver function, calcium, phosphate,   |
|     erythrocyte sedimentation rate, C-reactive protein, uric acid,    |
|     beta 2-microglobulin, lactate dehydrogenase, rheumatoid factor,   |
|     serum cryoglobulins                                               |
|                                                                       |
| 4.  Total immunoglobulin G (IgG), IgA, IgM concentrations             |
|                                                                       |
| 5.  Random urine collection for the detection of Bence-Jones protein  |
|     (free light chains), and, if positive, 24 h urine collection for  |
|     protein quantification                                            |
|                                                                       |
| 6.  Radiographic x-ray skeletal survey, including skull, pelvis,      |
|     spine, ribs, long bones (shoulder to wrist, and hip to ankle), to |
|     look for lytic or sclerotic lesions. If this is negative, then a  |
|     Technetium-99 (Tc-99)m sesta-MIBI (2-methoxy-isobutyl-isonitrile) |
|     scan if high degree of suspicion of myeloma (IgA lambda and IgG   |
|     lambda paraproteins are more frequently associated with           |
|     osteosclerotic myeloma)                                           |
|                                                                       |
| 7.  Ultrasound or computed tomography of abdomen and chest (to detect |
|     lymphadenopathy, hepatosplenomegaly)                              |
|                                                                       |
| 8.  Consultation with a hematologist and bone marrow examination      |
|     (morphology, immunophenotype, and biopsy)                         |
+-----------------------------------------------------------------------+

**Is the Paraprotein Causing the Neuropathy?**

+-----------------------------------------------------------------------+
| **Table. Causal Relationship between Paraprotein and Demyelinating    |
| Neuropathy**                                                          |
+=======================================================================+
| 1.  Highly probable if immunoglobulin M (IgM) paraprotein (monoclonal |
|     gammopathy of uncertain significance \[MGUS\] or Waldenstrom\'s)  |
|     and:                                                              |
|                                                                       |
|     a.  High titers of anti-myelin-associated glycoprotein (anti-MAG) |
|         or anti-GQ1b antibodies, or                                   |
|                                                                       |
|     b.  Nerve biopsy shows IgM or complement deposits on myelin, or   |
|         widely spaced myelin on electron microscopy                   |
+-----------------------------------------------------------------------+
| 2.  Probable if either:                                               |
|                                                                       |
|     a.  IgM paraprotein (MGUS or Waldenström\'s) with high titers of  |
|         IgM antibodies to other neural antigens (GM1, GD1a, GD1b,     |
|         GM2, sulfatide, etc.), and slowly progressive predominantly   |
|         distal symmetrical sensory neuropathy, or                     |
|                                                                       |
|     b.  IgG or IgA paraprotein and nerve biopsy evidence (as in1b but |
|         with IgG or IgA deposits)                                     |
+-----------------------------------------------------------------------+
| 3.  Less likely when any of the following are present in a patient    |
|     with MGUS and without anti-MAG antibodies (diagnosis may be       |
|     described as \'chronic inflammatory demyelinating                 |
|     polyradiculoneuropathy with coincidental paraprotein\'):          |
|                                                                       |
|     a.  Time to peak of neuropathy \<6 months                         |
|                                                                       |
|     b.  Relapsing/remitting or monophasic course                      |
|                                                                       |
|     c.  Cranial nerves involved (except chronic ataxic neuropathy     |
|         with ophthalmoplegia, IgM monoclonal gammopathy, cold         |
|         agglutinins, and disialoganglioside \[CANOMAD\])              |
|                                                                       |
|     d.  Asymmetry                                                     |
|                                                                       |
|     e.  History of preceding infection                                |
|                                                                       |
|     f.  Abnormal median with normal sural sensory action potential    |
|                                                                       |
|     g.  IgG or IgA paraprotein without biopsy features in 2b          |
+-----------------------------------------------------------------------+

**Cerebrospinal Fluid and Nerve Biopsy**

Cerebrospinal fluid (CSF) examination and nerve biopsy may be helpful in
selected circumstances (see Table, below, **good practice points**) but
are not usually necessary if there is clearly demyelinating physiology
with monoclonal gammopathy of uncertain significance (MGUS).

+-----------------------------------------------------------------------+
| **Table. Cerebrospinal Fluid (CSF) Examination and Nerve Biopsy**     |
+=======================================================================+
| 1.  CSF examination is most likely to be helpful in the following     |
|     situations:                                                       |
|                                                                       |
|     a.  In patients with borderline demyelinating or axonal           |
|         electrophysiology or atypical phenotype, where the presence   |
|         of raised CSF protein would help suggest that the neuropathy  |
|         is immune mediated                                            |
|                                                                       |
|     b.  The presence of malignant cells would confirm                 |
|         lymphoproliferative infiltration                              |
+-----------------------------------------------------------------------+
| 2.  Nerve biopsy (usually sural nerve) is most likely to be helpful   |
|     when the following conditions are being considered:               |
|                                                                       |
|     a.  Amyloidosis                                                   |
|                                                                       |
|     b.  Vasculitis (e.g., due to cryoglobulinemia)                    |
|                                                                       |
|     c.  Malignant lymphoproliferative infiltration of nerves, or      |
|                                                                       |
|     d.  Immunoglobulin M paraproteinemic demyelinating neuropathy     |
|         (IgM PDN) with negative anti-myelin-associated glycoprotein   |
|         antibodies, or IgG or IgA PDN with a chronic progressive      |
|         course, where the discovery of widely spaced myelin on        |
|         electron microscopy or deposits of immunoglobulin and/or      |
|         complement bound to myelin would support a causal             |
|         relationship between paraprotein and neuropathy. However,     |
|         clinical decisions on treatment are often made without a      |
|         biopsy.                                                       |
+-----------------------------------------------------------------------+

**Treatment of Paraproteinemic Demyelinating Neuropathy (PDN)**

*Good Practice Points for Treatment of IgM PDN*

1.  In patients without significant disability, consideration should be
    given to withholding immunosuppressive or immunomodulatory
    treatment, providing symptomatic treatment for tremor and
    paresthesiae, and giving reassurance that symptoms are unlikely to
    worsen significantly for several years.

2.  In patients with significant disability or rapid worsening,
    intravenous immunoglobulin (IVIg) or plasma exchange (PE) should be
    considered as initial treatment, although their efficacy is
    unproven.

3.  In patients with moderate or severe disability, immunosuppressive
    treatment should be considered, although its long-term efficacy
    remains unproven. Preliminary reports suggest that rituximab may be
    a promising therapy.

4.  More research is needed.

*Good Practice Points for Treatment of IgG and IgA PDN*

In patients with a chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP)-like neuropathy, the detection of IgG or
IgA MGUS does not justify a different therapeutic approach from CIDP
without a paraprotein.

*Good Practice Points for Treatment of POEMS (signs of polyneuropathy,
organomegaly, endocrinopathy, M band, and skin changes)*

1.  Patients should be managed in consultation with a hemato-oncologist.

2.  Local radiation or surgery should be considered as the initial
    treatment for isolated plasmacytoma.

3.  Melphalan (with or without corticosteroids) should be considered for
    patients with multiple or no detectable bone lesions.

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

**Evidence Classification Scheme for a Diagnostic Measure**

**Class I**: A prospective study in a broad spectrum of persons with the
suspected condition, using a \"gold standard\" for case definition,
where the test is applied in a blinded evaluation, and enabling the
assessment of appropriate tests of diagnostic accuracy

**Class II**: A prospective study of a narrow spectrum of persons with
the suspected condition, or a well-designed retrospective study of a
broad spectrum of persons with an established condition (by \"gold
standard\") compared to a broad spectrum of controls, where test is
applied in a blinded evaluation, and enabling the assessment of
appropriate tests of diagnostic accuracy

**Class III**: Evidence provided by a retrospective study where either
persons with the established condition or controls are of a narrow
spectrum, and where test is applied in a blinded evaluation

**Class IV**: Any design where test is not applied in blinded evaluation
OR evidence provided by expert opinion alone or in descriptive case
series (without controls)

**Evidence Classification Scheme for a Therapeutic Intervention**

**Class I**: An adequately powered prospective, randomized, controlled
clinical trial with masked outcome assessment in a representative
population or an adequately powered systematic review of prospective
randomized controlled clinical trials with masked outcome assessment in
representative populations. The following are required:

a.  Randomization concealment

b.  Primary outcome(s) is/are clearly defined

c.  Exclusion/inclusion criteria are clearly defined

d.  Adequate accounting for dropouts and crossovers with numbers
    sufficiently low to have minimal potential for bias

e.  Relevant baseline characteristics are presented and substantially
    equivalent among treatment groups or there is appropriate
    statistical adjustment for differences

**Class II**: Prospective matched-group cohort study in a representative
population with masked outcome assessment that meets a--e above or a
randomized, controlled trial in a representative population that lacks
one criteria a--e

**Class III**: All other controlled trials (including well-defined
natural history controls or patients serving as own controls) in a
representative population, where outcome assessment is independent of
patient treatment

**Class IV**: Evidence from uncontrolled studies, case series, case
reports, or expert opinion

**Rating of Recommendations for a Diagnostic Measure**

**Level A rating** (established as useful/predictive or not
useful/predictive) requires at least one convincing class I study or at
least two consistent, convincing class II studies.

**Level B rating** (established as probably useful/predictive or not
useful/predictive) requires at least one convincing class II study or
overwhelming class III evidence.

**Level C rating** (established as possibly useful/predictive or not
useful/predictive) requires at least two convincing class III studies.

**Rating of Recommendations for a Therapeutic Intervention**

**Level A rating** (established as effective, ineffective, or harmful)
requires at least one convincing class I study or at least two
consistent, convincing class II studies.

**Level B rating** (probably effective, ineffective, or harmful)
requires at least one convincing class II study or overwhelming class
III evidence.

**Level C rating** (possibly effective, ineffective, or harmful)
requires at least two convincing class III studies.

**Good Practice Point (GPP)** When only class IV evidence was available
but consensus could be reached, the Task Force offered advice as good
practice points.

#### 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 selected
recommendations (see \"Major Recommendations\").

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

Appropriate diagnosis, investigation, and treatment of paraproteinemic
demyelinating neuropathy (PDN)

#### POTENTIAL HARMS

Not stated

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

This guideline provides the view of an expert task force appointed by
the Scientific Committee of the European Federation of Neurological
Societies (EFNS). It represents a peer-reviewed statement of minimum
desirable standards for the guidance of practice based on the best
available evidence. It is not intended to have legally binding
implications in individual cases. This guideline is not intended to have
implications regarding reimbursement.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

The European Federation of Neurological Societies has a mailing list and
all guideline papers go to national societies, national ministries of
health, World Health Organisation, European Union, and a number of other
destinations. Corporate support is recruited to buy large numbers of
reprints of the guideline papers and permission is given to sponsoring
companies to distribute the guideline papers from their commercial
channels, provided there is no advertising attached.

#### IMPLEMENTATION TOOLS

Staff Training/Competency Material

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

Living with Illness

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Joint Task Force of the EFNS and the PNS. European Federation of
Neurological Societies/Peripheral Nerve Society guideline on management
of paraproteinemic demyelinating neuropathies. Report of a joint task
force of the European Federation of Neurological Societies and the
Peripheral Nerve Soc. J Peripher Nerv Syst 2006 Mar;11(1):9-19. \[60
references\]
[PubMed](http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16519778)

#### ADAPTATION

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

#### DATE RELEASED

2006 Mar

#### GUIDELINE DEVELOPER(S)

European Federation of Neurological Societies - Medical Specialty
Society\
Peripheral Nerve Society - Disease Specific Society

#### SOURCE(S) OF FUNDING

European Federation of Neurological Societies

#### GUIDELINE COMMITTEE

Joint Task Force of the European Federation of Neurological Societies
and the Peripheral Nerve Society

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Task Force Members*: Robert D. Hadden, UK; Eduardo Nobile-Orazio,
Italy; Claudia Sommer, Germany; Angelika Hahn, Canada; Isabel Illa,
Spain; Enrica Morra, Italy; John D. Pollard, Australia; Richard A.C.
Hughes (*Chair*), UK; Pierre Bouche, France; David R. Cornblath, USA;
Eileen Evers, UK; Carol L. Koski, USA; Jean-Marc Léger, France; Peter
Van den Bergh, Belgium; Pieter A. van Doorn, Netherlands; Ivo N. van
Schaik, Netherlands

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

The following authors have reported conflicts of interest as follows:

R. Hughes, personal none, departmental research grants or honoraria from
Bayer, Biogen-Idec, Schering-LFB, and Kedrion

D. Cornblath, personal honoraria from Aventis Behring and Baxter

C. Koski, personal honoraria from American Red Cross, Baxter, Bayer, and
ZLB-Behring

J. M. Leger, personal none, departmental research grants or honoraria
from Biogen-Idec, Baxter, Laboratoire Francais du Biofractionnement
(LFB), and Octapharma

E. Nobile-Orazio, personal honoraria from Kedrion, Grifols, Baxter, and
LFB (and he has been commissioned by Kedrion and Baxter to give expert
opinions to the Italian Ministry of Health on the use of IVIg in
dysimmune neuropathies)

J. Pollard, personal none, departmental research grants from Biogen-Idec
and Schering

P. van Doorn, personal none, departmental research grants or honoraria
from Baxter and Bayer

The other authors have nothing to declare.

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: Available to registered users from the [European
Federation of Neurological Societies Web
site](http://www.efns.org/content.php?pid=142).

Print copies: Available from Dr. Robert Hadden, Department of Neurology,
King\'s College Hospital, Denmark Hill, London SE5 9RS, UK; E-mail:
<rob.hadden@doctors.org.uk>

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   Brainin M, Barnes M, Baron JC, Gilhus NE, Hughes R, Selmaj K,
    Waldemar G; Guideline Standards Subcommittee of the EFNS Scientific
    Committee. Guidance for the preparation of neurological management
    guidelines by EFNS scientific task forces -- revised
    recommendations 2004. Eur J Neurol. 2004 Sep;11(9):577-81.
    Electronic copies: Available in Portable Document Format (PDF) from
    the [European Federation of Neurological Societies Web
    site](http://www.efns.org/files/guideline_23.pdf).

-   Guideline papers. European Federation of Neurological Societies.
    Electronic copies: Available from the [European Federation of
    Neurological Societies Web
    site](http://www.efns.org/content.php?pid=141).

-   Continuing Medical Education questions available from the [European
    Journal of Neurology Web
    site](http://www.blackwellpublishing.com/products/journals/ene/mcqs/).

#### PATIENT RESOURCES

None available

#### NGC STATUS

This NGC summary was completed by ECRI on December 8, 2006. The
information was verified by the guideline developer on January 2, 2007.
This summary was updated by ECRI Institute on October 8, 2008 following
the U.S. Food and Drug Administration advisory on Rituxan (rituximab).

#### COPYRIGHT STATEMENT

This NGC summary is based on the original guideline, which is subject to
the Blackwell-Synergy copyright restrictions.

### DISCLAIMER

#### NGC DISCLAIMER

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© 1998-2008 National Guideline Clearinghouse

Date Modified: 11/3/2008