menu-iconMore mobile-close-icon
Skip Navigation
Skip Navigation
PrintDownload PDFGet Adobe ReaderDownload to WordDownload as HTMLDownload as XMLCitation Manager
Save to Favorites
Guideline Summary
Guideline Title
European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision.
Bibliographic Source(s)
Van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, Koski CL, Leger JM, Nobile-Orazio E, Pollard J, Sommer C, van Doorn PA, van Schaik IN. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies [trunc]. Eur J Neurol. 2010 Mar;17(3):356-63. [47 references] PubMed External Web Site Policy
Guideline Status

This is the current release of the guideline.

This guideline updates previous versions: Hughes RA, Bouche P, Cornblath DR, Evers E, Hadden RD, Hahn A, Illa I, Koski CL, Leger JM, Nobile-Orazio E, Pollard J, Sommer C, Van den Bergh P, van Doorn PA, van Schaik IN. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the EFNS and PNS. Eur J Neurol 2006 Apr;13(4):326-32.

Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst 2005 Sep;10(3):220-8.

Scope

Disease/Condition(s)

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Guideline Category
Diagnosis
Evaluation
Management
Treatment
Clinical Specialty
Family Practice
Internal Medicine
Neurology
Intended Users
Physicians
Guideline Objective(s)

To update the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) (2005), based on newly available evidence and, where adequate evidence was not available, consensus

Target Population

Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Interventions and Practices Considered

Diagnosis

  1. Assessment of signs and symptoms
  2. Electrodiagnostic tests
  3. Cerebrospinal fluid (CSF) examination
  4. Magnetic resonance imaging (MRI)
  5. Nerve biopsy
  6. Assessment of treatment response
  7. Diagnosis of definite, probable, and possible chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Treatment

  1. Patient discussion of advantages and disadvantages of treatment options
  2. Intravenous immunoglobulin (IVIg)
  3. Corticosteroids
  4. Plasma exchange (PE)
  5. Combination treatments 
  6. Immunosuppressant or immunomodulatory drugs
  7. Advice about foot care, exercise, diet, driving, and lifestyle management
  8. Treatment of neuropathic pain
  9. Orthoses, physiotherapy, occupational therapy, psychological support, and referral to a rehabilitation specialist if indicated
  10. Information about patient support groups
Major Outcomes Considered
  • Sensitivity and specificity of diagnostic criteria
  • Effectiveness of treatment

Methodology

Methods Used to Collect/Select the Evidence
Searches of Electronic Databases
Description of Methods Used to Collect/Select the Evidence

The Task Force members searched MEDLINE and the Cochrane Library from August 2004 onwards to July 2009 for articles on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and 'diagnosis' or 'treatment' or 'guideline'.

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:

  1. Randomization concealment
  2. Primary outcome(s) is/are clearly defined
  3. Exclusion/inclusion criteria are clearly defined
  4. Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
  5. 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
Review of Published Meta-Analyses
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

Task Force members prepared draft statements about definition, diagnosis, and treatment. Evidence and recommendations were classified according to the scheme agreed for European Federation of Neurological Societies (EFNS) guidelines (see the "Availability of Companion Documents" field). When only class IV evidence was available but consensus could be reached, the Task Force offered advice as good practice points. The statements were revised and collated into a single document, which was then 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 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 (see the "Availability of Companion Documents" field).

Recommendations

Major Recommendations

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

Diagnostic Criteria for Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

Good Practice Points for defining diagnostic criteria for CIDP:

  1. Clinical: typical and atypical CIDP (see Table "Clinical Diagnostic Criteria" below)
  2. Electrodiagnostic: definite, probable, and possible CIDP (see Table "Electrodiagnostic Criteria" below)
  3. Supportive: including cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), nerve biopsy and treatment response (see Table "Supportive Criteria" below)
  4. Categories: definite, probable, and possible CIDP (see Table "Diagnostic Categories" below)
Table: Clinical Diagnostic Criteria
  1. Inclusion criteria
    1. Typical CIDP
      • Chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities, developing over at least 2 months; cranial nerves may be affected; and
      • Absent or reduced tendon reflexes in all extremities
    1. Atypical CIDP (still considered CIDP but with different features)

      One of the following, but otherwise as in (a) (tendon reflexes may be normal in unaffected limbs):

      • Predominantly distal (distal acquired demyelinating symmetric [DADS]) or
      • Asymmetric (multifocal acquired demyelinating sensory and motor neuropathy [MADSAM], Lewis–Sumner syndrome) or
      • Focal (e.g., involvement of the brachial or lumbosacral plexus or of one or more peripheral nerves in one upper or lower limb)
      • Pure motor or
      • Pure sensory (including chronic immune sensory polyradiculopathy affecting the central process of the primary sensory neuron)
  1. Exclusion criteria
    • Borrelia burgdorferi infection (Lyme disease), diphtheria, drug or toxin exposure probably to have caused the neuropathy
    • Hereditary demyelinating neuropathy
    • Prominent sphincter disturbance
    • Diagnosis of multifocal motor neuropathy
    • IgM monoclonal gammopathy with high titre antibodies to myelin-associated glycoprotein
    • Other causes for a demyelinating neuropathy including polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome); osteosclerotic myeloma; diabetic and non-diabetic lumbosacral radiculoplexus neuropathy. Peripheral nervous system (PNS) lymphoma and amyloidosis may occasionally have demyelinating features

 

Table: Electrodiagnostic Criteria
  1. Definite: at least one of the following
    1. Motor distal latency prolongation ≥50% above upper limit of normal values (ULN) in two nerves (excluding median neuropathy at the wrist from carpal tunnel syndrome), or
    2. Reduction of motor conduction velocity ≥30% below lower limit of normal values (LLN) in two nerves, or
    3. Prolongation of F-wave latency ≥30% above ULN in two nerves (≥50% if amplitude of distal negative peak compound muscle action potential (CMAP) <80% of LLN values), or
    4. Absence of F-waves in two nerves if these nerves have distal negative peak CMAP amplitudes ≥20% of LLN + ≥1 other demyelinating parametera in ≥1 other nerve, or
    5. Partial motor conduction block: ≥50% amplitude reduction of the proximal negative peak CMAP relative to distal, if distal negative peak CMAP >20% of LLN, in two nerves, or in one nerve + ≥1 other demyelinating parametera in ≥1 other nerve, or
    6. Abnormal temporal dispersion (>30% duration increase between the proximal and distal negative peak CMAP) in ≥2 nerves, or
    7. Distal CMAP duration (interval between onset of the first negative peak and return to baseline of the last negative peak) increase in ≥1 nerve (median ≥6.6 ms, ulnar ≥6.7 ms, peroneal ≥7.6 ms, tibial ≥8.8 ms) + ≥1 other demyelinating parametera in ≥1 other nerve
  1. Probable

    ≥30% amplitude reduction of the proximal negative peak CMAP relative to distal, excluding the posterior tibial nerve, if distal negative peak CMAP ≥20% of LLN, in two nerves, or in one nerve + ≥1 other demyelinating parametera in ≥1 other nerve

  2. Possible

    As in (1) but in only one nerve

To apply these criteria the median, ulnar (stimulated below the elbow), peroneal (stimulated below the fibular head), and tibial nerves on one side are tested. If criteria are not fulfilled, the same nerves are tested at the other side, and/or the ulnar and median nerves are stimulated bilaterally at the axilla and at Erb's point. Motor conduction block is not considered in the ulnar nerve across the elbow and at least 50% amplitude reduction between Erb's point and the wrist is required for probable conduction block. Temperatures should be maintained to at least 33 degrees C at the palm and 30 degrees C at the external malleolus (Good Practice Points).

aAny nerve meeting any of the criteria (a-g).

Table: Supportive Criteria
  1. Elevated CSF protein with leukocyte count <10/mm3 (Level A Recommendation)
  2. MRI showing gadolinium enhancement and/or hypertrophy of the cauda equina, lumbosacral or cervical nerve roots, or the brachial or lumbosacral plexuses (Level C Recommendation)
  3. Abnormal sensory electrophysiology in at least one nerve (Good Practice Points):
    1. Normal sural with abnormal median (excluding median neuropathy at the wrist from carpal tunnel syndrome) or radial sensory nerve action potential (SNAP) amplitudes; or
    2. Conduction velocity <80% of lower limit of normal (<70% if SNAP amplitude <80% of lower limit of normal); or
    3. Delayed somatosensory evoked potentials without central nervous system disease
  4. Objective clinical improvement following immunomodulatory treatment (Level A Recommendation)
  5. Nerve biopsy showing unequivocal evidence of demyelination and/or remyelination by electron microscopy or teased fibre analysis (Good Practice Point)

 

Table: Diagnostic Categories

Definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Clinical criteria 1 (a or b) and 2 with electrodiagnostic criterion 1; or

Probable CIDP + at least one supportive criterion; or

Possible CIDP + at least two supportive criteria

Probable CIDP

Clinical criteria 1 (a or b) and 2 with electrodiagnostic criterion 2; or

Possible CIDP + at least one supportive criterion

Possible CIDP

Clinical criteria 1 (a or b) and 2 with electrodiagnostic criterion 3

CIDP (definite, probable, possible) associated with concomitant diseases.

Treatment of CIDP

For induction of treatment:

  1. Intravenous immunoglobulin (IVIg) (Level A Recommendation) or corticosteroids (Level C Recommendation) should be considered in sensory and motor CIDP in the presence of disabling symptoms. Plasma exchange (PE) is similarly effective (Level A Recommendation) but may be less tolerated. The presence of relative contraindications to any of these treatments should influence the choice (Good Practice Point).
  2. The advantages and disadvantages should be explained to the patient who should be involved in the decision making (Good Practice Point).
  3. In pure motor CIDP IVIg should be considered as the initial treatment (Good Practice Point).

For maintenance treatment:

  1. If the first-line treatment is effective, continuation should be considered until the maximum benefit has been achieved and then the dose reduced to find the lowest effective maintenance dose (Good Practice Point).
  2. If the response is inadequate or the maintenance doses of the initial treatment (IVIg, steroids, or PE) result in adverse effects, the other first-line treatment alternatives should be tried before considering combination treatments or adding an immunosuppressant or immunomodulatory drug. There is no sufficient evidence to recommend any particular drug (see Table below) (Good Practice Point).
Table: Immunosuppressant and Immunomodulatory Drugs That Have Been Reported to Be Beneficial in CIDP (Class IV evidence)*

Alemtuzumab
Azathioprine
Cyclophosphamide
Ciclosporin
Etanercept
Interferon-alpha
Interferon-beta1a
Mycophenolate mofetil
Methotrexate
Rituximab
Stem cell transplantation (haematopoietic)

*Kuitwaard K, van Doorn PA. Newer therapeutic options for chronic inflammatory demyelinating polyradiculoneuropathy. Drugs 2009; 69: 987–1001; Hughes RAC, Swan AV, van Doorn PA. Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy (Update). Cochrane Database Syst Rev 2004; 4: CD003280.

  1. Advice about foot care, exercise, diet, driving, and lifestyle management should be considered. Neuropathic pain should be treated with drugs according to EFNS guideline on treatment of neuropathic pain. Depending on the needs of the patient, orthoses, physiotherapy, occupational therapy, psychological support and referral to a rehabilitation specialist should be considered (Good Practice Points).
  2. Information about patient support groups should be offered to those who would like it (Good Practice Point).

Definitions:

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:

  1. Randomization concealment
  2. Primary outcome(s) is/are clearly defined
  3. Exclusion/inclusion criteria are clearly defined
  4. Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
  5. 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 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 and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Potential Harms
  • Because of adverse events related to difficulty with venous access, use of citrate and haemodynamic changes are not uncommon in plasma exchange; either corticosteroids or intravenous immunoglobulin (IVIg) should be considered first.
  • Many observational studies report a beneficial effect from corticosteroids except in pure motor chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), where they can be harmful.

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.
  • There is a dearth of evidence concerning general aspects of treatment for symptoms of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) such as pain and fatigue. There is also a lack of research into the value of exercise and occupational and physical therapy in the management of CIDP. Evidence is limited concerning immunizations. International and national support groups offer information and support to patients (http://www.gbs-cidp.org External Web Site Policy).

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 Organization, 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, 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
Patient-centeredness

Identifying Information and Availability

Bibliographic Source(s)
Van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, Koski CL, Leger JM, Nobile-Orazio E, Pollard J, Sommer C, van Doorn PA, van Schaik IN. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies [trunc]. Eur J Neurol. 2010 Mar;17(3):356-63. [47 references] PubMed External Web Site Policy
Adaptation

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

Date Released
2006 Apr (revised 2010 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: P. Y. K. Van den Bergh, Centre de Référence Neuromusculaire, Cliniques universitaires St-Luc, Brussels, Belgium; R. D. M. Hadden, Department of Neurology, King's College London School of Medicine, London, UK; P. Bouche, Consultation de Pathologie Neuromusculaire, Groupe Hospitalier Pitié Salpêtrière, Paris, France; D. R. Cornblath, Department of Neurology, Johns Hopkins University, Baltimore, MD, USA; A. Hahn, Division of Neurology, London Health Sciences Centre, London, ON, Canada; I. Illa, Servei Neurologia, Hospital Universitari de la Sta Creu i Sant Pau, Barcelona, Spain; C. L. Koski, Department of Neurology, School of Medicine, University of Maryland, Baltimore, MD, USA; J.-M. Léger, Consultation de Pathologie Neuromusculaire Groupe Hospitalier, Pitié Saltpêtrière, Paris, France; E. Nobile-Orazio, Department of Translational Medicine, University of Milan IRCCS Humanitas Clinical Institute, Milan, Italy; J. Pollard, Neurology Department, University of Sydney, Sydney, NSW, Australia; C. Sommer, Department of Neurology, University of Würzburg, Würzburg, Germany; P. A. van Doorn, Erasmus Medical Centre Rotterdam, Department of Neurology, Rotterdam, The Netherlands; I. N. van Schaik, Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

Financial Disclosures/Conflicts of Interest

The following authors have reported conflicts of interest:

D. Cornblath, personal honoraria from Merck, Pfizer, Mitsubishi Pharma, Sangamo, Bristol-Myers Squibb, Eisai, Octapharma, Sun Pharma, Acorda, DP Clinical, Geron, Exelixis, Johnson&Johnson, Genzyme, Cebix, Abbott, CSL Behring, Pfizer, Schwartz Biosciences, Avigen, FoldRx

R.D.M. Hadden, personal honoraria from Janssen-Cilag and Talecris

A. Hahn, personal honoraria from Baxter, Bayer, and Biogen-Idec, Talecris

I. Illa, personal none, departmental research grant from Grifols

C. Koski, personal honoraria from Baxter, CSL, and Talecris

J.-M. Léger, personal none, departmental research grants or honoraria from Biogen-Idec, Baxter, 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, Talecris, and Bayer

The other authors have nothing to declare.

Guideline Status

This is the current release of the guideline.

This guideline updates previous versions: Hughes RA, Bouche P, Cornblath DR, Evers E, Hadden RD, Hahn A, Illa I, Koski CL, Leger JM, Nobile-Orazio E, Pollard J, Sommer C, Van den Bergh P, van Doorn PA, van Schaik IN. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the EFNS and PNS. Eur J Neurol 2006 Apr;13(4):326-32.

Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst 2005 Sep;10(3):220-8.

Guideline Availability

Electronic copies: Available in portable document format (PDF) from the European Federation of Neurological Societies (EFNS) Web site External Web Site Policy.

Print copies: Available from P. Y. K. Van den Bergh, Centre de Référence Neuromusculaire, Cliniques universitaires St-Luc, Université catholique de Louvain, 1200 Brussels, Belgium; Phone: +32 2 764 1311; Fax: +32 2 764 9052; E-mail: peter.vandenbergh@uclouvain.be.

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 External Web Site Policy.
  • Continuing Medical Education questions are available for registered users from the European Federation of Neurological Societies Web site External Web Site Policy.
Patient Resources

None available

NGC Status

This NGC summary was completed by ECRI on March 22, 2007. The information was verified by the guideline developer on May 3, 2007. This summary was updated by ECRI Institute on June 20, 2007 following the U.S. Food and Drug Administration (FDA) advisory on gadolinium-based contrast agents. This summary was updated by ECRI Institute on August 18, 2009, following the revised FDA advisory on CellCept (mycophenolate mofetil). This summary was updated by ECRI Institute on August 24, 2009, following the revised FDA advisory on CellCept (mycophenolate mofetil). This summary was updated by ECRI Institute on September 11, 2009, following the revised FDA advisory on Myfortic (mycophenolic acid). This summary was updated by ECRI Institute on November 16, 2010. The updated information was verified by the guideline developer on December 13, 2010. This summary was updated by ECRI Institute on October 12, 2011 following the U.S. Food and Drug Administration (FDA) advisory on Tumor Necrosis Factor-alpha (TNFα) Blockers. This summary was updated by ECRI Institute on November 21, 2013 following the U.S. Food and Drug Administration advisory on Arzerra (ofatumumab) and 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

The National Guideline Clearinghouse™ (NGC) does not develop, produce, approve, or endorse the guidelines represented on this site.

Read full disclaimer...