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

Numerous sets of diagnostic criteria have sought to define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and randomized trials and systematic reviews of treatment have been published. The objective is to prepare consensus guidelines on the definition, investigation and treatment of CIDP. Disease experts and a patient representative considered references retrieved from MEDLINE and Cochrane Systematic Reviews in May 2004 and prepared statements which were agreed in an iterative fashion. The Task Force agreed on good practice points to define clinical and electrophysiological diagnostic criteria for CIDP with or without concomitant diseases and investigations to be considered. The principal treatment recommendations were: (1) intravenous immunoglobulin (IVIg) or corticosteroids should be considered in sensory and motor CIDP (level B recommendation); (2) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (3) if IVIg and corticosteroids are ineffective plasma exchange (PE) should be considered (level A recommendation); (4) If the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug should be considered (Good Practice Point); (5) Symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).

[1]  C. Bolton,et al.  Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study. , 1996, Brain : a journal of neurology.

[2]  N. Latov Diagnosis of CIDP , 2002, Neurology.

[3]  G. Comi,et al.  Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy , 2001, Annals of neurology.

[4]  P. V. D. Van den Bergh,et al.  Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy , 2004, Muscle & nerve.

[5]  A. Ropper,et al.  Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2a. , 1998, Neurology.

[6]  J. Daube,et al.  Combined azathioprine and prednisone in chronic inflammatory‐demyelinating polyneuropathy , 1985, Neurology.

[7]  J. Mendell,et al.  Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. , 1989, Archives of neurology.

[8]  J. Meulstee,et al.  High‐dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy , 1990, Neurology.

[9]  P. Tonali,et al.  Pure motor chronic inflammatory demyelinating polyneuropathy , 2001, Journal of Neurology.

[10]  P. V. van Doorn,et al.  Distinguishing acute-onset CIDP from Guillain–Barré syndrome with treatment related fluctuations , 2005, Neurology.

[11]  W. Litchy,et al.  A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy , 1994, Annals of neurology.

[12]  M. Vermeulen,et al.  Pulsed high dose dexamethasone treatment in chronic inflammatory demyelinating polyneuropathy: a pilot study. , 1997, Journal of neurology, neurosurgery, and psychiatry.

[13]  P. Dyck,et al.  Chronic inflammatory polyradiculoneuropathy. , 1975, Mayo Clinic proceedings.

[14]  P. Vermersch,et al.  Relapsing demyelinating disease affecting both the central and peripheral nervous systems , 2008, Journal of Neurology, Neurosurgery, and Psychiatry.

[15]  J. A. Bragg,et al.  Sensory nerve conduction slowing is a specific marker for CIDP , 2008, Muscle & nerve.

[16]  C Chalk,et al.  Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. , 1996, Brain : a journal of neurology.

[17]  P. vanDoorn Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy , 1990 .

[18]  N. Latov,et al.  Peripheral neuropathy in patients with inflammatory bowel disease. , 2005, Brain : a journal of neurology.

[19]  R. Olney Guidelines in electrodiagnostic medicine. Consensus criteria for the diagnosis of partial conduction block. , 1999, Muscle & nerve. Supplement.

[20]  R. D. de Haan,et al.  Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. , 2009, The Cochrane database of systematic reviews.

[21]  G. Cruccu,et al.  EFNS guidelines on neuropathic pain assessment , 2004, European journal of neurology.

[22]  N. Gilhus,et al.  EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases , 2008, European journal of neurology.

[23]  R. Hughes,et al.  Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. , 2001, The Cochrane database of systematic reviews.

[24]  R. Hughes,et al.  Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. , 2003, The Cochrane database of systematic reviews.

[25]  R. Goswami High-dose intravenous immunoglobulin treatment in Graves' ophthalmopathy. , 1992, Acta endocrinologica.

[26]  Steve Vucic,et al.  Utility of somatosensory evoked potentials in chronic acquired demyelinating neuropathy , 2008, Muscle & nerve.

[27]  S. Jacob,et al.  Optimizing the use of electrophysiology in the diagnosis of chronic inflammatory demyelinating polyneuropathy: a study of 20 cases , 2005, Journal of the peripheral nervous system : JPNS.

[28]  H. Nagaraja,et al.  Welding-related parkinsonism: Clinical features, treatment, and pathophysiology , 2001, Neurology.

[29]  J. Daube,et al.  Chronic immune sensory polyradiculopathy , 2004, Neurology.

[30]  R. Hughes,et al.  Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. , 2004, The Cochrane database of systematic reviews.

[31]  H. Hartung,et al.  Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial , 2008, The Lancet Neurology.

[32]  R. Hughes,et al.  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 , 2005, Journal of the peripheral nervous system : JPNS.

[33]  W. Mcdonald,et al.  Chronic demyelinating peripheral neuropathy associated with multifocal central nervous system demyelination. , 1987, Brain : a journal of neurology.

[34]  P. Preux,et al.  Interferon beta-1a as an investigational treatment for CIDP , 2003, Neurology.

[35]  R. Dinapoli,et al.  Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment , 1982, Annals of neurology.

[36]  S. Boniface,et al.  Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[37]  J. Vallat Recommendations on diagnostic strategies for chronic inflammatory demyelinating polyradiculoneuropathy. , 2008, Postgraduate medical journal.

[38]  F. Jennekens,et al.  Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study. , 1993, Journal of neurology, neurosurgery, and psychiatry.

[39]  J. Albers,et al.  Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy , 1985, Muscle & nerve.

[40]  B. Sharrack,et al.  Randomized trial of interferon β-1a in chronic inflammatory demyelinating polyradiculoneuropathy , 1999, Neurology.

[41]  S. Jacob,et al.  Proximal nerve conduction studies in chronic inflammatory demyelinating polyneuropathy , 2006, Clinical Neurophysiology.

[42]  M. Bromberg,et al.  Corticosteroid use in the treatment of neuromuscular disorders: Empirical and evidence‐based data , 2004, Muscle & nerve.

[43]  R. Olney,et al.  Guidelines in electrodiagnostic medicine. Consensus criteria for the diagnosis of partial conduction block. , 1999, Muscle & nerve. Supplement.

[44]  M. Brainin,et al.  Guidance for the preparation of neurological management guidelines by EFNS scientific task forces – revised recommendations 2004 * , 2004, European journal of neurology.

[45]  A. Ropper,et al.  Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-α 2a , 1998, Neurology.

[46]  D. Menkes,et al.  Coincident membranous glomerulonephritis and chronic inflammatory demyelinating polyradiculoneuropathy: Questioning the autoimmunity hypothesis , 2008, Muscle & nerve.

[47]  Y. Rajabally,et al.  The value of sensory electrophysiology in chronic inflammatory demyelinating polyneuropathy , 2007, Clinical Neurophysiology.

[48]  S. Herskovitz,et al.  The restoration of IVIg efficacy by plasma exchange in CIDP , 1995, Neurology.