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

Authors


  • This article is published simultaneously by European Journal of Neurology and Journal of the Peripheral Nervous System.

  • This is a Continuing Medical Education article, and can be found with corresponding questions on the Internet at http://www.efns.org/EFNSContinuing-Medical-Education-online.301.0.html. Certificates for correctly answering the questions will be issued by the EFNS.

P. Y. K. Van den Bergh, Centre de Référence Neuromusculaire, Cliniques universitaires St-Luc, Université catholique de Louvain, 1200 Brussels, Belgium (tel.: +32 2 764 1311; fax: +32 2 764 9052; e-mail: peter.vandenbergh@uclouvain.be).

Abstract

Background:  Consensus guidelines on the definition, investigation, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been previously published in European Journal of Neurology and Journal of the Peripheral Nervous System.

Objectives:  To revise these guidelines.

Methods:  Disease experts, including a representative of patients, considered references retrieved from MEDLINE and Cochrane Systematic Reviews published between August 2004 and July 2009 and prepared statements that were agreed in an iterative fashion.

Recommendations:  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: (i) intravenous immunoglobulin (IVIg) (Recommendation Level A) or corticosteroids (Recommendation Level C) should be considered in sensory and motor CIDP; (ii) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (iii) if IVIg and corticosteroids are ineffective, plasma exchange (PE) should be considered (Recommendation Level A); (iv) 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); (v) symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).

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