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 Joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society J Peripher Nerv Syst 2010 15 1 9

Centre de Référence Neuromusculaire, Cliniques universitaires St-Luc, Brussels, Belgium.
European Journal of Neurology (Impact Factor: 4.06). 03/2010; 17(3):356-63. DOI: 10.1111/j.1468-1331.2009.02930.x
Source: PubMed


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.
To revise these guidelines.
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.
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).

Download full-text


Available from: Peter Y K Van den Bergh, Oct 07, 2015
180 Reads
  • Source
    • "We suggest the cut-off values shown in Table 3 for the electrodiagnosis of CIDP. We have proposed cut-off values in each nerve in a Japanese multicenter study (Isose et al., 2009), and the values are employed in revised EFFS/PNS 2010 criteria (Van den Bergh et al., 2010). However, that study used only a low frequency filter of 20 Hz, and therefore the reported cut-off values cannot be used in EMG laboratories using lower filtering (e.g., 2–10 Hz). "
    [Show abstract] [Hide abstract]
    ABSTRACT: The duration of the distal compound muscle action potential (DCMAP) is a useful index to detect demyelination in the distal nerve segments. However in published electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), the cut-off values of DCMAP duration are defined using an EMG low frequency filter of only 20Hz. We aimed to provide widely-available reference data using several low cut filters. In 13 Japanese and European tertiary centers, DCMAP duration data using 2, 5, 10, and 20Hz low frequency filters were prospectively collected from 147 normal controls, 59 patients with typical CIDP, and 100 with diabetic polyneuropathy. Optimal cut-off values were calculated with receiver-operating characteristic curves, offering 100% specificity versus normal controls. The higher low frequency filter was associated with significantly shorter DCMAP duration in all groups. For CIDP diagnosis, the calculated cut-off values had a sensitivity ranging from 51% to 66%, and a specificity versus diabetic neuropathy from 96% to 98%. Our results show that DCMAP duration is largely dependent on low frequency filter settings, but is a useful index for CIDP diagnosis when the cut-off values are properly determined at each filter setting. Our data provide the systematic reference values of DCMAP duration for CIDP diagnosis available for most EMG laboratories. Copyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.
    Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 12/2014; 126(9). DOI:10.1016/j.clinph.2014.11.027 · 3.10 Impact Factor
  • Source
    • "Pre-treatment sera were obtained from 30 patients (19 men) with CIDP, all of whom fulfilled the EFNS/PNS diagnostic criteria of CIDP. According to the clinical criteria of the EFNS/PNS guidelines, we classified the patients into the following two subgroups: typical CIDP and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) (Van den Bergh et al., 2010); patients with other clinical subtypes of atypical CIDP were excluded from this study because of the small number of patients (0 or 1) during the study period. "
    [Show abstract] [Hide abstract]
    ABSTRACT: To identify serum cytokine networks specific to chronic inflammatory demyelinating polyneuropathy (CIDP), serum samples of two subgroups (18 patients with typical CIDP and 12 patients with multifocal acquired demyelinating sensory and motor neuropathy [MADSAM]) were analyzed with multiplex magnetic bead-based cytokine assay. TNF-α, HGF, MIP-1β and IL-1β levels were significantly higher in total CIDP patients than in normal controls. Of these, HGF levels were elevated in typical CIDP patients, but not in MADSAM patients. Patients with high HGF levels showed good responses to steroid treatment. Different cytokine profiles among the CIDP subtypes presumably reflect differences in pathophysiology.
    Journal of Neuroimmunology 12/2014; 279. DOI:10.1016/j.jneuroim.2014.12.017 · 2.47 Impact Factor
  • Source
    • "Electrophysiological baseline parameters were re-assessed and scored into categories by a neurophysiologist blinded for treatment and treatment outcome. The EFNS/PNS criteria for CIDP were used to define a conduction block and number of segments with a MNCV within the demyelinating range (<70% of lower limit of normal) [2]. Nerve segments over pressure points (elbow and fibula) and nerves with distal compound muscle action potential (CMAP) peak-peak amplitude below 1 mV were excluded from analysis [2]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: In the PREDICT study, a randomised controlled trial comparing dexamethasone with prednisolone in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), almost a quarter of patients deteriorated soon after starting treatment. The primary objective of this post-hoc analysis was to test the hypothesis that a focal demyelination pattern is associated with early deterioration after corticosteroid treatment and to explore whether various clinical characteristics are associated with deterioration after corticosteroid treatment. Clinical outcome was categorised into early deterioration and non-early deterioration. A neurophysiologist blinded for treatment outcome scored electrophysiological data into following categories: pure focal versus non-focal distribution of demyelination and no/minor versus moderate/severe sensory involvement. Additionally, we compared electrophysiological and clinical baseline parameters, with emphasis on previously reported possible associations. Early deterioration was found in 7 out of 33 patients (21%). Ten patients had pure focal distribution of demyelination, of whom 5 had early deterioration; 23 patients had non-focal distribution, of whom 2 had early deterioration (p = 0.02). Higher mean median nerve sensory nerve conduction velocity (SNCV) was found in patients with early deterioration compared to patients with non-early deterioration (52.6 and respectively 40.8 m/s, p = 0.02). Pure focal distribution of demyelination and lesser sensory electrophysiological abnormalities may be associated with early deterioration in CIDP patients treated with corticosteroids.
    BMC Neurology 04/2014; 14(1):72. DOI:10.1186/1471-2377-14-72 · 2.04 Impact Factor
Show more