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BACKGROUND: 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. OBJECTIVES: The aim of this guideline was to prepare consensus guidelines on the definition, investigation, and treatment of CIDP. METHODS: Disease experts and a representative of patients considered references retrieved from MEDLINE and Cochrane Systematic Reviews in May 2004 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 as follows: (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 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); and (5) symptomatic treatment and multidisciplinary management should be considered (good practice point).
BACKGROUND: Several diagnostic criteria for multifocal motor neuropathy (MMN) have been proposed in recent years, and a beneficial effect of intravenous immunoglobulin (IVIg) and various other immunomodulatory drugs has been suggested in several trials and uncontrolled studies. OBJECTIVES: The aim of this guideline was to prepare consensus guidelines on the definition, investigation, and treatment of MMN. METHODS: Disease experts and a representative of patients considered references retrieved from MEDLINE and the Cochrane Library in July 2004 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 MMN and investigations to be considered. The principal recommendations and good practice points were as follows: (1) IVIg (2 g/kg given over 2-5 days) should be considered as the first line of treatment (level A recommendation) when disability is sufficiently severe to warrant treatment; (2) corticosteroids are not recommended (good practice point); (3) if initial treatment with IVIg is effective, repeated IVIg treatment should be considered (level C recommendation). The frequency of IVIg maintenance therapy should be guided by the individual response (good practice point). Typical treatment regimens are 1 g/kg every 2-4 weeks or 2 g/kg every 4-8 weeks (good practice point); (4) if IVIg is not (or not sufficiently) effective, then immunosuppressive treatment may be considered. Cyclophosphamide, cyclosporine, azathioprine, interferon-beta1a, or rituximab are possible agents (good practice point); and (5) toxicity makes cyclophosphamide a less desirable option (good practice point).
BACKGROUND: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological, and hematological features. OBJECTIVES: The aim of this guideline was to prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein [paraproteinemic demyelinating neuropathy (PDN)]. METHODS: Disease experts and a representative of patients considered references retrieved from MEDLINE and the Cochrane Library and prepared statements that were agreed in an iterative fashion. RECOMMENDATIONS: In the absence of adequate data, evidence-based recommendations were not possible, but the Task Force agreed on the following good practice points: (1) patients with PDN should be investigated for a malignant plasma cell dyscrasia; (2) the paraprotein is more likely to be causing the neuropathy if the paraprotein is immunoglobulin M (IgM), antibodies are present in serum or on biopsy, or the clinical phenotype is chronic distal sensory neuropathy; (3) patients with IgM PDN usually have predominantly distal and sensory impairment, with prolonged distal motor latencies, and often anti-myelin-associated glycoprotein antibodies; (4) IgM PDN sometimes responds to immunotherapies. Their potential benefit should be balanced against their possible side effects and the usually slow disease progression; (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy clinically, electrophysiologically, and in response to treatment; and (6) for POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with hemato-oncology advice.
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).
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