Table 5.

Recommendations and good practice points [Van Schaik et al. 2010].

Good practice points for diagnostic criteria

1. Clinical: the two core criteria and all exclusion criteria should be met (Table 1).

2. Electrodiagnostic: definite or probable CB in at least one nerve (Table 2).

3. Supportive: anti-GM1 antibodies, MRI, CSF, and treatment response (Table 3).

4. Categories: definite and probable MMN (Table 4).

Good practice points for diagnostic tests

1. Clinical examination and electrodiagnostic tests should be done in all patients.

2. Antiganglioside GM1 antibody testing, MRI of the brachial plexus, and CSF examination should be considered in selected patients.

3. Investigations to discover concomitant disease or exclude other possible causes should be considered, but the choice of tests will depend on the individual circumstances.

Good practice points for treatment

1. IVIg (2 g/kg given over 2–5 days) should be the first line treatment (level A) when disability is sufficiently severe to warrant treatment.

2. Corticosteroids are not recommended.

3. If an initial treatment with IVIg is effective, repeated IVIg treatment should be considered in selected patients (level C). The frequency of IVIg maintenance therapy should be guided by the response. Typical treatment regimens are 1 g/kg every 2–4 weeks or 2 g/kg every 1–2 months.

4. If IVIg is not sufficiently effective then immunosuppressive treatment may be considered. However, no agent has shown to be beneficial in a clinical trial and data from case series are conflicting.

5. Toxicity makes cyclophosphamide a less desirable option.

CB, conduction block; MRI, magnetic resonance imaging; CSF, cerebrospinal fluid; MMN, multifocal motor neuropathy; IVIg, intravenous immunoglobulin.