Table 4.

Treatment of paraproteinemic neuropathies (with and without anti-MAG antibodies) based on evidence of clinical trials.

Treatment	Trial	Year	Characteristics: design	Patients (n)	Outcome
Effective treatment
IVIg	Comi et al.	2002	IVIg (2 g/kg) (n ¼12) versus placebo, double-blind, randomized, controlled crossover trial	22	Decrease in overall disability during IVIg treatment (p ¼ 0.001). Improvement in secondary outcome measures (Rankin scale, time to walk 10 meters, grip strength) during IVIg treatment.
IVIg	Dalakas et al.	1996	IVIg (2g/kg 1 x/month for 3 months) versus placebo, double-blind, randomized, controlled crossover trial	11	Improvement only in 2/11 patients during IVIg treatment. No significant benefit of IVIg treatment
Plasma exchange	Dyck et al.	1991	PE (6x) versus sham-treatment, double-blind	39	Marked improvement in disability score. More benefit in the IgG and IgA gammopathy subgroup as compared with IgM gammopathy population.
Presumably ineffective treatment
Cyclophosphamide with prednisone	Niermeijer et al.	2007	Oral cyclophosphamide (500 mg/d, 4d + oral prednisone 60 mg/d 5d versus placebo every 28d (6x)	35	No difference in functional scale after 6 months. Improvement in several secondary outcomes.
Interferon-alpha	Mariette et al.	2000	Interferon-” (4.5 MIU 3 x week for 6 months) versus placebo, randomized, double blind trial	24	No clinical improvement compared to placebo.

PE, plasma exchange; IVIg, intravenous immunoglobulins; NDS, neurological disability score; MIU, million international units, s.c., subcutaneously.