Intravenous immunoglobulin therapy in multifocal motor neuropathy: A double-blind, placebo-controlled study

Abstract

We conducted a double-blind, placebo-controlled, study of 19 patients fulfilling eligibility criteria for multifocal motor neuropathy with persistent conduction block. They were enrolled and divided into two groups: those who had never been treated previously with intravenous immunoglobulins (IVIg) (Group 1: 10 patients) and those who presented recurrent symptoms after previously successful treatment with IVIg (Group 2: nine patients). They were randomized prospectively to receive either IVIg or placebo at a dose of 500 mg/kg/day for 5 consecutive days, once a month for 3 months. At month 4, patients found to be responders remained on the same treatment for the 3 following months, while non-responders were switched to the alternative study drug for the 3 following months. Clinical assessment was conducted with the MRC score in 28 muscles and a self-evaluation scale (five daily motor activities scored from 0 to 5). In Group 1, nine patients completed the study, of whom initially four received IVIg and five placebo; four patients responded to IVIg (two at months 4 and 7, and a further two at month 7 after switching treatment at month 4), two patients responded to placebo at months 4 and 7, and three patients did not respond to either treatment. In Group 2, nine patients completed the study. Five patients first received IVIg and all responded at months 4 and 7. Four patients first received placebo and none responded at month 4; all were then switched to IVIg and three responded at month 7. When the 18 patients were considered together, seven out of the nine patients who received IVIg first were responders at month 4, compared with two of the nine patients who received placebo first, a difference that was statistically significant (P = 0.03). On the other hand, there was no significant difference in MRC score but a significant difference in the self-evaluation score, at month 4, between IVIg patients and placebo patients. Electrophysiological studies did not show significant differences at month 4 in motor parameters between IVIg patients and placebo patients. IgM anti-GM1 titres did not change significantly in patients treated with IVIg compared with those who received placebo, between baseline, month 4 and month 7. However, of five patients who had significantly high anti-GM1 titres (>3200) at baseline, four responded to IVIg. This trial confirms that IVIg is a promising therapeutic option for multifocal motor neuropathy.

Combination of High-Dose Intravenous Immunoglobulins and Itraconozole in Treating Chronic Mycotic Demyelinating Optic Neuritis

“…Hence, IVIG can also reduce autoantibody levels through mechanisms such as downregulation of antibody production or neutralization by anti-idiotypic antibodies [20]. For chronic immune-mediated neuropathies such as demyelinating optic neuritis, the diagnosis is made on clinical, electrophysiological, and sometimes immunochemical and pathological criteria [21] and the treatments are sometimes prolonged. As we have observed in our patient, the efficacy of short-term treatments with IVIG depended on the type of the polyneuropathy, but for conditions such as demyelinating optic neuritis, high-dose IVIG may sometimes lead to continuous improvement.…”

Multifocal motor neuropathy: and then, 20 years later IVIg therapy

“…No significant change in serum anti-GM1 antibody titres was noted. Finally two doubleblind, placebo-controlled studies [32,33] confirmed the efficacy of IVIg versus placebo, given respectively during 2-6 months, in 16 and 18 patients, respectively. Furthermore, one of these studies [33] showed no significant changes in conduction block and serum anti-GM1 antibody titres, but all 6 patients with high titres responded to IVIg.…”

Immunotherapy in Peripheral Neuropathies

“…IVIG remains the treatment of choice for patients with MMN [5,[71][72][73][74][75][76]. IVIG is recommended as a first-line therapy, based on a meta-analysis of 4 randomized, double-blind, placebocontrolled, trials [73][74][75][76], involving a total of 34 patients: 78 % of study participants had a significant short-term improvement in strength, selected as primary outcome measure, following IVIG treatment compared with 4 % following placebo [77].…”

“…IVIG is recommended as a first-line therapy, based on a meta-analysis of 4 randomized, double-blind, placebocontrolled, trials [73][74][75][76], involving a total of 34 patients: 78 % of study participants had a significant short-term improvement in strength, selected as primary outcome measure, following IVIG treatment compared with 4 % following placebo [77]. Another RCT was conducted in the USA and Canada to assess the efficacy, safety, and tolerability of 10 % liquid IVIG in 44 patients with MMN [78].…”