How long is IVIg effective in multifocal motor neuropathy?

Terenghi, F.; Cappellari, A.; Bersano, Anna; Carpo, M.; Barbieri, S.; Nobile‐Orazio, Eduardo

doi:10.1212/01.wnl.0000110185.23464.a1

Cited by 128 publications

(116 citation statements)

References 9 publications

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“…It is notable that dosing remained constant or was only slightly changed in the majority of patients over the course of 1 year. Notably, authors of a retrospective study found reliably good shortterm response to IVIG, but declining effectiveness in long-term follow up of patients [Léger et al 2008], that was confirmed by other studies [Van Den BergVos et al 2002;Terenghi et al 2004], while in another study their IgG effectiveness persisted [Vucic et al 2004]. In our study, IgG treatment was stopped in only one patient who did not have a clinical benefit from the treatment.…”

Section: Treatmentsupporting

confidence: 88%

Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Stangel

Gold

Pittrow

et al. 2016

Ther Adv Neurol Disord

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Objectives:The management of patients with multifocal motor neuropathy (MMN) under everyday clinical conditions has been insufficiently studied. We therefore collected comprehensive observational data on patients with MMN who received intravenous (IV) or subcutaneous (SC) immunoglobulins (IGs) as maintenance therapy. Methods: This was a prospective, noninterventional study (registry) in neurological centres (hospitals and offices) throughout Germany. Results: As of 1 December 2015, 80 patients with MMN were included (mean age 55.4 ± 9.8 years, 67% males, mean disease duration 10.7 ± 10.2 years). The affected limb regions were predominantly distal muscle groups of the upper extremities. On the inflammatory neuropathy cause and treatment (INCAT) scale, 94% of the patients had some disability in the arms and 61% in the legs. At inclusion, 98.8% received IVIG and 1.3% SCIG. Substantial variation was observed between IVIG treatment intervals (every 0.7 to 17.3 weeks) and dosage (0.2-2.1 g/kg body weight received during a single administration; mean monthly dosage, 0.9 g/kg body weight). However, the mean monthly dosage was steady over time. At 1-year follow up, improvement was seen in muscle strength, INCAT and quality of life (QoL) scores (SF-36 questionnaire). Conclusions:The management of patients with MMN in everyday clinical practice demonstrates a wide range of absolute dosages and treatment intervals of IG, supporting the recommended practice of determining treatment dose on an individual patient basis. The improvements in muscle strength and reduction in disability, accompanied by increased QoL, strengthen the case for use of IG as a maintenance treatment for MMN.

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Section: Treatmentsupporting

confidence: 88%

Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Stangel

Gold

Pittrow

et al. 2016

Ther Adv Neurol Disord

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“…The authors concluded that IVIG maintenance therapy had a beneficial long-term effect on muscle strength and upper limb disability but may not prevent a slight decrease in muscle strength. Similar results were found by other authors who reported follow-up in 10 patients with MMN responding to an initial course of IVIg with periodic infusion for 5-12 years (mean 8.2 years) [80]. At the last follow-up, only 2 patients had maintained the maximal improvement achieved during therapy, while 8 worsened despite the immunoglobulin dosage.…”

Section: Ivigsupporting

confidence: 89%

Immunotherapy in Peripheral Neuropathies

Léger¹,

Guimarães‐Costa²,

Muntean³

2016

Neurotherapeutics

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Immunotherapy has been investigated in a small subset of peripheral neuropathies, including an acute one, Guillain-Barré syndrome, and 3 chronic forms: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and neuropathy associated with IgM anti-myelin-associated glycoprotein. Several experimental studies and clinical data are strongly suggestive of an immune-mediated pathogenesis. Either cell-mediated mechanisms or antibody responses to Schwann cell, compact myelin, or nodal antigens are considered to act together in an aberrant immune response to cause damage to peripheral nerves. Immunomodulatory treatments used in these neuropathies aim to act at various steps of this pathogenic process. However, there are many phenotypic variants and, consequently, there is a significant difference in the response to immunotherapy between these neuropathies, as well as a need to improve our knowledge and long-term management of chronic forms.

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“…The authors concluded that IVIg maintenance therapy has a beneficial long-term effect on muscle strength and upper limb disability but may not prevent a slight decrease in muscle strength, and that IVIg treatment favourably influences the mechanisms of remyelination or reinnervation but that axon loss cannot be prevented. Same data and conclusions were outlined by Terenghi et al [37] 10 patients with multifocal motor neuropathy responding to an initial course of IVIg with periodic infusion for 5 to 12 years (mean 8.2 years). At last follow-up only 2 patients had maintained the maximal improvement achieved during therapy, while 8 worsened despite increasing IVIg dosage.…”

Section: Ivig In the Long-term Treatment Of Multifocal Motor Neuropathymentioning

confidence: 53%

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

2007

Swiss Arch Neurol Psychiatr

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SummaryLéger J-M, Lievens I. Multifocal motor neuropathy: and then, 20 years later … IVIg therapy. Schweiz Arch Neurol Psychiatr. 2007;158:81-5. Multifocal motor neuropathy is a distinct entity, whose treatment differs from that of other chronic immune-mediated neuropathies, mainly chronic inflammatory demyelinating polyradiculoneuropathy and its variant, multifocal acquired demyelinating sensory and motor neuropathy, although they share some electrophysiological characteristics. From the first descriptions intravenous immunoglobulins have been considered to be the gold standard of treatment for multifocal motor neuropathy. New therapeutic strategies are required, however, that focus on the effects and the costs of this therapy over long-term follow-up.

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How long is IVIg effective in multifocal motor neuropathy?

Cited by 128 publications

References 9 publications

Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Immunotherapy in Peripheral Neuropathies

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

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