Multifocal motor neuropathy

Vucic, Steve; Black, Kristin R.; Chong, Peter Siao Tick; Cros, Didier

doi:10.1212/01.wnl.0000140497.85952.fa

Cited by 123 publications

(110 citation statements)

References 29 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: 87%

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: 87%

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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“…Elevated anti-ganglioside GM1 antibodies and definite conduction block have been shown to be correlated with a favourable response to IVIg (class IV evidence) (EFNS task force, 2008). However, in one retrospective study, treatment with higher than normal maintenance doses of IVIg (1.6-2.0 g/kg given over 4-5 days) promoted re-innervation, decreased the number of conduction blocks and prevented axonal degeneration in 10 MMN patients for up to 12 years (Vucic et al, 2004). Recommendations: -As there is no other treatment of proven benefit, the recommendation is to use IVIg (2 g/kg in 2-5 days) as a first-line treatment (level A).…”

Section: Intravenous Immunoglobulins In Neurological Diseases: Establmentioning

confidence: 98%

Intravenous Immunoglobulins in Neurological Diseases: Established and Novel Clinical Applications

Yiannopoulou¹

2011

Autoimmune Disorders - Current Concepts and Advances From Bedside to Mechanistic Insights

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“…This decline started after 3-7 years (mean 4.8 years) of therapy and correlated with a reduction of distal compound muscle action potential amplitudes. However, another study reported significant and sustained improvement in muscle strength (assessed byMRC score in 8 muscle groups) [81], disability (assessed by Modified Rankin Score), CB, and signs of axonal degeneration on electrophysiological studies in 10 patients with MMN, with a follow-up of 3.5-12.0 years (mean 7.3 years). The authors concluded that long-term IVIG therapy improved muscle strength and functional disability, decreased the number of CBs and the extent of axonal degeneration, and promoted reinnervation.…”

Section: Ivigmentioning

confidence: 99%

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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Multifocal motor neuropathy

Cited by 123 publications

References 29 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

Intravenous Immunoglobulins in Neurological Diseases: Established and Novel Clinical Applications

Immunotherapy in Peripheral Neuropathies

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