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Article highlights Multiple screening tests are available to screen patients for cognitive impairment, and the Confusion Assessment Method is a helpful test to screen for delirium in the immediate postoperative period. Medicine reconciliation and identification and removal of potentially inappropriate medications per Beer’s list are important tasks that should not be overlooked, especially during a preoperative appointment. […]

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Neuromuscular Diseases
2 mins

The Long-term Treatment of Multifocal Motor Neuropathy with Intravenous Immunoglobulin

Leonard H van den Berg
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Published Online: May 15th 2012 European Neurological Review, 2012;7(2):128-133 DOI: http://doi.org/10.17925/ENR.2012.07.02.128
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1

Abstract

Overview

Multifocal motor neuropathy (MMN) is a rare, purely motor neuropathy. It is a progressive disorder, most patients eventually developing severe fatigue and weakness in the arm muscles that severely impair daily functioning and quality of life. Unlike other motor neuropathies such as motor neurone disease, MMN is treatable with regular infusions of intravenous immunoglobulin (IVIg). Four double-blind, randomised, placebo-controlled studies have shown that in the short term, IVIg significantly improves muscle strength and disability in more than 70 % of patients. The 11 observational studies reviewed in this article confirm that long-term maintenance treatment with IVIg maintains clinical improvement compared to pre-treatment baseline in most patients. Infusions are generally well tolerated, but regular monitoring and re-evaluation of the IVIg maintenance regimen is essential, as most patients need progressive increases in dosage or reduced intervals between infusions to maintain their response to treatment. In the absence of accepted predictive markers, maintenance IVIg should be individualised, based on each patient’s initial response, disability and the interval between the first infusion and decline in muscle strength.

Keywords

Multifocal motor neuropathy, intravenous immunoglobulin, maintenance, treatment

2

Article

Multifocal motor neuropathy (MMN) is a rare, purely motor neuropathy with a prevalence of approximately 0.6 per 100,000 and a median age of onset of 40 years.1 Men are more likely than women to be affected, in a ratio of 2.7:1 and are usually diagnosed at an earlier age.1 Patients with MMN typically present with asymmetrical, predominantly distal limb weakness that follows individual nerves. There is no apparent sensory loss and weakness usually starts in the forearm or hand muscles, though the first symptoms may occur in the distal leg and upper arm. Respiratory and bulbar muscles are unaffected and patients have a normal life expectancy. However, since MMN is a progressive disorder, most patients eventually develop severe fatigue and weakness in the arm muscles, resulting in disability that can seriously impair daily functioning and quality of life.

Unlike other motor neuropathies such as motor neurone disease (MND), MMN is treatable with intravenous immunoglobulin (IVIg).2 Most patients require regular infusions in order to maintain clinical response and the aim of this review is to consider the effectiveness and safety of long-term or maintenance treatment of MMN with IVIg.

Method
The terms ‘multifocal motor neuropathy’, ‘treatment’, ‘long term’, ‘maintenance’ and ‘IVIg’ were used to conduct a PubMed search of articles published in English language journals between 1 January 1980 and 31 December 2011 (see Table 1). Papers were excluded ifthey were single case reports, or were superseded by subsequent publications following up the same group of patients.

Results
The manual search of the results of the PubMed literature searchidentified a total of 14 studies concerning the long-term or maintenance treatment of MMN with IVIg. Three papers were excluded:

  • a case report in one patient;3
  • a publication concerning six patients included in a subsequent, larger study;4,5
  • and a study of dose titration in patients on maintenance IVIg.6

The remaining 11 studies listed in Table 2 were included in the analysis.1,5,7–15 All were retrospective, observational studies, except for one cross-sectional, descriptive study.

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2

References

  1. Cats EA, van der Pol WL, Piepers S, et al., Correlates of
    outcome and response to IVIg in 88 patients with multifocal
    motor neuropathy, Neurology, 2010;75:818–25.

  2. Van Schaik IN, van den Berg LH, de Haan R, Vermeulen M,
    Intravenous immunoglobulin for multifocal motor neuropathy
    (Review), Cochrane Database Syst Rev, 2005;2:CD004429.

  3. Van den Berg LH, Franssen H, Wokke JH, Improvement of
    multifocal motor neuropathy during long-term weekly
    treatment with human immunoglobulin, Neurology,
    1995;45(5):987–8.

  4. Van den Berg LH, Franssen H, Wokke JH, The long-term
    effect of intravenous immunoglobulin treatment in
    multifocal motor neuropathy, Brain, 1998;121(Pt. 3):421–8.

  5. Van den Berg-Vos RM, Franssen H, Wokke JH, Van den Berg
    LH, Multifocal motor neuropathy: long-term clinical and
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    2002;125:1875–86.

  6. Baumann A, Hess CW, Sturzenegger M, IVIg dose increase
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    follow-up, J Neurol, 2009;256(4):608–14.

  7. Azulay JP, Rihet P, Pouget J, et al., Long term follow up of
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  8. Delmont E, Azulay JP, Giorgi R, et al., Multifocal motor
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  11. Lucas M, Hugh-Jones K, Welby A, et al., Immunomodulatory
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  15. Vucic S, Black KR, Chong PS, Cros D, Multifocal motor
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    reinnervation with long-term IVIg, Neurology, 2004;63:1264–9.

  16. Joint Task Force of the EFNS and PNS, European Federation
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    Society—first revision, Eur J Neurol, 2010;17:356–63.

  17. Van den Berg LH, Lokhorst H, Wokke JH, Pulsed high-dose
    dexamethasone is not effective in patients with multifocal
    motor neuropathy, Neurology, 1997;48:1135.

  18. Carpo M, Cappellari A, Mora G, et al., Deterioration of
    multifocal motor neuropathy after plasma exchange,
    Neurology, 1998;50:1480–2.

  19. Léger JM, Lievens I, Multifocal motor neuropathy:
    and then, 20 years later…IVIg therapy, Schweizer
    Archiv für Neurologie und Psychiatrie, 2007;158:81–5.

  20. Umapathi T, Hughes RAC, Nobile-Orazio E, Léger JM,
    Immunosuppressant and immunomodulatory treatments for
    multifocal motor neuropathy, Cochrane Database Syst Rev,
    2009;1:CD003217.

  21. Piepers S, Van den Berg-Vos R, Van der Pol WL, et al.,
    Mycophenolate mofetil as adjunctive therapy for MMN patients:
    a randomized, controlled trial, Brain, 2007;130:2004–10.

  22. Azulay JP, Blin O, Pouget J, et al., Intravenous
    immunoglobulin treatment in patients with motor neuron
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    placebo-controlled study, Neurology, 1994;44:429–32.

  23. Federico P, Zochodne DW, Hahn AF, et al., Multifocal motor
    neuropathy improved by IVIg: randomized, double-blind,
    placebo-controlled study, Neurology, 2000;55:1256–62.

  24. Léger JM, Chassande B, Musset L, et al., Intravenous
    immunoglobulin therapy in multifocal motor neuropathy: a
    double-blind, placebo-controlled study, Brain, 2001;124:145–53.

  25. Van den Berg LH, Kerkhoff H, Oey PL, et al., Treatment of
    multifocal motor neuropathy with high dose intravenous
    immunoglobulins: a double blind, placebo controlled study,
    J Neurol Neurosurg Psychiatry, 1995;59:248–52.

  26. Van Asseldonk JT, Van den Berg LH, Kalmijn S, et al., Axon
    loss is an important determinant of weakness in multifocal
    motor neuropathy, J Neurol Neurosurg Psychiatry,
    2006;77:743–7.

  27. Van der Pol W-L, Cats EA, van den Berg LH, Intravenous
    immunoglobulin treatment in multifocal motor neuropathy,
    J Clin Immunol, 2010;30(Suppl. 1):S79–83.

  28. Vlam L, van der Pol L, Cats EA, et al., Multifocal motor
    neuropathy: diagnosis, pathogenesis and treatment
    strategies, Nat Rev Neurol, 2011;8:48–58.

  29. Cats EA, van der Pol WL, Bertens AS, van den Berg LH,
    Home-based IVIg treatment is convenient and time-saving
    in patients with multifocal motor neuropathy,
    J Peripher Nerv Syst, 2011;16:147–9.

  30. Eftimov F, Vermeulen M, de Haan RJ, et al., Subcutaneous
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    J Peripher Nerv Syst, 2009;14:93–100.

  31. Harbo T, Andersen H, Hess A, et al., Subcutaneous versus
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  32. Misbah SA, Baumann A, Fazio R, et al., A smooth transition
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3

Article Information

Disclosure

Leonard H van den Berg has received a travel grant and honoraria from Baxter International Inc.

Correspondence

Leonard H van den Berg, Department of Neurology G03.228, PO Box 85500, 3508 GA Utrecht, The Netherlands. E: l.h.vandenberg@umcutrecht.nl

Support

The publication of this article was funded by Baxter Innovations GmbH. The views and opinions expressed are those of the author and not necessarily those of Baxter Innovations GmbH.

Received

2012-03-05T00:00:00

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Duchenne muscular dystrophy (DMD) is an X-linked recessive, progressive and universally fatal disease in the spectrum of dystrophinopathies,1 with an incidence of 21.4 patients in 100,000 live male births worldwide.2,3 Historically, patients with DMD would lose ambulation by the age of 10 due to muscle weakness and die by the age of 20 from respiratory failure […]

European Neurological Review, 2012;7(2):128-133 DOI: http://doi.org/10.17925/ENR.2012.07.02.128
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