This medical condition has either been superseded or has become inactive
| Specific Conditions |
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| Indication for Ig Use |
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| Level of Evidence | Clear evidence of benefit (Category 1) |
| Description and Diagnostic Criteria |
MMN is a relatively rare disorder characterised by slowly progressive, asymmetric, predominately distal limb weakness without sensory impairment. Weakness often begins in the arms and the combination of weakness, wasting, cramps and fasciculations may suggest a diagnosis of motor neuron disease. However, clinical examination may demonstrate that the pattern of weakness follows the distribution of individual nerves rather than a spinal segmental pattern. Investigations will typically show conduction block on nerve conduction studies. Immunoglobulin M (IgM) anti-GM-1 antibodies have been reported in a large number of patients with MMN and provide confirmatory evidence, but are not essential for the diagnosis. |
| Justification for Evidence Category |
The Biotext (2004) review found six low-quality case studies or crossover randomised controlled trials (RCTs) with a total sample size of 68 patients. A possible benefit of intravenous immunoglobulin (IVIg) treatment in these patients was observed, although five studies were not controlled. The Frommer and Madronio (2006) review found one high-quality systematic review (a Cochrane review) of four crossover RCTs with 34 patients. Evidence for improvement in muscle strength with IVIg and limited evidence of a reduction in disability after IVIg administration. Consensus statements assert that IVIg is the only safe treatment demonstrated to work in patients with MMN. It is recommended in those who have significant disability. Dose and monitoring is similar to chronic inflammatory demyelinating polyneuropathy. IVIg therapy is usually long term, but the minimum effective dose for each patient should be sought. Plasma exchange and steroids appear to cause a worsening in the condition of patients with MMN with conduction block. Regular maintenance doses of IVIg are needed. The National Guideline Clearinghouse recommends the use of IVIg in the treatment of patients with progressive, symptomatic MMN that has been diagnosed using electrophysiology, ruling out other possible conditions that may not respond to IVIg treatment. |
| Diagnosis Requirements |
A diagnosis must be made by a Neurologist. |
| Qualifying Criteria for Ig Therapy |
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| Exclusion Criteria |
Presence of upper motor neuron signs. Significant sensory impairment without an adequate alternative explanation. |
| Review Criteria for Assessing the Effectiveness of Ig Use |
IVIg should be used for three to six months (three to six courses) before determining whether the patient has responded. Most individuals will respond within three months, unless there is significant axonal degeneration whereby a six-month course will be necessary. If there is no benefit after three to six courses, IVIg therapy should be abandoned.
Review by a Neurologist is required within six months and at least annually thereafter. Documentation of clinical effectiveness is necessary for continuation of Ig therapy. On review of the initial authorisation period Clinical effectiveness of Ig therapy can be demonstrated by:
On review of a continuing authorisation period For stable patients on maintenance treatment, review by a Neurologist is required at least annually.Clinical effectiveness of Ig therapy can be demonstrated by:
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| Dose |
Aim for the minimum dose to maintain optimal functional status.
Dosing above 1 g/kg per day is contraindicated for some IVIg products. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Refer to the current product information sheet for further information. |
| Bibliography |
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| Association of British Neurologists 2005, Guidelines for the use of intravenous immunoglobulin in neurological diseases, The Association, London. Available from: http://www.theabn.org/media/docs/ABN%20publications/IVIg-guidelines-final-July05.pdf Biotext 2004, ‘Summary data on conditions and papers’, in A systematic literature review and report on the efficacy of intravenous immunoglobulin therapy and its risks, commissioned by the National Blood Authority on behalf of all Australian Governments. Available from: http://www.nba.gov.au/pubs/pdf/report-lit-rev.pdf European Federation of Neurological Societies, Peripheral Nerve Society, van Schaik, IN, Bouche, P, et al 2006, ‘European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy’, European Journal of Neurology, vol. 13, pp. 802–8. Federico, P, Zochodne, DW, Hahn, AF, et al 2000, ‘Multifocal motor neuropathy improved by IVIg: randomized, double-blind, placebo-controlled study’ (comment), Neurology, vol. 55, no. 9, pp. 1256–62. Frommer, M & Madronio, C 2006, The use of intravenous immunoglobulin in Australia. A report for the National Blood Authority, Part B: systematic literature review, Sydney Health Projects Group, University of Sydney, Sydney, pp. 35–7. Kornberg, AJ, for the Asia–Pacific IVIg Advisory Board 2004, Bringing consensus to the use of IVIg in neurology. Expert consensus statements on the use of IVIg in neurology, 1st edn, Asia–Pacific IVIg Advisory Board, Melbourne, pp. 30–4. Van den Berg-Vos, RM, Franssen, H, Wokke, JH, et al 2002, ‘Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment’, Brain, vol. 125, pt 8, pp. 1875–86. Van Schaik, IN, van den Berg, LH, de Haan, R, et al 2005, ‘Intravenous immunoglobulin for multifocal motor neuropathy (Cochrane Review)’, in The Cochrane Library, Issue 2, John Wiley & Sons, Ltd, Chichester, United Kingdom. |