Abstract
Multifocal motor neuropathy (MMN) is a relatively recently recognized clinical entity that was formally described by Parry and Clark in the mid-1980s (1,2). The clinical presentation in each of the patients in this original series had suggested a diagnosis of motor neuron disease, but nerve conduction studies showed multifocal conduction block in motor nerves. The recognition that this syndrome represented a form of a chronic demyelinating neuropathy has spawned much debate regarding its relationship to other chronic demyelinating neuropathies such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and the Lewis-Sumner syndrome, and has also raised the possibility that patients with the disorder might respond to immunosuppressive therapy. What are the clinical and electrophysiological features of MMN, and how does it differ from other chronic demyelinating neuropathies? What is the diagnostic accuracy of anti GM1 antibodies? Do patients with MMN respond to immunosuppressive therapy, and what is the long term prognosis for patients with this disorder? These and other questions are the focus of this chapter.
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References
Parry GJ, Clarke S. Pure motor neuropathy with multifocal conduction block masquerading as motor neurone disease. Muscle Nerve 1985;8:617.
Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve 1988;11:103–107.
Pestronk A, Cornblath D, Ilyas A, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 1988;24:73–78.
Krarup C, Stewart J, Sumner A, Pestronk A, Lipton S. A syndrome of asymmetric limb weakness with motor conduction block. Neurology 1990;40:118–127.
Comi G, Roveri L, Swan A, et al. A randomised controlled trial of intravenous immunoglobulin in IgM paraprotein associated demyelinating neuropathy. J Neurol 2002;249:1370–1377.
Bouche P, Moulonguet A, Younes-Chennoufi AB, et al. Multifocal motor neuropathy with conduction block: a study of 24 patients. J Neurol Neurosurg Psychiatry 1995;59:38–44.
Pestronk A, Chaudhry V, Feldman E, et al. Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies. Ann Neurol 1990;27:316–326.
Katz J, Wolfe G, Bryan W, Jackson C, Amato A, Barohn R. Electrophysiologic findings in multifocal motor neuropathy. Neurology 1997;48:700–707.
Chaudhry V, Corse AM, Cornblath DR, et al. Multifocal motor neuropathy: response to human immune globulin. Ann Neurol 1993;33:237–242.
Pakiam A, Parry G. Multifocal motor neuropathy without overt conduction block. Muscle Nerve 1998;21:243–245.
Comi G, Amadio S, Galardi G, Fazio R, Nemni R. Clinical and neurophysiological assessment of immunoglobulin therapy in five patients with multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 1994;57(Suppl):35–37.
Chaudhry V, Corse AM, Cornblath DR, Kuncl RW, Freimer ML, Griffin JW. Multifocal motor neuropathy: electrodiagnostic features. Muscle Nerve 1994;17:198–205.
Nobile-Orazio E, Meucci N, Barbieri S, Carpo M, Scarlato G. High-dose intravenous immunoglobulin therapy in multifocal motor neuropathy. Neurology 1993;43:537–544.
Adams D, Kuntzer T, Burger D, et al. Predictive value of anti-GM1 ganglioside antibodies in neuromuscular diseases: a study of 180 sera. J Neuroimmunol 1991;32:223–230.
Taylor B, Gross L, Windebank A. The sensitivity and specificity of anti-GM1 antibody testing. Neurology 1996;47:951–955.
Pestronk A, Choksi R. Multifocal motor neuropathy. Serum IgM anti-GM1 ganglioside antibodies in most patients detected using covalent linkage of GM1 to ELISA plates. Neurology 1997;49:1289–1292.
Lewis R, Sumner A, Brown M, Asbury A. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982;32:958–964.
Saperstein DS, Amato AA, Wolfe GI, et al. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve 1999;22:560–566.
Gorson KC, Ropper AH, Weinberg DH. Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 1999;22:758–765.
Van den Berg-Vos R, Van den Berg L, Franssen H, et al. Multifocal inflammatory demyelinating neuropathy. A distinct clinical entity? Neurology 2000;54:26–32.
Oh SJ, Claussen GC, Kim DS. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy. J Periph Nerv Syst 1997;2:362–369.
Viala K, Renie L, Maisonobe T, et al. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndome. Brain 2004;127:2010–2017.
Verschueren A, Azulay JP, Attarian S, Boucraut J, Pellissier JF, Pouget J. Lewis-Sumner syndrome and multifocal motor neuropathy. Muscle Nerve 2005;31:88–94.
Azulay JP, Blin O, Pouget J, et al. Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study. Neurology 1994;44:429–432.
Van den Berg L, Kerkhoff H, Oey P, et al. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry 1995;59:248–252.
Federico P, Zochodne D, Hahn A, Brown W, Feasby T. Multifocal motor neuropathy improved by IVIg: randomized, double-blind, placebo-controlled study. Neurology 2000;55:1256–1262.
Leger J, Chassande B, Musset L, Meininger V, Bouche P, Baumann N. Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind, placebo-controlled study. Brain 2001;124:145–153.
Feldman E, Bromberg M, Albers J, Pestronk A. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol 1991;30:397–401.
Kaji R, Shibasaki H, Kimura J. Multifocal demyelinating motor neuropathy: cranial nerve involvement and immunoglobulin therapy. Neurology 1992;42:506–509.
Donaghy M, Mills K, Boniface S, et al. Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 1994;57:778–783.
Meucci N, Cappellari A, Barbieri S, Scarlato G, Nobile-Orazio E. Long term effect of intravenous immunoglobulins and oral cyclophosphamide in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 1997;63:765–769.
Azulay J-P, Rihet P, Pouget J, et al. Long term follow-up of multifocal motor neuropathy with conduction block under treatment. J Neurol Neurosurg Psychiatry 1997;62:391–394.
Van den Berg L, Lokhorst H, Wokke J. Pulsed high-dose dexamethasone is not effective in patients with multifocal motor neuropathy. Neurology 1997;48:1135.
Carpo M, Cappellari A, Mora G, et al. Deterioration of multifocal motor neuropathy after plasma exchange. Neurology 1998;50:1480–2.
Martina I, van Doorn P, Schmitz P, Meulstee J, van der meche F. Chronic motor neuropathies: response to interferon-beta-1a after failure of conventional therapies. J Neurol Neurosurg Psychiatry 1999;66:917–201.
Van den Berg-Vos R, Van den Berg L, Franssen H, van Doorn P, Merkies I, Wokke J. Treatment of multifocal motor neuropathy with interferon-beta-1A. Neurology 2000;54:1518–1521.
Van den Berg L, Franssen H, Wokke J. The long-term effect of intravenous immunoglobulin treatment in multifocal motor neuropathy. Brain 1998;121(Pt 3):421–8.
Taylor B, Wright R, Harper C, Dyck P. Natural history of 46 patients with multifocal motor neuropathy with conduction block. Muscle Nerve 2000;23:900–8.
Van den Berg-Vos R, Franssen H, Wokke J, van Es H, Van den Berg L. Multifocal motor neuropathy: diagnostic criteria that predict the response to immunoglobulin treatment. Ann Neurol 2000;48:919–926.
Nobile-Orazio E, Cappellari A, Meucci N, et al. Multifocal motor neuropathy: clinical and immunological features and response to IVIg in relation to the presence and degree of motor conduction block. J Neurol Neurosurg Psychiatry 2002;72:761–766.
Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001;24:311–324.
Jaspert A, Claus D, Grehl H, Neundorfer B. Multifocal motor neuropathy: clinical and electrophysiological findings. J Neurol 1996;243:684–692.
Kermode A, Laing B, Carroll W, Mastaglia F. Intravenous immunoglobulin for multifocal motor neuropathy. Lancet 1992;340:920–921.
Charles N, Benoit P, Vial C, Bierme T, Moreau T, Bady B. Intravenous immunoglobulin treatment in multifocal motor neuropathy. Lancet 1992;340:182.
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© 2006 Humana Press Inc., Totowa, NJ
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Benatar, M. (2006). Multifocal Motor Neuropathy. In: Benatar, M. (eds) Neuromuscular Disease. Humana Press. https://doi.org/10.1007/978-1-59745-106-2_13
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DOI: https://doi.org/10.1007/978-1-59745-106-2_13
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