Abstract
We describe a patient with a pure motor chronic demyelinating polyneuropathy with an IgM monoclonal component showing anti-GM2, GalNAc-GD1a and GalNAc-GM1b reactivity whose common epitope appears to be -[GalNAcbeta1-4Gal(3-2alphaNeuAc)beta1]. We used intracellular recording to study how IgM from this patient affected neurotransmitter release in the mouse diaphragm in vitro. Adding serum (and specifically, the purified monoclonal IgM component) blocked the nerve-evoked response in both quantal content and evoked endplate potential (EPP) amplitude in a complement-independent and reversible manner. The IgM increased the frequency of spontaneous miniature endplate potentials (MEPPs) in a complement-dependent and reversible manner but had no effect on MEPP amplitude.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Animals
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Antibodies, Monoclonal / immunology*
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Antibody Specificity
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Blood Physiological Phenomena
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Chronic Disease
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Demyelinating Diseases / blood
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Demyelinating Diseases / immunology*
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Demyelinating Diseases / metabolism
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Demyelinating Diseases / physiopathology
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Diaphragm / innervation
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Electrophysiology
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G(M1) Ganglioside / analogs & derivatives
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G(M1) Ganglioside / immunology*
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G(M2) Ganglioside / immunology*
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Gangliosides / immunology*
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Humans
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Immunoglobulin M / immunology*
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Immunoglobulin M / pharmacology
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Male
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Mice
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Motor Endplate / physiology
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Neuromuscular Junction / metabolism
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Neurotransmitter Agents / metabolism
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Peripheral Nervous System Diseases / blood
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Peripheral Nervous System Diseases / immunology*
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Peripheral Nervous System Diseases / metabolism
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Peripheral Nervous System Diseases / physiopathology
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Reference Values
Substances
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Antibodies, Monoclonal
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Gangliosides
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Immunoglobulin M
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Neurotransmitter Agents
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G(M2) Ganglioside
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G(M1) Ganglioside
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ganglioside M1b
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IV(4)-galactosyl-N-acetylganglioside GD1a