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) An illustration of the VGKC antibodies at work in the autoimmune process.
Acquired neuromyotonia: Evidence for autoantibodies directed against K+ channels of peripheral nerves Paul Shillito, MRCP 1, Peter C. Molenaar, PhD 2, Dr Angela Vincent, MRCPath 1 *, Katherine Leys, DPhil 1, Wang Zheng, MSc 2, Rutgeris J. van den Berg, PhD 2, Jaap J. Plomp, MSc 2, Gertrudis Th. H. Van Kempen, BSc 2, Guy Chauplannaz 4, Axel R. Wintzen, MD 3, J. Gert van Dijk, MD 3, John Newsom-Davis, FRS 1
1Neurosciences Group, Institute of Molecular Medicine, University of Oxford, United Kingdom
2Department of Physiology, University of Leiden, Leiden, Netherlands
3Neurology, University of Leiden, Leiden, Netherlands
4Hôpital Neurologique, Université Claude Bernard, Lyon, France
*Correspondence to Angela Vincent, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, OX3 9DU United Kingdom
#Abstract
Acquired neuromyotonia is characterized by hyperexcitability of motor nerves leading to muscle twitching, cramps, and weakness. The symptoms may improve following plasma exchange, and injection of immunoglobulin G (IgG) from 1 neuromyotonia patient into mice increased the resistance of neuromuscular transmission to d-tubocurarine. Here we examine nerves and muscle in vitro from mice injected with plasma or purified IgG from 6 neuromyotonia patients or pooled control subjects, and cultured dorsal root ganglion cells after treatment with IgG. Three of the patients had antibodies against human voltage-gated potassium channels labeled with 125I--dendrotoxin. The quantal release of acetylcholine (quantal content) at end-plates in diaphragms from mice treated with neuromyotonia IgG preparations was increased by 21% relative to control values (p = 0.0053). With one IgG preparation, the duration of the superficial peroneal nerve compound action currents was increased by 93%. The dorsal root ganglion cells treated with this IgG showed a Marchked increase in repetitive firing of action potentials. All effects were similar to those obtained with aminopyridines. We conclude that at least some patients with acquired neuromyotonia have antibodies directed against aminopyridine- or -dendrotoxin-sensitive K+ channels in motor and sensory neurons, and they are likely to be implicated in the disease process.
Received: 20 March 1995; Revised: 30 June 1995; Accepted: 3 July 1995
) An illustration of the VGKC antibodies at work in the autoimmune process.Acquired neuromyotonia: Evidence for autoantibodies directed against K+ channels of peripheral nerves Paul Shillito, MRCP 1, Peter C. Molenaar, PhD 2, Dr Angela Vincent, MRCPath 1 *, Katherine Leys, DPhil 1, Wang Zheng, MSc 2, Rutgeris J. van den Berg, PhD 2, Jaap J. Plomp, MSc 2, Gertrudis Th. H. Van Kempen, BSc 2, Guy Chauplannaz 4, Axel R. Wintzen, MD 3, J. Gert van Dijk, MD 3, John Newsom-Davis, FRS 1
1Neurosciences Group, Institute of Molecular Medicine, University of Oxford, United Kingdom
2Department of Physiology, University of Leiden, Leiden, Netherlands
3Neurology, University of Leiden, Leiden, Netherlands
4Hôpital Neurologique, Université Claude Bernard, Lyon, France
*Correspondence to Angela Vincent, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, OX3 9DU United Kingdom
#Abstract
Acquired neuromyotonia is characterized by hyperexcitability of motor nerves leading to muscle twitching, cramps, and weakness. The symptoms may improve following plasma exchange, and injection of immunoglobulin G (IgG) from 1 neuromyotonia patient into mice increased the resistance of neuromuscular transmission to d-tubocurarine. Here we examine nerves and muscle in vitro from mice injected with plasma or purified IgG from 6 neuromyotonia patients or pooled control subjects, and cultured dorsal root ganglion cells after treatment with IgG. Three of the patients had antibodies against human voltage-gated potassium channels labeled with 125I--dendrotoxin. The quantal release of acetylcholine (quantal content) at end-plates in diaphragms from mice treated with neuromyotonia IgG preparations was increased by 21% relative to control values (p = 0.0053). With one IgG preparation, the duration of the superficial peroneal nerve compound action currents was increased by 93%. The dorsal root ganglion cells treated with this IgG showed a Marchked increase in repetitive firing of action potentials. All effects were similar to those obtained with aminopyridines. We conclude that at least some patients with acquired neuromyotonia have antibodies directed against aminopyridine- or -dendrotoxin-sensitive K+ channels in motor and sensory neurons, and they are likely to be implicated in the disease process.
Received: 20 March 1995; Revised: 30 June 1995; Accepted: 3 July 1995
