Autoimmune Disorders of Neuronal Potassium Channels

Authors

  • JOHN NEWSOM-DAVIS,

    Corresponding author
    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
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  • CAMILLA BUCKLEY,

    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
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  • LINDA CLOVER,

    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
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  • IAN HART,

    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
    2. Department of Neurology, Walton Hospital, Liverpool, United Kingdom
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  • PAUL MADDISON,

    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
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  • ERDEM TÜZÜM,

    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
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  • ANGELA VINCENT

    1. Weatherall Institute of Molecular Medicine and Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom
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Address for correspondence: John Newsom-Davis, Department of Clinical Neurology, University of Oxford, Oxford OX2 6HE, United Kingdom. Voice: +44 (0)1865 224940; fax: +44 (0)1865 224273. john.newsomdavis@btinternet.com

Abstract

Abstract: Antibodies to voltage-gated potassium channels (VGKCs) appear likely to be the effector mechanisms in many patients with acquired peripheral nerve hyperexcitability (APNH) syndromes, a group of disorders that include neuromyotonia, cramp-fasciculation syndrome, and Isaacs' syndrome. They may contribute to the associated autonomic changes. Through a central action, they may also be the effector mechanism in those with Morvan's syndrome and in some patients with limbic encephalitis. Evidence supporting this hypothesis includes the increased association of APNH with autoimmune diseases (in particular, myasthenia gravis and thymoma), the response to plasmapheresis, passive transfer of APNH to experimental animals by patients' plasma or immunoglobulins, the action of their serum on VGKC currents studied in vitro, and the presence in many patients of IgG antibodies to VGKCs.

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