Isaacs' syndrome as a potassium channelopathy of the nerve.

Journal Article (Journal Article;Review)

Isaacs' syndrome (acquired neuromyotonia) is an antibody-mediated potassium channel disorder (channelopathy). The target channel proteins of the antigens are voltage-gated potassium channels (VGKCs), especially dendrotoxin-sensitive fast potassium channels. The suppression of voltage-gated outward K(+) current by antibodies induces hyperexcitability of the peripheral nerve. Patch clamp studies show that antibodies may not directly block the kinetics of VGKCs but may decrease channel density. Electrophysiological, pharmacological, and immunological findings indicate that the site of origin of spontaneous discharges is principally in the distal portion of the motor nerve and/or within the terminal arborization. The spectrum of potassium channelopathies is expanding. The existence of antibodies against VGKCs should be considered in patients who present with generalized nerve hyperexcitability of undetermined etiology.

Full Text

Duke Authors

Cited Authors

  • Arimura, K; Sonoda, Y; Watanabe, O; Nagado, T; Kurono, A; Tomimitsu, H; Otsuka, R; Kameyama, M; Osame, M

Published Date

  • 2002

Published In

  • Muscle Nerve Suppl

Volume / Issue

  • 11 /

Start / End Page

  • S55 - S58

PubMed ID

  • 12116286

Digital Object Identifier (DOI)

  • 10.1002/mus.10148


  • eng

Conference Location

  • United States