Kimiyoshi Arimura, Yoshito Sonoda, Osamu Watanabe, Tatsui Nagado, Asutsugu Kurono, Hisanori Tomimitsu, Reika Otsuka, Masaki Kameyama, Mitsuhiro Osame
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...
2002: Muscle & Nerve. Supplement